PMID- 24633036 OWN - NLM STAT- In-Data-Review DA - 20140317 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 53 IP - 6 DP - 2014 TI - Successful use of intensive

immunosuppressive therapy for treating simulta neously occurring cerebral lesions and pulmonary arterial hypertension in a patien t with systemic lupus erythematosus. PG - 627-31 AB - A 59-year-old woman who had been diagnosed with systemic lupus erythematos us (SLE) was admitted to our hospital due to paralysis in all of her limbs. T he patient presented with dysarthria, cerebellar ataxia and hypoxia. Magnetic resonance imaging (MRI) revealed vasogenic edema in the brain stem and the cerebellum. She was diagnosed with neuropsychiatric lupus syndrome (NPSLE) and pulmonary arterial hypertension (PAH), and was successfully treated using immunosuppressive therapy. To our knowledge, this is the first reported ca se of simultaneously developing NPSLE and PAH. FAU - Watanabe, Ryu AU - Watanabe R AD - Department of Hematology and Rheumatology, Tohoku University Graduate Scho ol of Medicine, Japan. FAU - Fujii, Hiroshi AU - Fujii H FAU - Shirai, Tsuyoshi AU - Shirai T FAU - Saito, Shinichiro AU - Saito S FAU - Hatakeyama, Akira AU - Hatakeyama A FAU - Sugimura, Koichiro AU - Sugimura K FAU - Fukumoto, Yoshihiro AU - Fukumoto Y FAU - Ishii, Tomonori AU - Ishii T FAU - Harigae, Hideo AU - Harigae H LA - eng PT - Journal Article PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM EDAT- 2014/03/19 06:00 MHDA- 2014/03/19 06:00 CRDT- 2014/03/18 06:00 AID - DN/JST.JSTAGE/internalmedicine/53.0514 [pii] PST - ppublish

SO - Intern Med. 2014;53(6):627-31. PMIDOWN STATDA IS IS VI IP DP TI irin: 24520278 NLM Publisher 20140212 1792-0981 (Print) 1792-0981 (Linking) 7 3 2014 Mar Effectiveness of cilostazol in transient ischemic attack refractory to asp A report of two cases. PG - 739-741 AB - Transient ischemic attack (TIA) is a warning of impending ischemic stroke. It provides an important therapeutic time window in which appropriate interve ntion may prevent permanent neurological injury. The anti-platelet agent, aspiri n, is an option for reducing the risk of stroke following TIA. However, for pati ents who are not responsive to aspirin, cilostazol may be an effective treatmen t. The current study presents two cases of TIA that were refractory to aspirin bu t were successfully treated with cilostazol. In case 1, an 83-year-old female pat ient suffered from episodes of weakness and numbness of the left extremities. A spirin alone or aspirin in combination with clopidogrel were not effective. Anticoagulation therapy with low molecular heparin decreased the frequency of ischemic episodes with complete remission following antiplatelet therapy w ith cilostazol. In case 2, a 51-year-old male presentedwith episodes of paroxy smal weakness of the left extremities with dysarthria. Antiplatelet therapy wit h aspirin was initiated. Eight episodes of ischemic attack recurred on the s eventh day following admission. After the change of the antiplatelet agent to cilostazol, no ischemic episodes recurred, with the exception of three on the first day. This study suggested that cilostazol may be efficacious in the prevention of ischemic stoke following TIA of a non-cardiac origin that wa s not responsive to aspirin. FAU - Lin, Gaoping AU - Lin G AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. FAU - Ren, Dongdong AU - Ren D AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. FAU - Guo, Shunyuan AU - Guo S AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou,

Zhejiang 310014, P.R. China. FAU - Geng, Yu AU - Geng Y AD - Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou, Zhejiang 310014, P.R. China. LA - ENG PT - JOURNAL ARTICLE DEP - 20131231 TA - Exp Ther Med JT - Experimental and therapeutic medicine JID - 101531947 PMC - PMC3919941 OTO - NOTNLM OT - aspirin OT - cilostazol OT - transient ischemic attack EDAT- 2014/02/13 06:00 MHDA- 2014/02/13 06:00 CRDT- 2014/02/13 06:00 PHST- 2013/08/07 [received] PHST- 2013/12/23 [accepted] PHST- 2013/12/31 [epublish] AID - 10.3892/etm.2013.1468 [doi] AID - etm-07-03-0739 [pii] PST - ppublish SO - Exp Ther Med. 2014 Mar;7(3):739-741. Epub 2013 Dec 31. PMIDOWN STATDA IS IS VI DP TI PG LID AB ions) 24438445 NLM In-Process 20140122 1471-2377 (Electronic) 1471-2377 (Linking) 14 2014 High-resolution MRI findings in patients with capsular warning syndrome. 16 10.1186/1471-2377-14-16 [doi] BACKGROUND: Capsular warning syndrome (CWS) is rare (1.5% of TIA presentat

but has a poor prognosis (7-day stroke risk of 60%). Up to date, the exact pathogenic mechanism of CWS has not been fully understood. We report the c linical presentations and high-resolution MRI (HR MRI) findings of two cases with capsular warning symptoms. CASE PRESENTATION: Case 1 was a 63-year-old man with a history of hypertension with recurrent episodes of left hemiparesis and dysarthria lasting 10 ~ 30 minutes. Case 2 was a 54-year-old woman with repetitive episodes of transient left hemiparesis and dysarthria lasting a bout 10 minutes. Capsular infarctions on DWI were demonstrated in the territory of a lenticulostriate artery in both 2 patients. HR MRI disclosed atherosclerot ic plaques on the ventral wall of the MCA where enticulostriate arteries were arisen from, although traditional digital subtraction angiography showed normal. Aggressive medical therapy with dual antithrombotic agents and statin was effective in these two cases. CONCLUSION: Our HR MRI data offer an insight into

the pathophysiology of CWS which might be caused by atherosclerotic plaque in non-stenotic MCA wall. HR MRI might be a useful modality for characterizin g atherosclerotic plaques in the MCA and detecting the pathophysiology of th e CWS. FAU - Zhou, Lixin AU - Zhou L FAU - Ni, Jun AU - Ni J FAU - Xu, Weihai AU - Xu W FAU - Yao, Ming AU - Yao M FAU - Peng, Bin AU - Peng B FAU - Li, Mingli AU - Li M FAU - Cui, Liying AU - Cui L AD - Department of Neurology, Peking Union Medical College Hospital and Chinese Academy of Medical Science, Shuai Fu Yuan 1#, Dong Cheng District, Beijing 100730, China. pumchcuily@sina.com. LA - eng PT - Journal Article DEP - 20140120 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 SB - IM PMC - PMC3898091 OID - NLM: PMC3898091 EDAT- 2014/01/21 06:00 MHDA- 2014/01/21 06:00 CRDT- 2014/01/21 06:00 PHST- 2013/09/04 [received] PHST- 2014/01/15 [accepted] PHST- 2014/01/20 [aheadofprint] AID - 1471-2377-14-16 [pii] AID - 10.1186/1471-2377-14-16 [doi] PST - epublish SO - BMC Neurol. 2014 Jan 20;14:16. doi: 10.1186/1471-2377-14-16. PMIDOWN STATDA IS IS DP TI e 24390182 NLM Publisher 20140106 1349-8029 (Electronic) 0470-8105 (Linking) 2013 Dec 27 Vertebrobasilar Infarction Related to Giant Cell (Temporal) Arteritis: Cas

Report. AB - An 84-year-old male with a 3-month history of headache and elevated C-reac tive protein levels was admitted for biopsy of the superficial temporal artery, which led to the diagnosis of giant cell arteritis (GCA). Two days after prednis olone

therapy was initiated, the patient began to experience transient vertigo a ttacks. Two days later, dysarthria, left-sided hemiparesis, right abducens palsy, and horizontal nystagmus developed. Magnetic resonance (MR) imaging disclosed fresh infarctions in the vertebrobasilar territory. Since the patient became dro wsy because of brainstem compression and hydrocephalus due to cerebellar swell ing, emergency suboccipital decompression surgery and ventricular drainage were performed. Subsequently, the patient's consciousness levels improved. MR angiography revealed right vertebral artery (VA) occlusion and left VA ste nosis due to arteritis. Ischemic stroke is a serious though relatively rare complication of GCA. Similar cases have been reported, in which ischemic s troke developed despite or possibly due to steroid therapy. To our knowledge, th is is the first description of vertebrobasilar infarction associated with GCA in the Japanese population. The merits and potential demerits of steroid therapy are briefly discussed. FAU - Haisa, Toshihiko AU - Haisa T AD - Department of Neurosurgery, JR Tokyo General Hospital. FAU - Tsuda, Tokutaro AU - Tsuda T FAU - Hagiwara, Kiyofumi AU - Hagiwara K FAU - Kikuchi, Takeshi AU - Kikuchi T FAU - Seki, Kunihiko AU - Seki K LA - ENG PT - JOURNAL ARTICLE DEP - 20131227 TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 EDAT- 2014/01/07 06:00 MHDA- 2014/01/07 06:00 CRDT- 2014/01/07 06:00 AID - DN/JST.JSTAGE/nmc/cr.2013-0038 [pii] PST - aheadofprint SO - Neurol Med Chir (Tokyo). 2013 Dec 27. PMIDOWN STATDA IS IS VI DP TI PG LID AB 24359465 NLM In-Process 20140106 1471-2377 (Electronic) 1471-2377 (Linking) 13 2013 Marchiafava-Bignami disease mimics motor neuron disease: case report. 208 10.1186/1471-2377-13-208 [doi] BACKGROUND: Marchiafava-Bignami disease (MBD) is a rare neurologic complic

ation of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. Various reversible neurologic sympto ms are found in patients with MBD. Dysarthria and dysphagia are found in various neurological diseases. CASE PRESENTATION: We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. On admission, the patient was alert with mild cognitive dysfunc tion. The facial expression was flat, and there was weakness of the orbicularis oris bilaterally. The patient's speech was slurred, there was difficulty swallo wing, and the gag reflex and palate elevation were poor. The jaw jerk reflex was brisk and the snout reflex was positive. Neither tongue atrophy nor fasciculatio n were found. Bilateral upper and lower limb weakness with increased bilateral up per limb reflexes and Babinski reflexes were found. Because he had progressive dysarthria and dysphagia with upper and lower motor neuron signs, the init ial diagnosis was motor neuron disease. However, electrophysiological analysis was normal. The vitamin B1 level was 14 ng/mL (normal: >24 ng/mL), and MRI rev ealed hyperintense lesions in the splenium of the corpus callosum and the primar y motor cortices bilaterally. After vitamin B therapy for 17 days, the neurologica l disorders alleviated concurrently with disappearance of the lesions on MRI , which led to the definitive diagnosis of MBD. CONCLUSIONS: MBD presenting with t hese lesions can mimic motor neuron disease clinically. FAU - Hoshino, Yasunobu AU - Hoshino Y FAU - Ueno, Yuji AU - Ueno Y AD - Department of Neurology, Juntendo University Urayasu Hospital, 2-1-1 Tomio ka, Urayasu, Chiba 279-0021, Japan. yuji-u@juntendo.ac.jp. FAU - Shimura, Hideki AU - Shimura H FAU - Miyamoto, Nobukazu AU - Miyamoto N FAU - Watanabe, Masao AU - Watanabe M FAU - Hattori, Nobutaka AU - Hattori N FAU - Urabe, Takao AU - Urabe T LA - eng PT - Journal Article DEP - 20131221 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555

SB PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO -

IM PMC3880166 NLM: PMC3880166 2013/12/24 06:00 2013/12/24 06:00 2013/12/24 06:00 2013/09/19 [received] 2013/12/17 [accepted] 2013/12/21 [aheadofprint] 1471-2377-13-208 [pii] 10.1186/1471-2377-13-208 [doi] epublish BMC Neurol. 2013 Dec 21;13:208. doi: 10.1186/1471-2377-13-208.

PMID- 24316510 OWN - NLM STAT- MEDLINE DA - 20140115 DCOM- 20140311 IS - 1460-2156 (Electronic) IS - 0006-8950 (Linking) VI - 137 IP - Pt 1 DP - 2014 Jan TI - Pantethine treatment is effective in recovering the disease phenotype indu ced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model. PG - 57-68 LID - 10.1093/brain/awt325 [doi] AB - Pantothenate kinase-associated neurodegeneration, caused by mutations in t he PANK2 gene, is an autosomal recessive disorder characterized by dystonia, dysarthria, rigidity, pigmentary retinal degeneration and brain iron accumulation. PANK2 encodes the mitochondrial enzyme pantothenate kinase t ype 2, responsible for the phosphorylation of pantothenate or vitamin B5 in the biosynthesis of co-enzyme A. A Pank2 knockout (Pank2(-/-)) mouse model did not recapitulate the human disease but showed azoospermia and mitochondrial dysfunctions. We challenged this mouse model with a low glucose and high l ipid content diet (ketogenic diet) to stimulate lipid use by mitochondrial beta-oxidation. In the presence of a shortage of co-enzyme A, this diet co uld evoke a general impairment of bioenergetic metabolism. Only Pank2(-/-) mic e fed with a ketogenic diet developed a pantothenate kinase-associated neurodegeneration-like syndrome characterized by severe motor dysfunction, neurodegeneration and severely altered mitochondria in the central and per ipheral nervous systems. These mice also showed structural alteration of muscle morphology, which was comparable with that observed in a patient with pantothenate kinase-associated neurodegeneration. We here demonstrate that pantethine administration can prevent the onset of the neuromuscular pheno type in mice suggesting the possibility of experimental treatment in patients with pantothenate kinase-associated neurodegeneration. FAU - Brunetti, Dario AU - Brunetti D

AD - 1 Unit of Molecular Neurogenetics, Foundation IRCCS Neurological Institute C. Besta, Milan, Italy. FAU - Dusi, Sabrina AU - Dusi S FAU - Giordano, Carla AU - Giordano C FAU - Lamperti, Costanza AU - Lamperti C FAU - Morbin, Michela AU - Morbin M FAU - Fugnanesi, Valeria AU - Fugnanesi V FAU - Marchet, Silvia AU - Marchet S FAU - Fagiolari, Gigliola AU - Fagiolari G FAU - Sibon, Ody AU - Sibon O FAU - Moggio, Maurizio AU - Moggio M FAU - d'Amati, Giulia AU - d'Amati G FAU - Tiranti, Valeria AU - Tiranti V LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131206 PL - England TA - Brain JT - Brain : a journal of neurology JID - 0372537 RN - 0 (Triglycerides) RN - 496-65-1 (Pantetheine) RN - 7K81IL792L (pantethine) RN - 97C5T2UQ7J (Cholesterol) RN - EC 2.7.1.- (Phosphotransferases (Alcohol Group Acceptor)) RN - EC 2.7.1.33 (pantothenate kinase) SB - AIM SB - IM CIN - Brain. 2014 Jan;137(Pt 1):8-11. PMID: 24424915 MH - Animals MH - Behavior, Animal/physiology MH - Brain/pathology MH - Cholesterol/blood MH - Energy Metabolism/physiology MH - Female MH - Heredodegenerative Disorders, Nervous System/*genetics/physiopathology/psy chology MH - Immunohistochemistry MH - Ketogenic Diet/*adverse effects MH - Male MH - Membrane Potential, Mitochondrial/physiology MH - Mice MH - Mice, Knockout MH - Microscopy, Electron MH - Mitochondria/pathology MH - Motor Skills/physiology MH - Neurons/pathology

MH MH MH MH MH MH PMC OID OTO OT OT OT OT EDATMHDACRDTPHSTAID AID PST SO 6. PMIDOWN STATDA IS IS VI IP DP TI -

Pantetheine/*analogs & derivatives/therapeutic use Peripheral Nervous System/pathology/physiopathology Phenotype Phosphotransferases (Alcohol Group Acceptor)/*genetics/physiology Sciatic Nerve/pathology Triglycerides/blood PMC3891449 NLM: PMC3891449 NOTNLM ketogenic diet mitochondria pantethine pantothenate kinase-associated neurodegeneration (PKAN) 2013/12/10 06:00 2014/03/13 06:00 2013/12/10 06:00 2013/12/06 [aheadofprint] awt325 [pii] 10.1093/brain/awt325 [doi] ppublish Brain. 2014 Jan;137(Pt 1):57-68. doi: 10.1093/brain/awt325. Epub 2013 Dec

24223913 NLM In-Process 20131113 1932-6203 (Electronic) 1932-6203 (Linking) 8 11 2013 Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis. PG - e79241 LID - 10.1371/journal.pone.0079241 [doi] AB - BACKGROUND: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therap y. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these pa tients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS . METHODS: Data were collected in a standardized way 2-6 months preoperative ly, 1-5 months (early) and 6 months or more (late) after surgery at the last follo w-up visit (mean follow-up: 29.5 months). RESULTS: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mea n improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Fun ctional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independen ce

Score) improvement reached significance at early post-surgery only (mean 3 7.3%). Long term significant improvement of motor symptom severity (>/= 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia impr oved, whereas effects on dysarthria and swallowing were variable. Parkinsonism d id not improve. Linear regression analysis showed that preoperative motor severit y predicted motor improvement at both post-surgery time points. The most ser ious adverse event was device infection and cerebral abscess, and one patient d ied suddenly of unclear cause, 4 years after surgery. CONCLUSION: This study s hows that bilateral DBS of the GPi effectively reduces the severity of drug-res istant hyperkinetic movement disorders such as present in ChAc. FAU - Miquel, Marie AU - Miquel M AD - Service de Neurologie, CHU Bordeaux, Bordeaux, France ; Service de Neurolo gie, CH Francois Mitterrand, Pau, France. FAU - Spampinato, Umberto AU - Spampinato U FAU - Latxague, Chrystelle AU - Latxague C FAU - Aviles-Olmos, Iciar AU - Aviles-Olmos I FAU - Bader, Benedikt AU - Bader B FAU - Bertram, Kelly AU - Bertram K FAU - Bhatia, Kailash AU - Bhatia K FAU - Burbaud, Pierre AU - Burbaud P FAU - Burghaus, Lothar AU - Burghaus L FAU - Cho, Jin Whan AU - Cho JW FAU - Cuny, Emmanuel AU - Cuny E FAU - Danek, Adrian AU - Danek A FAU - Foltynie, Thomas AU - Foltynie T FAU - Garcia Ruiz, Pedro J AU - Garcia Ruiz PJ FAU - Gimenez-Roldan, Santiago AU - Gimenez-Roldan S FAU - Guehl, Dominique AU - Guehl D FAU - Guridi, Jorge AU - Guridi J FAU - Hariz, Marwan AU - Hariz M FAU - Jarman, Paul AU - Jarman P FAU - Kefalopoulou, Zinovia Maria

AU - Kefalopoulou ZM FAU - Limousin, Patricia AU - Limousin P FAU - Lipsman, Nir AU - Lipsman N FAU - Lozano, Andres M AU - Lozano AM FAU - Moro, Elena AU - Moro E FAU - Ngy, Dhita AU - Ngy D FAU - Rodriguez-Oroz, Maria Cruz AU - Rodriguez-Oroz MC FAU - Shang, Huifang AU - Shang H FAU - Shin, Hyeeun AU - Shin H FAU - Walker, Ruth H AU - Walker RH FAU - Yokochi, Fusako AU - Yokochi F FAU - Zrinzo, Ludvic AU - Zrinzo L FAU - Tison, Francois AU - Tison F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131105 PL - United States TA - PLoS One JT - PloS one JID - 101285081 SB - IM PMC - PMC3818425 OID - NLM: PMC3818425 EDAT- 2013/11/14 06:00 MHDA- 2013/11/14 06:00 CRDT- 2013/11/14 06:00 PHST- 2013 [ecollection] PHST- 2013/07/15 [received] PHST- 2013/09/19 [accepted] PHST- 2013/11/05 [epublish] AID - 10.1371/journal.pone.0079241 [doi] AID - PONE-D-13-29059 [pii] PST - epublish SO - PLoS One. 2013 Nov 5;8(11):e79241. doi: 10.1371/journal.pone.0079241. eCol lection 2013. PMID- 24135395 OWN - NLM STAT- In-Process DA - 20140116 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 8 DP - 2013 TI - Niemann-Pick disease type C symptomatology: an expert-based clinical descr iption.

PG - 166 LID - 10.1186/1750-1172-8-166 [doi] AB - Niemann-Pick disease type C (NP-C) is a rare, progressive, irreversible di sease leading to disabling neurological manifestations and premature death. The estimated disease incidence is 1:120,000 live births, but this likely repr esents an underestimate, as the disease may be under-diagnosed due to its highly heterogeneous presentation. NP-C is characterised by visceral, neurologica l and psychiatric manifestations that are not specific to the disease and that c an be found in other conditions. The aim of this review is to provide non-specia lists with an expert-based, detailed description of NP-C signs and symptoms, inc luding how they present in patients and how they can be assessed. Early disease detection should rely on seeking a combination of signs and symptoms, rath er than isolated findings. Examples of combinations which are strongly suggestive of NP-C include: splenomegaly and vertical supranuclear gaze palsy (VSGP); splenom egaly and clumsiness; splenomegaly and schizophrenia-like psychosis; psychotic s ymptoms and cognitive decline; and ataxia with dystonia, dysarthria/dysphagia and cognitive decline. VSGP is a hallmark of NP-C and becomes highly specific of the disease when it occurs in combination with other manifestations (e.g. splenomegaly, ataxia). In young infants (<2 years), abnormal saccades may first manifest as slowing and shortening of upward saccades, long before gaze pa lsy onset. While visceral manifestations tend to predominate during the perina tal and infantile period (2 months-6 years of age), neurological and psychiatric involvement is more prominent during the juvenile/adult period (>6 years o f age). Psychosis in NP-C is atypical and variably responsive to treatment. Progre ssive cognitive decline, which always occurs in patients with NP-C, manifests as memory and executive impairment in juvenile/adult patients. Disease prognosis mai nly correlates with the age at onset of the neurological signs, with early-ons et forms progressing faster. Therefore, a detailed and descriptive picture of NP-C signs and symptoms may help improve disease detection and early diagnosis, so that therapy with miglustat (Zavesca((R))), the only available treatment a pproved to date, can be started as soon as neurological symptoms appear, in order to slow disease progression. FAU - Mengel, Eugen AU - Mengel E AD - Department of Lysosomal Storage Disorder, Villa Metabolica, Center for Pae diatric and Adolescent Medicine, University Medical Center of the Johannes Gutenbe

rg FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID SB PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMIS IS VI DP TI ome PG LID AB als oman carrying a homozygous mutation (p.A899T) in mitochondrial polymerase gamma (POLG) and manifesting with a complex neurological phenotype including Dopamine-a gonist University Mainz, Langenbeckstrasse 1, 55131 Mainz, Germany. mengel@kinder.klinik.uni-mainz.de. Klunemann, Hans-Hermann Klunemann HH Lourenco, Charles M Lourenco CM Hendriksz, Christian J Hendriksz CJ Sedel, Frederic Sedel F Walterfang, Mark Walterfang M Kolb, Stefan A Kolb SA eng Journal Article Research Support, Non-U.S. Gov't 20131017 England Orphanet J Rare Dis Orphanet journal of rare diseases 101266602 IM PMC3853996 NLM: PMC3853996 2013/10/19 06:00 2013/10/19 06:00 2013/10/19 06:00 2013/06/04 [received] 2013/10/01 [accepted] 2013/10/17 [aheadofprint] 1750-1172-8-166 [pii] 10.1186/1750-1172-8-166 [doi] epublish Orphanet J Rare Dis. 2013 Oct 17;8:166. doi: 10.1186/1750-1172-8-166. 24099403 NLM MEDLINE 20131115 20140106 1471-2350 (Electronic) 1471-2350 (Linking) 14 2013 Dopamine-agonist responsive Parkinsonism in a patient with the SANDO syndr

caused by POLG mutation. - 105 - 10.1186/1471-2350-14-105 [doi] - BACKGROUND: Disorders of oxidative phosphorylation affects 1/5000 individu and present heterogeneous involvement of tissues highly dependent upon ATP production. CASE PRESENTATION: Here we present the case of a 48-year-old w

responsive Parkinsonism. CONCLUSION: This case report is further evidence that mitochondrial dysfunction might play a role in Parkinson's Disease pathoge nesis and helps in identification of apparent mutation-specific clinical characteristics. Mutations in POLG should be looked for in cases of Parkin sonism, especially when multisystem neurological involvement is found. FAU - Bandettini di Poggio, Monica AU - Bandettini di Poggio M AD - Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Mater nal and Child Health, University of Genova and IRCSS Azienda Opedaliera Universita ria San Martino-IST, Largo Daneo 3-16132, Genova, Italy. monicabandettini@yahoo.it . FAU - Nesti, Claudia AU - Nesti C FAU - Bruno, Claudio AU - Bruno C FAU - Meschini, Maria Chiara AU - Meschini MC FAU - Schenone, Angelo AU - Schenone A FAU - Santorelli, Filippo M AU - Santorelli FM LA - eng PT - Case Reports PT - Journal Article DEP - 20131007 PL - England TA - BMC Med Genet JT - BMC medical genetics JID - 100968552 RN - 0 (Benzothiazoles) RN - 0 (Dopamine Agonists) RN - 0 (Thiophenes) RN - 83619PEU5T (pramipexole) RN - EC 2.7.7.- (POLG protein, human) RN - EC 2.7.7.7 (DNA-Directed DNA Polymerase) RN - O5TNM5N07U (duloxetine) RN - Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis SB - IM MH - Benzothiazoles/therapeutic use MH - DNA Mutational Analysis MH - DNA-Directed DNA Polymerase/*genetics/metabolism MH - Dopamine Agonists/*therapeutic use MH - Dysarthria/complications/*genetics/pathology MH - Female MH - Hereditary Sensory and Motor Neuropathy/complications/*genetics/pathology MH - Humans MH - Middle Aged MH - Mitochondria/enzymology MH - Ophthalmoplegia/complications/*genetics/pathology MH - Parkinson Disease/complications/*drug therapy MH - Thiophenes/therapeutic use MH - Tomography, Emission-Computed, Single-Photon PMC - PMC3851930 OID - NLM: PMC3851930 EDAT- 2013/10/09 06:00

MHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA IS IS VI IP DP TI ance

2014/01/07 06:00 2013/10/09 06:00 2013/03/05 [received] 2013/09/25 [accepted] 2013/10/07 [aheadofprint] 1471-2350-14-105 [pii] 10.1186/1471-2350-14-105 [doi] epublish BMC Med Genet. 2013 Oct 7;14:105. doi: 10.1186/1471-2350-14-105. 24081890 NLM In-Process 20131001 1576-6578 (Electronic) 0210-0010 (Linking) 57 8 2013 Oct 16 [CLIPPERS syndrome with atypical distribution of lesions in magnetic reson

imaging of the brain]. PG - 354-8 AB - INTRODUCTION: CLIPPERS syndrome (chronic lymphocytic inflammation with pon tine perivascular enhancement responsive to steroids) is an inflammatory proces s of the central nervous system whose distinguishing features are the enhancing punctiform lesions in the brainstem that appear in the magnetic resonance images. Clinically, it is accompanied by dysarthria, ataxia and diplopia, and usua lly responds to treatment with corticoids. Pathologically, T lymphocytes appea r infiltrated in the perivascular spaces of the brainstem. CASE REPORT: We r eport the case of a 40-year-old woman with an initial subacute clinical picture of binocular diplopia, ataxia and dysarthria. The magnetic resonance brain sc an revealed T2 hyperintense punctiform lesions in the stem, cerebellum, diencephalons and cortico-subcortical areas of both hemispheres, which wer e enhanced with contrast. An aetiological study was performed to rule out an y underlying infectious, neoplastic or inflammatory origin, the results bein g negative. The patient was treated on two occasions with methylprednisolone , with a gradual lowering of the dosage, the response being favourable. CONCLUSIO NS: Diplopia and ataxia, as in our case, are practically always present. The M R findings consist of punctiform enhancing lesions located in the pons exten ding towards the cerebellum, basal ganglia and corpus callosum, the enhancement gradient becoming lower as the distance increases rostrally away from the cortex, and caudally towards the spinal cord. In the case of our patient, this gra dient

is not respected, and the density found was similar to that of lesions at the supratentorial level. The differential diagnosis is wide-ranging and justi fies an extensive diagnostic study with, in certain cases, a biopsy study of brain tissue. The disease courses in a relapsing-remitting pattern and the earli er steroid therapy is established and the more prolonged it is, the better th e prognosis will be. FAU - Canneti, Beatrice AU - Canneti B AD - Hospital Universitario de la Princesa, 28006 Madrid, Espana. FAU - Mosqueira, Antonio J AU - Mosqueira AJ FAU - Gilo, Francisco AU - Gilo F FAU - Carreras, Teresa AU - Carreras T FAU - Barbosa, Antonio AU - Barbosa A FAU - Meca-Lallana, Virginia AU - Meca-Lallana V FAU - Vivancos, Jose AU - Vivancos J LA - spa PT - English Abstract PT - Journal Article TT - Sindrome CLIPPERS con distribucion atipica de las lesiones en la resonanci a magnetica cerebral. PL - Spain TA - Rev Neurol JT - Revista de neurologia JID - 7706841 SB - IM OAB - Publisher: Abstract available from the publisher. EDAT- 2013/10/02 06:00 MHDA- 2013/10/02 06:00 CRDT- 2013/10/02 06:00 AID - rn2013126 [pii] PST - ppublish SO - Rev Neurol. 2013 Oct 16;57(8):354-8. PMIDOWN STATDA IS IS VI DP TI 24067156 NLM In-Process 20131115 1471-2377 (Electronic) 1471-2377 (Linking) 13 2013 Unusual epileptic deterioration and extensive white matter lesion during treatment in Wilson's disease. PG - 127 LID - 10.1186/1471-2377-13-127 [doi] AB - BACKGROUND: Wilson's disease (WD) is a genetic disorder which can be contr olled fairly well with decupuration therapy. However, symptoms, on rare occasion s, can

worsen even when WD is being treated. Herein, we report a case involving u nusual neurological deterioration during decupuration therapy for WD. CASE PRESEN TATION: A 28-year-old man was diagnosed with WD 13 years prior to his clinical vis it; however, his drug compliance has been poor over the years. He was treated with trientine because tremors and dysarthria have presented in recent years. H owever, dysarthria and dystonia developed in his limbs, which were worse on the ri ght side and had been aggravated for several weeks despite good drug complianc e. His symptoms were fluctuating. It was initially misdiagnosed as dystonia; alth ough, it turned out to be a seizure due to cortical degeneration. These symptoms were completely resolved with antiepileptic drugs. Moreover, the cortical enhan cement of bifrontal degeneration has disappeared on the MRI. CONCLUSION: This cas e showed unusual epileptic neurologic deterioration due to cortical degenera tion during decupuration therapy. Seizures in WD can easily be mistaken as part of dystonia. However, the fluctuating symptoms suggest a seizure. FAU - Kim, Young Eun AU - Kim YE AD - Department of Neurology, Ulsan University Hospital, Ulsan, Korea. brain@snu.ac.kr. FAU - Yun, Ji Young AU - Yun JY FAU - Yang, Hui-Jun AU - Yang HJ FAU - Kim, Han-Joon AU - Kim HJ FAU - Jeon, Beom S AU - Jeon BS LA - eng PT - Journal Article DEP - 20130925 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 SB - IM PMC - PMC3851542 OID - NLM: PMC3851542 EDAT- 2013/09/27 06:00 MHDA- 2013/09/27 06:00 CRDT- 2013/09/27 06:00 PHST- 2012/09/21 [received] PHST- 2013/09/03 [accepted] PHST- 2013/09/25 [aheadofprint] AID - 1471-2377-13-127 [pii] AID - 10.1186/1471-2377-13-127 [doi] PST - epublish SO - BMC Neurol. 2013 Sep 25;13:127. doi: 10.1186/1471-2377-13-127.

PMIDOWN STATDA DCOMIS IS VI IP DP TI ing

23812076 NLM MEDLINE 20130701 20140228 1349-7413 (Electronic) 0911-4300 (Linking) 36 3 2013 [Diagnostic value of brain biopsy in a pediatric multiple sclerosis mimick

brain stem glioma]. PG - 175-9 AB - Diagnosis of multiple sclerosis (MS) is difficult when the lesion mimics g lioma or cerebral enchephalitis. We report a case of pediatric MS initially susp ected as brain stem glioma. An 11-year-old boy developed left foot joint pain fo llowed by progressive symptoms such as left arm and leg weakness, dysarthria, paraplegia, and decreased level of consciousness. He subsequently develope d respiratory distress requiring endotracheal intubation and mechanical ventilation. Magnetic resonance imaging showed a mass measuring 2 cm in th e medulla oblongata. Although this mass was initially suspected as a glioma, the patient's acutely progressive disease course was not consistent with this diagnosis. Open biopsy revealed inflammation and demyelination, but no mal ignant cells were detected. He was treated with steroid pulse therapy, which show ed dramatic effects. Nine months later, he developed another episode characte rized by several neurological symptoms, and the diagnosis of MS was clinically confirmed. Open brain stem biopsy is technically demanding, but this case demonstrates that appropriate neurosurgical evaluation can play an importa nt role in diagnosis by ruling out glioma and confirming MS. FAU - Nakazawa, Yumiko AU - Nakazawa Y AD - Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development. FAU - Maekawa, Takanobu AU - Maekawa T FAU - Oana, Shinji AU - Oana S FAU - Ishiguro, Akira AU - Ishiguro A FAU - Ohta, Sayaka AU - Ohta S FAU - Terashima, Hiroshi AU - Terashima H FAU - Kashii, Hirofumi AU - Kashii H FAU - Kubota, Masaya AU - Kubota M FAU - Tsutsumi, Yoshiyuki

AU FAU AU FAU AU FAU AU LA PT PT PT PL TA JT gy JID SB MH MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMIS IS VI DP TI f the

Tsutsumi Y Nakazawa, Atsuko Nakazawa A Morota, Nobuhito Morota N Sakai, Hirokazu Sakai H jpn Case Reports English Abstract Journal Article Japan Nihon Rinsho Meneki Gakkai Kaishi Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunolo 9505992 IM Biopsy Brain/*pathology Brain Stem Neoplasms/*pathology Child *Diagnosis, Differential Glioma/*pathology Humans Male Multiple Sclerosis/*pathology 2013/07/03 06:00 2014/03/01 06:00 2013/07/02 06:00 DN/JST.JSTAGE/jsci/36.175 [pii] ppublish Nihon Rinsho Meneki Gakkai Kaishi. 2013;36(3):175-9. 23742248 NLM MEDLINE 20130626 20140102 1750-1172 (Electronic) 1750-1172 (Linking) 8 2013 Biotin-responsive basal ganglia disease should be renamed biotin-thiamine-responsive basal ganglia disease: a retrospective review o

clinical, radiological and molecular findings of 18 new cases. PG - 83 LID - 10.1186/1750-1172-8-83 [doi] AB - BACKGROUND: Biotin-responsive basal ganglia disease (BBGD) is an autosomal recessive neurometabolic disorder. It is characterized by sub acute encephalopathy with confusion, seizure, dysarthria and dystonia following a history of febrile illness. If left untreated with biotin, the disease can progress to severe quadriparesis and even death. METHOD: A retrospective c hart review of 18 patients with BBGD from two tertiary institutions describing their clinical, magnetic resonance imaging and molecular findings was conducted. RESULT: Eighteen children from 13 families seen over a period of nine year s

(2003-2012) were included. (Age range: 14month to 23 years, M: F: 1:1). Th e clinical features included sub acute encephalopathy, ataxia (n= 18), seizu res (n= 13) dystonia (n=12) ,dysarthria (n= 9), quadriparesis and hyperreflexia (n =9). Magnetic resonance imaging demonstrated abnormal signal intensity with swe lling in the basal ganglia during acute crises (n= 13/13) and atrophy of the bas al ganglia and necrosis during follow up (n= 13/13). One-third of the present patients showed the recurrence of acute crises while on biotin therapy alo ne, but after the addition of thiamine, crises did not recur. All of the patients have a homozygous missense mutation in exon 5 of the SLC19A3 gene. The frequency of acute crises, delay in diagnosis and initiation of treatment significantly influenced the outcome. On follow up, four patients died, two had spastic quadriplegia, six had normal outcome and the rest had speech and motor dysfunctions. CONCLUSION: Clinicians should suspect BBGD in any child pres enting with sub acute encephalopathy, abnormal movement and MRI findings as descr ibed above. Both biotin and thiamine are essential for disease management. Sinc e biotin alone could not prevent the recurrence of crises in some patients, a more appropriate term to describe the disease would be biotin-thiamine-responsi ve basal ganglia disease (BTBGD). FAU - Alfadhel, Majid AU - Alfadhel M AD - Division of Genetics, Department of Pediatrics, King Abdulaziz Medical Cit y, Riyadh, Saudi Arabia. dralfadhel@yahoo.com FAU - Almuntashri, Makki AU - Almuntashri M FAU - Jadah, Raafat H AU - Jadah RH FAU - Bashiri, Fahad A AU - Bashiri FA FAU - Al Rifai, Muhammad Talal AU - Al Rifai MT FAU - Al Shalaan, Hisham AU - Al Shalaan H FAU - Al Balwi, Mohammed AU - Al Balwi M FAU - Al Rumayan, Ahmed AU - Al Rumayan A FAU - Eyaid, Wafaa AU - Eyaid W FAU - Al-Twaijri, Waleed AU - Al-Twaijri W LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20130606 PL - England

TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (Membrane Transport Proteins) RN - 0 (SLC19A3 protein, human) RN - 6SO6U10H04 (Biotin) RN - X66NSO3N35 (Thiamine) RN - Basal ganglia disease, biotin-responsive SB - IM MH - Adolescent MH - Adult MH - Basal Ganglia/pathology MH - Basal Ganglia Diseases/*classification/*drug therapy/physiopathology/radio graphy MH - Biotin/therapeutic use MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Infant MH - Magnetic Resonance Imaging MH - Male MH - Membrane Transport Proteins/genetics MH - Mutation MH - Thiamine/*therapeutic use MH - Wernicke Encephalopathy/drug therapy/physiopathology/radiography MH - Young Adult PMC - PMC3691666 OID - NLM: PMC3691666 EDAT- 2013/06/08 06:00 MHDA- 2014/01/03 06:00 CRDT- 2013/06/08 06:00 PHST- 2013/02/14 [received] PHST- 2013/05/21 [accepted] PHST- 2013/06/06 [aheadofprint] AID - 1750-1172-8-83 [pii] AID - 10.1186/1750-1172-8-83 [doi] PST - epublish SO - Orphanet J Rare Dis. 2013 Jun 6;8:83. doi: 10.1186/1750-1172-8-83. PMIDOWN STATDA DCOMLR IS VI DP TI e 23734128 NLM PubMed-not-MEDLINE 20130604 20130605 20130813 1663-9812 (Electronic) 4 2013 Burden of Friedreich's Ataxia to the Patients and Healthcare Systems in th

United States and Canada. PG - 66 LID - 10.3389/fphar.2013.00066 [doi] AB - Objective: The study intended to substantiate healthcare resource utilizat ion, costs, and funding patterns of US and Canadian Friedreich's Ataxia (FRDA) populations, to assess compliance with treatment guidance and to identify areas where novel healthcare measures or improved access to existing care may im

prove patients' functional and social capabilities and reduce the financial impa ct on the healthcare systems. Methods: Healthcare resource utilization and costs were collected in a cross-sectional study in the US (N = 197) and Canada (N = 4 3) and analyzed across severity of disease categories. Descriptive statistics, correlation analysis, and hypothesis testing were applied. Results: In the US, healthcare costs of FRDA patients were higher than those of "adults with t wo and more chronic conditions." Significantly higher costs were incurred in adva nced stages of the disease, with paid homecare being the main driver. This patt ern was also observed in Canada. Compliance with the recommended annual neurologic al and cardiological follow-up was high, but was low for the recommended regular speech therapy. In the US public and private funding ratios were similar for the FRDA and the general populations. In Canada the private funding ratio for FRDA was higher than average. Conclusion: The variety of healthcare measures addres sing the broad range of symptoms of FRDA, and the increasing use of paid home c are as disease progresses made total US healthcare costs of FRDA exceed the costs of US adults with two and more chronic conditions. Therefore, measures delaying disease progression will allow patients to maintain their independence longer and may reduce costs to the healthcare system. Novel measures to address dysarthri a and to ensure access to them should be further investigated. The higher than a verage private funding ratio in Canada was due to the relatively high cost of the pharmacological treatment of FRDA. FAU - Polek, Barbara AU - Polek B AD - Santhera Pharmaceuticals Ltd Liestal, Switzerland. FAU - Roach, M J AU - Roach MJ FAU - Andrews, William T AU - Andrews WT FAU - Ehling, Manfred AU - Ehling M FAU - Salek, Sam AU - Salek S LA - eng PT - Journal Article DEP - 20130522 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC3660667 OID - NLM: PMC3660667

OTO OT OT OT OT OT OT EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO ion PMIDOWN STATDA DCOMLR IS IS VI IP DP TI (110

NOTNLM Friedreich ataxia cost of illness cross-sectional studies healthcare resource rare diseases resource utilization 2013/06/05 06:00 2013/06/05 06:01 2013/06/05 06:00 2013 [ppublish] 2013/03/06 [received] 2013/04/29 [accepted] 2013/05/22 [epublish] 10.3389/fphar.2013.00066 [doi] epublish Front Pharmacol. 2013 May 22;4:66. doi: 10.3389/fphar.2013.00066. eCollect 2013. 23687505 NLM PubMed-not-MEDLINE 20130520 20130521 20130522 1664-5456 (Electronic) 1664-5456 (Linking) 3 1 2013 Jan Increase in the Size of an Intracardiac Thrombus during Dabigatran Therapy

mg b.i.d.) in an Acute Cardioembolic Stroke Patient. PG - 78-80 LID - 10.1159/000351137 [doi] AB - We report a case of atrial fibrillation in a patient in whom a mobile thro mbus in the left atrial appendage increased in size after low-dose dabigatran ther apy. A 74-year-old man was admitted to our hospital because of sudden onset of ri ght hemiplasia and dysarthria. On admission, his National Institutes of Health Stroke Scale score was three. Axial diffusion-weighted magnetic resonance images and magnetic resonance angiography images showed hyperintense signals in the l eft front-parietal cerebral cortex without any intracranial stenotic lesions, and acute cardioembolic stroke associated with nonvalvular atrial fibrillation was diagnosed. Transesophageal echocardiography revealed a mobile thrombosis ( 1.0 x 2.2 cm) in the left atrial appendage, and dabigatran therapy (110 mg b.i.d .) was initiated to prevent stroke recurrence. Transesophageal echocardiography performed 6 days later revealed that the size of the thrombus had increase d to 1.5 x 3.0 cm. Medication was changed to warfarin, and the thrombosis subse

quently decreased in size. The patient did not have a recurrent stroke and was dis charged with a National Institutes of Health Stroke Scale score of zero. This case demonstrates that low-dose dabigatran may not be effective in reducing the size of a thrombus. FAU - Tabata, Emi AU - Tabata E AD - Department of Cerebrovascular Medicine and Clinical Research Institute, Na tional Hospital Organization Kyushu Medical Center, Fukukuoka, Japan. FAU - Yasaka, Masahiro AU - Yasaka M FAU - Wakugawa, Yoshiyuki AU - Wakugawa Y FAU - Komori, Motohiro AU - Komori M FAU - Mori, Kohta AU - Mori K FAU - Tsurusaki, Yuichiro AU - Tsurusaki Y FAU - Kokuba, Kazuhito AU - Kokuba K FAU - Sambongi, Yoshiki AU - Sambongi Y FAU - Maeda, Koichiro AU - Maeda K FAU - Okada, Yasushi AU - Okada Y LA - eng PT - Journal Article DEP - 20130503 PL - Switzerland TA - Cerebrovasc Dis Extra JT - Cerebrovascular diseases extra JID - 101577885 PMC - PMC3656689 OID - NLM: PMC3656689 OTO - NOTNLM OT - Acute cardioembolic stroke OT - Dabigatran OT - Intracardiac thrombus OT - Nonvalvular atrial fibrillation OT - Transesophageal echocardiography EDAT- 2013/05/21 06:00 MHDA- 2013/05/21 06:01 CRDT- 2013/05/21 06:00 PHST- 2013 [ppublish] PHST- 2013/05/03 [epublish] AID - 10.1159/000351137 [doi] AID - cee-0003-0078 [pii] PST - epublish SO - Cerebrovasc Dis Extra. 2013 May 3;3(1):78-80. doi: 10.1159/000351137. Prin t 2013 Jan. PMID- 23640737 OWN - NLM STAT- MEDLINE

DA - 20130503 DCOM- 20130702 LR - 20140319 IS - 1097-0142 (Electronic) IS - 0008-543X (Linking) VI - 118 IP - 23 DP - 2012 Dec 1 TI - Late dysphagia after radiotherapy-based treatment of head and neck cancer. PG - 5793-9 LID - 10.1002/cncr.27631 [doi] AB - BACKGROUND: Changing trends in head and neck cancer (HNC) merit an underst anding of the late effects of therapy, but few studies examine dysphagia beyond 2 years of treatment. METHODS: A case series was examined to describe the pathophy siology and outcomes in dysphagic HNC survivors referred for modified barium swall ow (MBS) studies >/= 5 years after definitive radiotherapy or chemoradiothera py (January 2001 through May 2011). Functional measures included the penetration-aspiration scale (PAS), performance status scale-head and neck (PSS-HN), National Institutes of Health Swallowing Safety Scale (NIH-SSS), and MBS impairment profile (MBSImp). RESULTS: Twenty-nine patients previously treated with radiotherapy (38%) or chemoradiotherapy (62%) were included (median y ears posttreatment, 9; range, 5-19). The majority (86%) had oropharyngeal cance r; 52% were never-smokers. Seventy-five percent had T2 or T3 tumors; 52% were N+. The median age at diagnosis was 55 (range, 38-72). Abnormal late examination f indings included: dysarthria/dysphonia (76%), cranial neuropathy (48%), trismus (3 8%), and radionecrosis (10%). MBS studies confirmed pharyngeal residue and aspi ration in all dysphagic cases owing to physiologic impairment (median PAS, 8; med ian NIH-SSS, 10; median MBSImp, 18), whereas stricture was confirmed endoscopi cally in 7 (24%). Twenty-five (86%) developed pneumonia, half requiring hospitalization. Swallow postures/strategies helped 69% of cases, but no p atient achieved durable improvement across functional measures at last follow-up. Ultimately, 19 (66%) were gastrostomy-dependent. CONCLUSIONS: Although fun ctional organ preservation is commonly achieved, severe dysphagia represents a challenging late effect that may develop or progress years after radiation -based therapy for HNC. These data suggest that novel approaches are needed to mi nimize and better address this complication that is commonly refractory to many s tandard dysphagia therapies. CI - Copyright (c) 2012 American Cancer Society. FAU - Hutcheson, Katherine A AU - Hutcheson KA

AD - Department of Head and Neck Surgery, The University of Texas MD Anderson C ancer Center, Houston, Texas 77030, USA. karnold@mdanderson.org FAU - Lewin, Jan S AU - Lewin JS FAU - Barringer, Denise A AU - Barringer DA FAU - Lisec, Asher AU - Lisec A FAU - Gunn, G Brandon AU - Gunn GB FAU - Moore, Michael W S AU - Moore MW FAU - Holsinger, F Christopher AU - Holsinger FC LA - eng GR - P30 CA016672/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120517 PL - United States TA - Cancer JT - Cancer JID - 0374236 SB - AIM SB - IM MH - Adult MH - Aged MH - Deglutition Disorders/diagnosis/*etiology MH - Female MH - Head and Neck Neoplasms/*radiotherapy MH - Humans MH - Male MH - Middle Aged MH - Radiation Injuries/diagnosis/*etiology MH - Retrospective Studies MH - Treatment Outcome EDAT- 2013/05/04 06:00 MHDA- 2013/07/03 06:00 CRDT- 2013/05/04 06:00 PHST- 2012/01/23 [received] PHST- 2012/03/27 [revised] PHST- 2012/04/02 [accepted] PHST- 2012/05/17 [aheadofprint] AID - 10.1002/cncr.27631 [doi] PST - ppublish SO - Cancer. 2012 Dec 1;118(23):5793-9. doi: 10.1002/cncr.27631. Epub 2012 May 17. PMIDOWN STATDA DCOMLR IS IS VI IP DP 23559290 NLM MEDLINE 20130405 20130930 20131112 0717-6163 (Electronic) 0034-9887 (Linking) 140 10 2012 Oct

TI - [Posterior reversible encephalopathy as the first manifestation of Guillai n-Barre syndrome. Report of one case]. PG - 1316-20 LID - 10.4067/S0034-98872012001000012 [doi] LID - S0034-98872012001000012 [pii] AB - BACKGROUND: We report a 56 year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifest ation of Guillain-Barre syndrome (GBS). His first symptoms were right hemiparesi s and hemihypoesthesia, followed by headache, dizziness, dysarthria and a genera l feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an ax onal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS . Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an importan t clinical and imaging remission of PRES, but maintaining tetraparesis durin g the hospitalization. Twelve months after discharge and regular motor rehabilit ation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulatin g pro-inflammatory cytokines in GBS may be the cause of a breach in the bloo d-brain barrier, thus causing PRES, that can completely remit with an adequate management. FAU - Urrutia L, Sergio AU - Urrutia L S AD - Unidad de Neurologia Adultos, Hospital Clinico FUSAT, Rancagua, Chile. drsergiourrutia@gmail.com FAU - Venegas R, Eduardo AU - Venegas R E FAU - Figueroa V, Cristian AU - Figueroa V C FAU - Carrizo C, Catalina AU - Carrizo C C LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Encefalopatia posterior reversible como primera manifestacion del Sindrome de Guillain-Barre PL - Chile TA - Rev Med Chil JT - Revista medica de Chile JID - 0404312

SB MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTPHSTAID AID PST SO 12. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI le

IM Diagnosis, Differential Guillain-Barre Syndrome/*complications/diagnosis Humans Hypertension/*complications Magnetic Resonance Imaging Male Middle Aged Posterior Leukoencephalopathy Syndrome/diagnosis/*etiology 2013/04/06 06:00 2013/10/01 06:00 2013/04/06 06:00 2011/03/14 [received] 2011/05/17 [accepted] S0034-98872012001000012 [pii] 10.4067/S0034-98872012001000012 [doi] ppublish Rev Med Chil. 2012 Oct;140(10):1316-20. doi: 10.4067/S0034-988720120010000 23475537 NLM MEDLINE 20130430 20130617 20131121 1465-3621 (Electronic) 0368-2811 (Linking) 43 5 2013 May Anti-Yo antibody-mediated paraneoplastic cerebellar degeneration in a fema

patient with pleural malignant mesothelioma. PG - 563-8 LID - 10.1093/jjco/hyt031 [doi] AB - Paraneoplastic cerebellar degeneration is a rare non-metastatic complicati on of malignancies. It presents with acute or subacute onset of ataxia, dysarthr ia and intention tremor. Paraneoplastic cerebellar degeneration is most commonly associated with malignancies of the ovary, breast and lung. The anti-Yo (anti-Purkinje cells) antibodies that specifically damage the Purkinje cel ls of the cerebellum are found in the serum and cerebrospinal fluid. Anti-Yo-rel ated paraneoplastic cerebellar degeneration is most commonly found in women wit h gynecological and breast cancers, but it is reported in other malignancies . Patients with paraneoplastic syndromes most often present with neurologic symptoms before an underlying cancer is detected. We report a case of anti-Yo-related paraneoplastic cerebellar degeneration associated with ple ural malignant mesothelioma in a 51-year-old female patient. She presented to o ur department with a 2-week history after the last chemotherapy of progressiv e dizziness related to head movement, nausea, vomiting, ataxia and unsteady gait. A

western blot assay was negative for anti-Hu, anti-Ri, anti-Ma2, anti-CV2 a nd anti-amphiphysin paraneoplastic antibody markers but positive for anti-Yo. In conclusion, we report a case of paraneoplastic cerebellar degeneration in a patient with pleural malignant mesothelioma because of the rarity of this neurologic presentation after the diagnosis of malignant mesothelioma and of the association with anti-Yo antibodies. FAU - Tanriverdi, Ozgur AU - Tanriverdi O AD - Department of Medical Oncology, Mugla Sitki Kocman University Education an d Research Hospital, Mugla, Turkey. ozgurtanriverdi@hotmail.com FAU - Meydan, Nezih AU - Meydan N FAU - Barutca, Sabri AU - Barutca S FAU - Ozsan, Nazan AU - Ozsan N FAU - Gurel, Duygu AU - Gurel D FAU - Veral, Ali AU - Veral A LA - eng PT - Case Reports PT - Journal Article DEP - 20130308 PL - England TA - Jpn J Clin Oncol JT - Japanese journal of clinical oncology JID - 0313225 RN - 0 (CDR2 protein, human) RN - 0 (Nerve Tissue Proteins) SB - IM MH - Female MH - Humans MH - Mesothelioma/*complications/diagnosis MH - Middle Aged MH - Multimodal Imaging MH - Nerve Tissue Proteins/*immunology MH - Paraneoplastic Cerebellar Degeneration/*diagnosis/drug therapy/*immunology MH - Pleural Neoplasms/*complications/diagnosis MH - Positron-Emission Tomography MH - Tomography, X-Ray Computed EDAT- 2013/03/12 06:00 MHDA- 2013/06/19 06:00 CRDT- 2013/03/12 06:00 PHST- 2013/03/08 [aheadofprint] AID - hyt031 [pii] AID - 10.1093/jjco/hyt031 [doi] PST - ppublish SO - Jpn J Clin Oncol. 2013 May;43(5):563-8. doi: 10.1093/jjco/hyt031. Epub 201 3 Mar 8. PMID- 23468819 OWN - NLM STAT- MEDLINE

DA DCOMLR IS IS VI IP DP TI -

20130307 20131122 20131203 1866-0452 (Electronic) 1866-0452 (Linking) 110 7 2013 Feb Who receives rehabilitation after stroke?: Data from the quality assurance project "Stroke Register Northwest Germany". PG - 101-7 LID - 10.3238/arztebl.2013.0101 [doi] AB - BACKGROUND: Neurological rehabilitation after stroke lowers rates of death , dependency, and institutionalization. Little research has yet addressed th e factors affecting the selection of ischemic stroke patients for rehabilita tive treatment. METHOD: The database for this study consisted of all cases of i schemic stroke (ICD-10 code I63) that occurred in 2010 and 2011 in the neurologica l inpatient care facilities participating in the "Stroke Register Northwest Germany" quality assurance project. A primary target group for rehabilitat ion was defined a priori (Barthel Index at discharge </= 65, no premorbid nursing dependency, no transfer to another acute-care hospital after initial treat ment of stroke). Among these patients, factors potentially affecting the provision of rehabilitative treatment were studied with binary logistic regression and multilevel logistic regression. RESULTS: There were 96 955 cases of ischem ic stroke in the 127 participating hospitals. 40.8% and 11.4% of these patien ts underwent neurological and geriatric rehabilitation, respectively. The pri mary target group for rehabilitation contained 14 486 patients, 14.9% of whom underwent no rehabilitation after their acute treatment. The chances of undergoing subsequent rehabilitation were higher for patients with paresis and dysarthria on admission. Female sex, older age, impaired consciousness at admission, prior history of stroke, and lack of counseling by the hospital social services were all associated with a lower probability of undergoing rehabilitation. CONCLUSION: In this study, 54.4% of all ischemic stroke pa tients and 85.1% of all patients in a primary target group for rehabilitation tha t was defined a priori underwent rehabilitation after acute care for stroke. Old er patients and those who had had a previous stroke were less likely to under go rehabilitation. Counseling by hospital social services increased the proba bility of rehabilitation. The potential exclusion of stroke patients from rehabil itation because of old age should be critically examined in every relevant case. FAU - Unrath, Michael AU - Unrath M

AD - Institute of Epidemiology and Social Medicine, University of Munster, Germ any. unrathm@uni-muenster.de FAU - Kalic, Marianne AU - Kalic M FAU - Berger, Klaus AU - Berger K LA - eng PT - Journal Article DEP - 20130215 PL - Germany TA - Dtsch Arztebl Int JT - Deutsches Arzteblatt international JID - 101475967 SB - IM CIN - Dtsch Arztebl Int. 2013 Jun;110(26):459. PMID: 23885282 CIN - Dtsch Arztebl Int. 2013 Jun;110(26):459. PMID: 23885281 MH - Age Distribution MH - Aged MH - Aged, 80 and over MH - Comorbidity MH - Directive Counseling/*utilization MH - Female MH - Germany/epidemiology MH - Health Care Rationing/*utilization MH - Humans MH - Male MH - Middle Aged MH - Nervous System Diseases/*epidemiology/rehabilitation MH - *Registries MH - Rehabilitation/*statistics & numerical data MH - Sex Distribution MH - Stroke/*epidemiology/*rehabilitation PMC - PMC3585451 OID - NLM: PMC3585451 EDAT- 2013/03/08 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/03/08 06:00 PHST- 2012/06/08 [received] PHST- 2012/11/15 [accepted] PHST- 2013/02/15 [epublish] AID - 10.3238/arztebl.2013.0101 [doi] PST - ppublish SO - Dtsch Arztebl Int. 2013 Feb;110(7):101-7. doi: 10.3238/arztebl.2013.0101. Epub 2013 Feb 15. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI kers 23464033 NLM MEDLINE 20130307 20130822 20140320 1520-8524 (Electronic) 0001-4966 (Linking) 133 3 2013 Mar Relative fundamental frequency during vocal onset and offset in older spea

with and without Parkinson's disease. PG - 1637-43 LID - 10.1121/1.4776207 [doi] AB - The relative fundamental frequency (RFF) surrounding production of a voice less consonant has previously been shown to be lower in speakers with hypokinet ic dysarthria and Parkinson's disease (PD) relative to age/sex matched contro ls. Here RFF was calculated in 32 speakers with PD without overt hypokinetic dysarthria and 32 age and sex matched controls to better understand the relationships between RFF and PD progression, medication status, and sex. Results showed that RFF was statistically significantly lower in individuals with PD compared with healthy age-matched controls and was statistically significa ntly lower in individuals diagnosed at least 5 yrs prior to experimentation rel ative to individuals recorded less than 5 yrs past diagnosis. Contrary to previo us trends, no effect of medication was found. However, a statistically signif icant effect of sex on offset RFF was shown, with lower values in males relative to females. Future work examining the physiological bases of RFF is warranted . FAU - Stepp, Cara E AU - Stepp CE AD - Department of Speech, Language, and Hearing Sciences, Boston University, B oston, Massachusetts 02215, USA. cstepp@bu.edu LA - eng GR - 5T32HD007424/HD/NICHD NIH HHS/United States GR - H133P080008/PHS HHS/United States GR - T32 DC000038/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - J Acoust Soc Am JT - The Journal of the Acoustical Society of America JID - 7503051 RN - 0 (Antiparkinson Agents) SB - IM MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Antiparkinson Agents/therapeutic use MH - Biomechanical Phenomena MH - Case-Control Studies MH - Female MH - Humans MH - Linear Models MH - Male MH - Middle Aged MH - Parkinson Disease/*complications/drug therapy/physiopathology MH - *Phonation MH - Sex Factors MH - Sound Spectrography

MH MH MH MH MH MH PMC OID EDATMHDACRDTAID PST SO -

*Speech Acoustics Speech Disorders/*etiology/physiopathology Speech Production Measurement Time Factors Vocal Cords/*physiopathology *Voice Quality PMC3606308 NLM: PMC3606308 2013/03/08 06:00 2013/08/24 06:00 2013/03/08 06:00 10.1121/1.4776207 [doi] ppublish J Acoust Soc Am. 2013 Mar;133(3):1637-43. doi: 10.1121/1.4776207.

PMID- 23440537 OWN - NLM STAT- MEDLINE DA - 20130226 DCOM- 20140207 IS - 1578-2735 (Electronic) IS - 1139-9287 (Linking) VI - 41 IP - 1 DP - 2013 Jan-Feb TI - [Olanzapine long-acting post-injection syndrome: a case report and brief r eview]. PG - 60-2 AB - The introduction of long-acting injectable atypical antipsychotics has ens ured adherence to treatment in patients with low awareness of the disorder, wit h an acceptable rate of side effects. In the case of long acting olanzapine inj ection in particular, has particular relevance the existence of a special side-ef fect called post-injection syndrome. This rare side effect consisting in the pr esence of symptoms of olanzapine overdose after intramuscular administration of medication has led to restrictions on the use of the drug and the need for patient observation for three hours after each injection. We report a case of postinjection syndrome, to our knowledge, the first in Spain since the commercialization of Zypadhera. As in most cases described in the literatu re have symptoms of overdosage of olanzapine (dysarthria, sedation, fatigue, etc.) that are selflimiting without any therapeutic measure and are accompanied by supratherapeutic plasma levels of olanzapine. FAU - Duran-Sindreu, Santiago F AU - Duran-Sindreu SF AD - Servicio de Psiquiatria del Hospital de la Santa Creu i Sant Pau Barcelona , Espana. Sdurant@santpau.cat FAU - Grasa-Bello, Eva AU - Grasa-Bello E FAU - Corripio-Collado, Illuminada AU - Corripio-Collado I FAU - Sauras-Quetcuti, Rosa B AU - Sauras-Quetcuti RB

FAU - Keymer-Gausset, Alejandro AU - Keymer-Gausset A FAU - Roldan-Bejarano, Alexandra AU - Roldan-Bejarano A FAU - Alonso-Solis, Anna AU - Alonso-Solis A FAU - Figueras-Vilalta, Maria AU - Figueras-Vilalta M FAU - Alvarez-Martinez, Enric AU - Alvarez-Martinez E LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Sindrome post-inyeccion por olanzapina de liberacion retardada: Breve revi sion a proposito de un caso. DEP - 20130101 PL - Spain TA - Actas Esp Psiquiatr JT - Actas espanolas de psiquiatria JID - 100886502 RN - 0 (Antipsychotic Agents) RN - 12794-10-4 (Benzodiazepines) RN - 132539-06-1 (olanzapine) SB - IM MH - Antipsychotic Agents/administration & dosage/*adverse effects MH - Benzodiazepines/administration & dosage/*adverse effects MH - Humans MH - Injections, Intramuscular MH - Male MH - Middle Aged MH - Syndrome EDAT- 2013/02/27 06:00 MHDA- 2014/02/08 06:00 CRDT- 2013/02/27 06:00 PHST- 2013/01/01 [received] PHST- 2013/01/01 [accepted] PHST- 2013/01/01 [aheadofprint] PST - ppublish SO - Actas Esp Psiquiatr. 2013 Jan-Feb;41(1):60-2. Epub 2013 Jan 1. PMID- 23430551 OWN - NLM STAT- PubMed-not-MEDLINE DA - 20130222 DCOM- 20130225 LR - 20130415 IS - 2192-8304 (Print) IS - 2192-8304 (Linking) VI - 9 DP - 2013 TI - The mild form of menkes disease: a 34 year progress report on the original case. PG - 81-4 LID - 10.1007/8904_2012_183 [doi] AB - Classical Menkes disease is a neurodegenerative disorder caused by mutatio ns in the copper-transporting ATPase ATP7A gene which, when untreated, is usuall y fatal

in early childhood. A mild form of Menkes disease was originally reported in 1981 and clinical progress of the patient at 10 years described subsequently. T he causative mutation is c.4085C>T in exon 21, causing an alanine to valine substitution in the highly conserved TM7 domain at the C-terminal end of t he Menkes protein. Here we report his status at 34 years of age. Intellectual impairment is mild. Ataxia has nearly resolved but motor retardation, dysa rthria and an extreme slow speech rate remain. In contrast to patients with the occipital horn syndrome, there have been no connective tissue complication s of his mild Menkes disease. He has been under long-term copper therapy for mo re than 30 years and he continues to enjoy a good quality of life. FAU - Tchan, M C AU - Tchan MC AD - Department of Genetic Medicine, Westmead Hospital, 2145, Sydney, Australia , michel.tchan@health.nsw.gov.au. FAU - Wilcken, B AU - Wilcken B FAU - Christodoulou, J AU - Christodoulou J LA - eng PT - Journal Article DEP - 20121013 PL - Germany TA - JIMD Rep JT - JIMD reports JID - 101568557 PMC - PMC3565640 OID - NLM: PMC3565640 EDAT- 2013/02/23 06:00 MHDA- 2013/02/23 06:01 CRDT- 2013/02/23 06:00 PHST- 2012/08/10 [received] PHST- 2012/09/18 [accepted] PHST- 2012/09/17 [revised] PHST- 2012/10/13 [aheadofprint] AID - 10.1007/8904_2012_183 [doi] PST - ppublish SO - JIMD Rep. 2013;9:81-4. doi: 10.1007/8904_2012_183. Epub 2012 Oct 13. PMID- 23430549 OWN - NLM STAT- PubMed-not-MEDLINE DA - 20130222 DCOM- 20130225 LR - 20130418 IS - 2192-8304 (Print) IS - 2192-8304 (Linking) VI - 9 DP - 2013 TI - Ceftriaxone for Alexander's Disease: A Four-Year Follow-Up. PG - 67-71 LID - 10.1007/8904_2012_180 [doi] AB - In 2010, we reported the successful clinical outcome related to a 20-month course

of intravenous, cyclical ceftriaxone, in a patient with adult-onset Alexan der's disease. We now provide evidence that the progression of the patient's signs/symptoms was halted and reversed with a 4-year-long extension of the trial.The patient's clinical signs/symptoms were evaluated before the star t and every 6 months for 6 years. For the early 2 years, without therapy, and fo r the following 4 years, after intravenous ceftriaxone 2 g daily, for 3 weeks mo nthly during the initial 4 months, then for 15 days monthly.Gait ataxia and dysa rthria were assessed clinically on a 0 to 4 scale. Palatal myoclonus and nystagmus/oscillopsia were monitored by videotape and a self-evaluation sc ale. The degree of disability, measured by a modified Rankin scale, and the bra in MRI were periodically evaluated.Before ceftriaxone therapy, in a 2-year period , gait ataxia and dysarthria worsened from mild to marked, palatal myoclonus spre ad from the soft palate to lower facial muscles, and the patient complained of oscillopsia. After 4 years of ceftriaxone therapy, gait ataxia and dysarth ria improved, from marked to mild at clinical rating scales. The palatal myocl onus was undetectable; the patient did not complained of oscillopsia and declar ed a progressively better quality of life. Ceftriaxone was safe.This case repor t provides Class IV evidence that intravenous cycles of ceftriaxone may halt and/or reverse the progression of neurodegeneration in patients with adult-onset Alexander's disease and may significantly improve their quality of life. FAU - Sechi, Gianpietro AU - Sechi G AD - Department Clinical and Experimental Medicine, University of Sassari, Vial e S. Pietro 10, 07100, Sassari, Italy, gpsechi@uniss.it. FAU - Ceccherini, Isabella AU - Ceccherini I FAU - Bachetti, Tiziana AU - Bachetti T FAU - Deiana, Giovanni A AU - Deiana GA FAU - Sechi, Elia AU - Sechi E FAU - Balbi, Pietro AU - Balbi P LA - eng PT - Journal Article DEP - 20121013 PL - Germany TA - JIMD Rep JT - JIMD reports JID - 101568557 PMC - PMC3565626 OID - NLM: PMC3565626 EDAT- 2013/02/23 06:00 MHDA- 2013/02/23 06:01

CRDTPHSTPHSTPHSTPHSTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI DP TI -

2013/02/23 06:00 2012/06/05 [received] 2012/09/10 [accepted] 2012/09/06 [revised] 2012/10/13 [aheadofprint] 10.1007/8904_2012_180 [doi] ppublish JIMD Rep. 2013;9:67-71. doi: 10.1007/8904_2012_180. Epub 2012 Oct 13.

23324478 NLM MEDLINE 20130130 20130723 20131114 1750-1172 (Electronic) 1750-1172 (Linking) 8 2013 Disease and patient characteristics in NP-C patients: findings from an international disease registry. PG - 12 LID - 10.1186/1750-1172-8-12 [doi] AB - BACKGROUND: Niemann-Pick disease type C (NP-C) is a rare neurovisceral dis ease characterized by progressive neurodegeneration and premature death. We rep ort data recorded at enrolment in an ongoing international NP-C registry initi ated in September 2009 to describe disease natural history, clinical course and tr eatment experience of NP-C patients in clinical practice settings. METHODS: The NP C Registry is a prospective observational cohort study. Participating sites are encouraged to evaluate all consecutive patients with a confirmed diagnosis of NP-C, regardless of their treatment status. All patients undergo clinical assessments and medical care as determined by their physicians. Data are collected through a secure internet-based data collection system. RESULTS: As of 19th March, 2012, 163 patients have been enrolled in centres across 14 Eur opean countries, Australia, Brazil and Canada. The mean (SD) age at enrolment wa s 19.6 (13.0) years. In general there was a long lag time between the mean (SD) a ge at neurological onset (10.9 (9.8) years) and age at diagnosis (15.0 (12.2) ye ars). Among all enrolled patients, 107 were diagnosed based on combined genetic testing and filipin staining. Sixteen (11%) out of 145 patients with available age-at-neurological-onset data had early-infantile neurological onset, 45 (31%) had late-infantile onset; 45 (31%) had juvenile onset and 39 (27%) had adolescent/adult onset. The frequencies of neonatal jaundice, hepatomegaly and/or splenomegaly during infancy were greatest among early-infantile patients, and decreased with increasing age at neurological onset. The most frequent

neurological manifestations were: ataxia (70%), vertical supranuclear gaze palsy (VSGP; 70%), dysarthria (66%), cognitive impairment (62%), dysphagia (52%) . There were no notable differences in composite NP-C disability scores between age-at-neurological-onset groups. Miglustat therapy at enrolment was recor ded in 117/163 (72%) patients. CONCLUSIONS: Approximately two-thirds of this NP-C cohort had infantile or juvenile onset of neurological manifestations, while the remaining third presented in adolescence or adulthood. While systemic symp toms were most common among patients with early-childhood onset disease, they w ere also common among patients with adolescent/adult onset. The profiles of neurological manifestations in this Registry were in line with previous publications. FAU - Patterson, Marc C AU - Patterson MC AD - Mayo Clinic, Rochester, MN, USA. patterson.marc@mayo.edu FAU - Mengel, Eugen AU - Mengel E FAU - Wijburg, Frits A AU - Wijburg FA FAU - Muller, Audrey AU - Muller A FAU - Schwierin, Barbara AU - Schwierin B FAU - Drevon, Harir AU - Drevon H FAU - Vanier, Marie T AU - Vanier MT FAU - Pineda, Merce AU - Pineda M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130116 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (Enzyme Inhibitors) RN - 0 (miglustat) RN - 19130-96-2 (1-Deoxynojirimycin) SB - IM EIN - Orphanet J Rare Dis. 2013;8:73 MH - 1-Deoxynojirimycin/analogs & derivatives/therapeutic use MH - Adolescent MH - Age of Onset MH - Child MH - Child, Preschool MH - Disabled Persons MH - Enzyme Inhibitors/therapeutic use MH - Female MH - Humans MH - Infant MH - Male MH - Mutation MH - Niemann-Pick Disease, Type C/drug therapy/genetics/*physiopathology

MH MH PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI PG FAU AU AD FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH -

Prospective Studies *Registries PMC3558399 NLM: PMC3558399 2013/01/18 06:00 2013/07/24 06:00 2013/01/18 06:00 2012/11/05 [received] 2013/01/07 [accepted] 2013/01/16 [aheadofprint] 1750-1172-8-12 [pii] 10.1186/1750-1172-8-12 [doi] epublish Orphanet J Rare Dis. 2013 Jan 16;8:12. doi: 10.1186/1750-1172-8-12. 23291698 NLM MEDLINE 20130107 20130725 1349-7235 (Electronic) 0918-2918 (Linking) 52 1 2013 Intravenous t-PA for the occlusion of an accessory MCA. 163 Kuwahara, Hiroya Kuwahara H Department of Neurology, Tokyo Metropolitan Bokutoh Hospital, Japan. h-kuwahara.nuro@tmd.ac.jp Matsumura, Ken Matsumura K Watanabe, Mutsufusa Watanabe M Fujigasaki, Hiroto Fujigasaki H eng Case Reports Journal Article 20130101 Japan Intern Med Internal medicine (Tokyo, Japan) 9204241 EC 3.4.21.68 (Tissue Plasminogen Activator) IM Adult Dysarthria/diagnosis/etiology Hemiplegia/diagnosis/etiology Humans Infarction, Middle Cerebral Artery/*drug therapy/radiography Infusions, Intravenous Magnetic Resonance Angiography/methods Male Middle Cerebral Artery/*drug effects/pathology/*radiography Risk Assessment Severity of Illness Index Tissue Plasminogen Activator/*administration & dosage Treatment Outcome

MH EDATMHDACRDTPHSTAID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI e

Vascular Patency/drug effects 2013/01/08 06:00 2013/07/26 06:00 2013/01/08 06:00 2013/01/01 [epublish] DN/JST.JSTAGE/internalmedicine/52.8999 [pii] ppublish Intern Med. 2013;52(1):163. Epub 2013 Jan 1. 23269054 NLM MEDLINE 20121227 20131203 1349-8029 (Electronic) 0470-8105 (Linking) 52 12 2012 Repeated delayed onset cerebellar radiation injuries after linear accelerator-based stereotactic radiosurgery for vestibular schwannoma: cas

report. PG - 933-6 AB - A 63-year-old woman presented with right hearing disturbance and vertigo. Magnetic resonance (MR) imaging revealed the presence of right vestibular schwannoma (VS). Stereotactic radiosurgery (SRS) was performed with a tumo r marginal dose of 14 Gy using two isocenters. She was followed up clinicall y and neuroradiologically using three-dimensional spoiled gradient-echo MR imagi ng. She experienced temporal neurological deterioration due to peritumoral edema i n her right cerebellar peduncle and pons for a few months beginning 1.5 years af ter SRS, when she experienced transient right facial dysesthesia and hearing deterioration. Ten years after SRS, the patient presented with sudden onse t of vertigo, gait disturbance, diplopia, dysarthria, and nausea. MR imaging demonstrated a new lesion in the right cerebellar peduncle, which was diag nosed as radiation-induced stroke. The patient was followed up conservatively an d her symptoms disappeared within a few months. Multiple delayed onset radiation injuries are possible sequelae of SRS for VS. FAU - Ujifuku, Kenta AU - Ujifuku K AD - Department of Neurosurgery, Nagasaki University Graduate School of Biomedi cal Sciences, Nagasaki, Nagasaki. FAU - Matsuo, Takayuki AU - Matsuo T FAU - Toyoda, Keisuke AU - Toyoda K FAU - Baba, Shiro AU - Baba S FAU - Okunaga, Tomohiro AU - Okunaga T FAU - Hayashi, Yukishige

AU FAU AU FAU AU FAU AU FAU AU FAU AU LA PT PT PL TA JT JID SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI ernal

Hayashi Y Kamada, Kensaku Kamada K Morikawa, Minoru Morikawa M Suyama, Kazuhiko Suyama K Nagata, Izumi Nagata I Hayashi, Nobuyuki Hayashi N eng Journal Article Research Support, Non-U.S. Gov't Japan Neurol Med Chir (Tokyo) Neurologia medico-chirurgica 0400775 IM Brain Edema/diagnosis Cerebellum/blood supply/*radiation effects Female Follow-Up Studies Humans Image Interpretation, Computer-Assisted Imaging, Three-Dimensional Magnetic Resonance Imaging Middle Aged Neurologic Examination Neuroma, Acoustic/*surgery Postoperative Complications/*diagnosis Radiation Injuries/*diagnosis Radiosurgery/*adverse effects Stroke/*diagnosis 2012/12/28 06:00 2013/12/16 06:00 2012/12/28 06:00 DN/JST.JSTAGE/nmc/52.933 [pii] ppublish Neurol Med Chir (Tokyo). 2012;52(12):933-6. 23269047 NLM MEDLINE 20121227 20131203 1349-8029 (Electronic) 0470-8105 (Linking) 52 12 2012 External carotid artery stenting and superficial temporal artery to middle cerebral artery anastomosis for internal carotid artery occlusion with ext

carotid artery severe stenosis: case report. PG - 906-9 AB - Superficial temporal artery (STA) to middle cerebral artery (MCA) anastomo sis may have inadequate effects in patients with internal carotid artery (ICA) occ lusion

and severe stenosis of the ipsilateral external carotid artery (ECA), beca use poor blood flow in the STA leads to insufficient flow to the MCA. In these patients, dilation of the stenotic ECA is required to improve the blood fl ow in the STA before STA-MCA anastomosis. A 71-year-old man presented with left hemiparesis and dysarthria. Magnetic resonance imaging revealed an old wat ershed infarction in the right cerebral hemisphere. Right carotid angiography sho wed right ICA occlusion and severe ipsilateral ECA stenosis. Single photon emi ssion computed tomography (SPECT) demonstrated severe hemodynamic insufficiency in the right MCA territory. Instead of endarterectomy of the ECA, angioplasty and stenting (CAS) for ECA was performed to ensure adequate blood flow in the STA, due to the history of myocardial infarction and bifurcation of the common carotid artery at a high level (C2 level). Then STA-MCA anastomosis was performed 1 month later. Postoperative SPECT demonstrated marked improvement of hemodynamic insufficiency in the right MCA territory. After treatment, the patient had no ischemic events. This case suggests external CAS together with STA-MCA anastomosis is a good therapeutic option for a patient with symptomatic IC A occlusion and severe stenosis of the ipsilateral ECA if external CEA is di fficult to perform. FAU - Oku, Takayuki AU - Oku T AD - Department of Neurosurgery, Yamaguchi University School of Medicine, Ube, Yamaguchi. FAU - Nogami, Kenichiro AU - Nogami K FAU - Koizumi, Hiroyasu AU - Koizumi H FAU - Ishihara, Hideyuki AU - Ishihara H FAU - Kato, Shoichi AU - Kato S FAU - Fujisawa, Hirosuke AU - Fujisawa H FAU - Suzuki, Michiyasu AU - Suzuki M LA - eng PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 RN - C2A5X08042 (Iofetamine) SB - IM MH - Aged MH - Anastomosis, Surgical MH - *Carotid Artery, External MH - *Carotid Artery, Internal MH - Carotid Stenosis/diagnosis/*surgery MH - Cerebral Revascularization/*methods

MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI ave

Combined Modality Therapy Dysarthria/etiology Humans Image Interpretation, Computer-Assisted Iofetamine/diagnostic use Magnetic Resonance Angiography Magnetic Resonance Imaging Male Middle Cerebral Artery/surgery Paresis/etiology Postoperative Complications/diagnosis *Stents Temporal Arteries/surgery Tomography, Emission-Computed, Single-Photon 2012/12/28 06:00 2013/12/16 06:00 2012/12/28 06:00 DN/JST.JSTAGE/nmc/52.906 [pii] ppublish Neurol Med Chir (Tokyo). 2012;52(12):906-9. 23250678 NLM MEDLINE 20121219 20130528 1576-6578 (Electronic) 0210-0010 (Linking) 56 1 2013 Jan 1 [Neurological manifestations and their functional impact in subjects who h

suffered heatstroke]. PG - 19-24 AB - INTRODUCTION: Heatstroke is a potentially fatal medical emergency characte rised by an increase in body temperature above 40 degrees C and which is accompa nied by negative effects on the central nervous system secondary to failure of the thermo-regulatory system. If timely care is not provided, the consequences can be fatal and the patient dies, but they can also result in permanent disablin g neurological sequelae that compromise the individual's quality of life. CA SE REPORTS: Following a retrospective search on patients diagnosed with heats troke who were admitted to a brain damage rehabilitation unit, we report the cas es of five subjects. The clinical spectrum ranged from motor weakness with sever ely compromised coordination and balance with ataxia and dysarthria, which wer e accompanied by cognitive impairment of varying degrees of severity. All th e patients required help in performing their basic activities of daily livin g both before and after treatment with rehabilitation. CONCLUSIONS: No functional improvement was observed in the five subjects after completing rehabilitat

ion, which left them wholly dependent on some caregiver. It is therefore essent ial to prevent heatstroke, provide immediate medical care and, finally, once the clinical signs and symptoms have set in, rehabilitation treatment must be focused on preventing the appearance of complications. In such a situation the exi stence of social support for relatives and for patients plays a crucial role. FAU - Laxe, Sara AU - Laxe S AD - Institut de Neurorehabilitacio Guttmann-UAB, 08916 Badalona, Espana. slaxe@guttmann.com FAU - Zuniga-Inestroza, Lucia AU - Zuniga-Inestroza L FAU - Bernabeu-Guitart, Montserrat AU - Bernabeu-Guitart M LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Manifestaciones neurologicas y su impacto funcional en sujetos que han pad ecido un golpe de calor. PL - Spain TA - Rev Neurol JT - Revista de neurologia JID - 7706841 SB - IM MH - Adult MH - Bipolar Disorder/complications MH - Brain Damage, Chronic/*etiology/prevention & control/rehabilitation MH - Cognition Disorders/etiology MH - Disseminated Intravascular Coagulation/etiology MH - Dysarthria/etiology MH - Executive Function MH - Gait Disorders, Neurologic/etiology MH - Heat Stroke/*complications MH - Humans MH - Male MH - Middle Aged MH - Multiple Organ Failure/etiology MH - Occupational Exposure MH - Psychomotor Agitation/etiology MH - Retrospective Studies MH - Rhabdomyolysis/etiology EDAT- 2012/12/20 06:00 MHDA- 2013/05/29 06:00 CRDT- 2012/12/20 06:00 AID - rn2012145 [pii] PST - ppublish SO - Rev Neurol. 2013 Jan 1;56(1):19-24. PMIDOWN STATDA DCOMLR IS 23166429 NLM MEDLINE 20121120 20130426 20131121 1598-6357 (Electronic)

IS - 1011-8934 (Linking) VI - 27 IP - 11 DP - 2012 Nov TI - The return of an old worm: cerebral paragonimiasis presenting with intrace rebral hemorrhage. PG - 1428-32 LID - 10.3346/jkms.2012.27.11.1428 [doi] AB - Paragonimiasis is caused by ingesting crustaceans, which are the intermedi ate hosts of Paragonimus. The involvement of the brain was a common presentati on in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain ima ging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by th e hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. T his case alerts practicing clinicians to the domestic transmission of a forgot ten parasitic disease due to environmental changes. FAU - Koh, Eun Jung AU - Koh EJ AD - Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Korea. FAU - Kim, Seung-Ki AU - Kim SK FAU - Wang, Kyu-Chang AU - Wang KC FAU - Chai, Jong-Yil AU - Chai JY FAU - Chong, Sangjoon AU - Chong S FAU - Park, Sung-Hye AU - Park SH FAU - Cheon, Jung-Eun AU - Cheon JE FAU - Phi, Ji Hoon AU - Phi JH LA - eng PT - Case Reports PT - Journal Article DEP - 20121030 PL - Korea (South) TA - J Korean Med Sci JT - Journal of Korean medical science JID - 8703518 RN - 0 (Anthelmintics) RN - 6490C9U457 (Praziquantel)

SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID OTO OT OT OT EDATMHDACRDTPHSTPHSTPHSTAID PST SO 28. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG FAU AU AD FAU AU FAU AU LA PT PT PL TA JT -

IM Animals Anthelmintics/therapeutic use Brain/parasitology/pathology Cerebral Hemorrhage/*etiology Child Dysarthria/etiology Facial Paralysis/etiology Female Humans Magnetic Resonance Imaging Paragonimiasis/*diagnosis/drug therapy/parasitology Paragonimus/isolation & purification Praziquantel/therapeutic use Tomography, X-Ray Computed Vascular Malformations/etiology PMC3492682 NLM: PMC3492682 NOTNLM Cerebral Paragonimiasis Diagnosis Intracerebral Hemorrhage 2012/11/21 06:00 2013/04/27 06:00 2012/11/21 06:00 2011/12/21 [received] 2012/07/05 [accepted] 2012/10/30 [epublish] 10.3346/jkms.2012.27.11.1428 [doi] ppublish J Korean Med Sci. 2012 Nov;27(11):1428-32. doi: 10.3346/jkms.2012.27.11.14 Epub 2012 Oct 30. 23152461 NLM MEDLINE 20121115 20130312 20131121 1715-5258 (Electronic) 0008-350X (Linking) 58 11 2012 Nov Unusual case of recurrent falls: myasthenia gravis in an elderly patient. 1231-2 Alaama, Tareef Alaama T University of Western Ontario, London. dr_tareef@yahoo.com Basharat, Pari Basharat P Nicolle, Michael W Nicolle MW eng Case Reports Journal Article Canada Can Fam Physician Canadian family physician Medecin de famille canadien

JID RN RN RN RN RN RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI DP TI -

0120300 0 (Antibodies) 0 (Cholinesterase Inhibitors) 0 (Glucocorticoids) 0 (Immunoglobulins, Intravenous) 0 (Receptors, Cholinergic) KVI301NA53 (Pyridostigmine Bromide) VB0R961HZT (Prednisone) IM *Accidental Falls Aged, 80 and over Antibodies/analysis Blepharoptosis/etiology Cholinesterase Inhibitors/therapeutic use Dysarthria/etiology Electrodiagnosis Facial Muscles/physiopathology Female Glucocorticoids/therapeutic use Humans Immunoglobulins, Intravenous/therapeutic use Muscle Weakness/physiopathology Myasthenia Gravis/*diagnosis/drug therapy Neurologic Examination Prednisone/therapeutic use Pyridostigmine Bromide/therapeutic use Receptors, Cholinergic/immunology Recurrence PMC3498017 NLM: PMC3498017 2012/11/16 06:00 2013/03/13 06:00 2012/11/16 06:00 58/11/1231 [pii] ppublish Can Fam Physician. 2012 Nov;58(11):1231-2.

23039766 NLM MEDLINE 20130124 20130621 20131114 1750-1172 (Electronic) 1750-1172 (Linking) 7 2012 Dysphagia as a risk factor for mortality in Niemann-Pick disease type C: systematic literature review and evidence from studies with miglustat. PG - 76 LID - 10.1186/1750-1172-7-76 [doi] AB - Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease charact erised by progressive neurological deterioration and premature death, and has an estimated birth incidence of 1:120,000. Mutations in the NPC1 gene (in 95% of cases) and the NPC2 gene (in approximately 4% of cases) give rise to impai red intracellular lipid metabolism in a number of tissues, including the brain .

Typical neurological manifestations include vertical supranuclear gaze pal sy, saccadic eye movement abnormalities, cerebellar ataxia, dystonia, dysmetri a, dysphagia and dysarthria. Oropharyngeal dysphagia can be particularly prob lematic as it can often lead to food or fluid aspiration and subsequent pneumonia. Epidemiological data suggest that bronchopneumonia subsequent to food or f luid aspiration is a major cause of mortality in NP-C and other neurodegenerati ve disorders. These findings indicate that a therapy capable of improving or stabilising swallowing function might reduce the risk of aspiration pneumo nia, and could have a positive impact on patient survival. Miglustat, currently the only approved disease-specific therapy for NP-C in children and adults, ha s been shown to stabilise key neurological manifestations in NP-C, including dysp hagia. In this article we present findings from a systematic literature review of published data on bronchopneumonia/aspiration pneumonia as a cause of deat h, and on the occurrence of dysphagia in NP-C and other neurodegenerative disease s. We then examine the potential links between dysphagia, aspiration, pneumonia and mortality with a view to assessing the possible effect of miglustat on pat ient lifespan. FAU - Walterfang, Mark AU - Walterfang M AD - Royal Melbourne Hospital and Melbourne Neuropsychiatry Centre, Melbourne, Australia. mark.walterfang@mh.org.au FAU - Chien, Yin-Hsiu AU - Chien YH FAU - Imrie, Jackie AU - Imrie J FAU - Rushton, Derren AU - Rushton D FAU - Schubiger, Danielle AU - Schubiger D FAU - Patterson, Marc C AU - Patterson MC LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20121006 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (miglustat) RN - 19130-96-2 (1-Deoxynojirimycin) SB - IM MH - 1-Deoxynojirimycin/*analogs & derivatives/therapeutic use MH - Animals MH - Deglutition Disorders/*complications/mortality MH - Humans

MH ty PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI -

Niemann-Pick Disease, Type C/*diagnosis/*drug therapy/epidemiology/mortali PMC3552828 NLM: PMC3552828 2012/10/09 06:00 2013/06/26 06:00 2012/10/09 06:00 2012/05/12 [received] 2012/09/24 [accepted] 2012/10/06 [aheadofprint] 1750-1172-7-76 [pii] 10.1186/1750-1172-7-76 [doi] epublish Orphanet J Rare Dis. 2012 Oct 6;7:76. doi: 10.1186/1750-1172-7-76.

23011859 NLM MEDLINE 20120926 20130405 20131121 1576-6578 (Electronic) 0210-0010 (Linking) 55 7 2012 Oct 1 [Response to treatment with corticoids in a case of inflammatory amyloid angiopathy without performing a biopsy]. PG - 408-12 AB - INTRODUCTION: Inflammatory amyloid angiopathy (IAA) is an infrequent prese nting symptom of the recently recognised cerebral amyloid angiopathy and its def initive diagnosis is reached by means of pathological analyses. AIM: We report the case of a male patient with IAA and good clinical, neuropsychological and neuro imaging response to treatment with corticoids; a biopsy of brain tissue was not considered necessary. CASE REPORT: The patient, 68 years old and diagnosed with Alzheimer's disease, suffered from generalised seizures followed by a lang uage disorder and hemiparesis of the right-hand side. A magnetic resonance imag ing scan showed a lesion displaying infiltrating behaviour in the left hemisph ere and multiple instances of microbleeding. Clinical and radiological features su ggested IAA and treatment was established with corticoids. Neuroimaging and neuropsychological tests revealed a notable improvement at 30 days after beginning treatment with immunosuppressants. The genotype was ApoE e4/e4. The need to perform a biopsy of brain tissue was ruled out. CONCLUSIONS: The c ase described here suggests that, in individualised cases with clinical and radiological features that are characteristic of IAA, it may be possible t o establish an empirical treatment with corticoids with a probability diagno sis and perform a biopsy of brain tissue in the event of a lack of response to tre

atment. FAU - de la Riva, Patricia AU - de la Riva P AD - Servicio de Neurologia, Hospital Donostia, San Sebastian, Guipuzcoa, Espan a. patricia.delarivajuez@gmail.com FAU - Moreno, Fermin AU - Moreno F FAU - Carrera, Nieves AU - Carrera N FAU - Barandiaran, Myriam AU - Barandiaran M FAU - Arruti, Maialen AU - Arruti M FAU - Marti-Masso, Jose F AU - Marti-Masso JF LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Respuesta al tratamiento con corticoides en un caso de angiopatia amiloide inflamatoria sin realizacion de biopsia. PL - Spain TA - Rev Neurol JT - Revista de neurologia JID - 7706841 RN - 0 (Apolipoprotein E4) RN - 0 (Immunosuppressive Agents) RN - VB0R961HZT (Prednisone) SB - IM MH - Aged MH - Alzheimer Disease/complications/genetics MH - Apolipoprotein E4/genetics MH - Biopsy MH - Cerebral Amyloid Angiopathy/complications/*drug therapy/pathology MH - Diagnosis, Differential MH - Dysarthria/etiology MH - Epilepsy, Generalized/etiology MH - Homozygote MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Inflammation MH - Magnetic Resonance Imaging MH - Male MH - Neoplasms, Neuroepithelial/diagnosis MH - Paresis/etiology MH - Prednisone/*therapeutic use MH - Remission Induction EDAT- 2012/09/27 06:00 MHDA- 2013/04/06 06:00 CRDT- 2012/09/27 06:00 AID - rn2012155 [pii] PST - ppublish SO - Rev Neurol. 2012 Oct 1;55(7):408-12. PMIDOWN STATDA DCOM22994551 NLM MEDLINE 20121210 20130517

LR IS IS VI DP TI netic

20131114 1471-2377 (Electronic) 1471-2377 (Linking) 12 2012 Neurosyphilis with dementia and bilateral hippocampal atrophy on brain mag

resonance imaging. PG - 96 LID - 10.1186/1471-2377-12-96 [doi] AB - BACKGROUND: This article reports a rare case of active neurosyphilis in a man with mild to moderate dementia and marked hippocampal atrophy, mimicking e arly onset Alzheimer's disease. Few cases have so far described bilateral hippo campal atrophy mimicking Alzheimer's disease in neurosyphilis. CASE PRESENTATION: The patient presented here is a 33 year old Bulgarian male, whose clinical fea tures include progressive cognitive decline and behavioral changes over the last 18 months. Neuropsychological examination revealed mild to moderate dementia (Mini Mental State Examination score was 16/30) with impaired memory and attenti on, and executive dysfunction. Pyramidal, and extrapyramidal signs, as well as dys arthria and impairment in coordination, were documented. Brain magnetic resonance imaging showed cortical atrophy with noticeable bilateral hippocampal atrophy. The diagnosis of active neurosyphilis was based on positive results of the Ven ereal Disease Research Laboratory test/Treponema pallidum hemagglutination react ions in blood and cerebrospinal fluid samples. In addition, cerebrospinal fluid an alysis showed pleocytosis and elevated protein levels. High-dose intravenous peni cillin therapy was administered. At 6 month follow up, improvements were noted clinically, on neuropsychological examinations, and in cerebrospinal fluid samples. CONCLUSION: This case underlines the importance of early diagnosi s of neurosyphilis. The results suggest that neurosyphilis should be considered when magnetic resonance imaging results indicate mesiotemporal abnormalities an d hippocampal atrophy. Neurosyphilis is a treatable condition which requires early aggressive antibiotic therapy. FAU - Mehrabian, Shima AU - Mehrabian S AD - University Hospital Alexandrovska, Department of Neurology, 1, Georgi Sofi iski str, 1431 Sofia, Bulgaria. shima_meh@yahoo.com FAU - Raycheva, Margarita AU - Raycheva M FAU - Traykova, Martina AU - Traykova M FAU - Stankova, Tonya

AU FAU AU FAU AU FAU AU LA PT PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI ve PG LID LID AB VZV)

Stankova T Penev, Latchezar Penev L Grigorova, Olga Grigorova O Traykov, Latchezar Traykov L eng Case Reports Journal Article Research Support, Non-U.S. Gov't 20120920 England BMC Neurol BMC neurology 100968555 IM Adult Alzheimer Disease/pathology Atrophy/pathology Dementia/*complications/*diagnosis Diagnosis, Differential Hippocampus/*pathology Humans Magnetic Resonance Imaging/*methods Male Neurosyphilis/*complications/*pathology PMC3517431 NLM: PMC3517431 2012/09/22 06:00 2013/05/18 06:00 2012/09/22 06:00 2012/02/17 [received] 2012/09/17 [accepted] 2012/09/20 [aheadofprint] 1471-2377-12-96 [pii] 10.1186/1471-2377-12-96 [doi] epublish BMC Neurol. 2012 Sep 20;12:96. doi: 10.1186/1471-2377-12-96. 22935406 NLM MEDLINE 20121029 20130418 20131218 1878-5883 (Electronic) 0022-510X (Linking) 323 1-2 2012 Dec 15 Varicella zoster virus vasculopathy: a treatable form of rapidly progressi multi-infarct dementia after 2 years' duration. 245-7 10.1016/j.jns.2012.07.059 [doi] S0022-510X(12)00413-3 [pii] We describe an extraordinarily protracted case of varicella zoster virus ( multifocal vasculopathy in a man who presented initially with ischemic opt

ic neuropathy and then suffered 4 episodes of stroke manifesting as multi-inf arct dementia over a 2-year period. Brain magnetic resonance imaging (MRI) and angiography (MRA) revealed cortical and subcortical infarctions as well as vasculitic occlusion and stenosis. The patient was treated with corticoste roids and later with cyclophosphamide. More than 2 years after the onset of neurological disease, two cerebrospinal fluid (CSF) examinations revealed the presence of anti-VZV IgG antibody with reduced serum-to-CSF ratios of anti -VZV IgG compared with ratios for total IgG and albumin, indicative of intrathe cal synthesis of anti-VZV IgG. After definitive diagnosis, immunosuppressive d rugs were discontinued and he was treated with intravenous acyclovir; both ment al status and gait improved and no further episodes of neurological dysfuncti on ensued. The favorable outcome in this patient indicates that VZV vasculopa thy can be treated successfully even after 26 months. VZV must be considered as a possible cause of neurological disease in any patient with idiopathic mult ifocal vasculopathy. CI - Copyright (c) 2012 Elsevier B.V. All rights reserved. FAU - Silver, Brian AU - Silver B AD - Department of Neurology, Warren Alpert Medical School of Brown University, Providence, RI, USA. FAU - Nagel, Maria A AU - Nagel MA FAU - Mahalingam, Ravi AU - Mahalingam R FAU - Cohrs, Randall AU - Cohrs R FAU - Schmid, D Scott AU - Schmid DS FAU - Gilden, Don AU - Gilden D LA - eng GR - AG006127/AG/NIA NIH HHS/United States GR - AG032958/AG/NIA NIH HHS/United States GR - K08 NS067070/NS/NINDS NIH HHS/United States GR - P01 AG032958/AG/NIA NIH HHS/United States GR - R01 AG006127/AG/NIA NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20120827 PL - Netherlands TA - J Neurol Sci JT - Journal of the neurological sciences JID - 0375403 RN - 0 (Antiviral Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - HG18B9YRS7 (Valine) RN - MZ1IW7Q79D (valacyclovir) RN - VB0R961HZT (Prednisone)

RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID AID PST SO Epub PMIDOWN STATDA DCOMLR IS IS VI IP DP TI -

X4HES1O11F (Acyclovir) IM Acyclovir/analogs & derivatives/therapeutic use Aged Antiviral Agents/therapeutic use Apraxias/etiology Basal Ganglia Cerebrovascular Disease/etiology Cyclophosphamide/therapeutic use Dementia, Multi-Infarct/drug therapy/*etiology Disease Progression Drug Therapy, Combination Dysarthria/etiology Encephalitis, Varicella Zoster/*complications/diagnosis/drug therapy Gait Disorders, Neurologic/etiology Humans Magnetic Resonance Angiography Male Memory Disorders/etiology Prednisone/therapeutic use Pupil Disorders/etiology Recovery of Function Thalamus/blood supply Valine/analogs & derivatives/therapeutic use Vertebrobasilar Insufficiency/complications Vision Disorders/etiology PMC3483445 NIHMS404013 NLM: NIHMS404013 NLM: PMC3483445 2012/09/01 06:00 2013/04/20 06:00 2012/09/01 06:00 2012/07/06 [received] 2012/07/24 [revised] 2012/07/26 [accepted] 2012/08/27 [aheadofprint] S0022-510X(12)00413-3 [pii] 10.1016/j.jns.2012.07.059 [doi] ppublish J Neurol Sci. 2012 Dec 15;323(1-2):245-7. doi: 10.1016/j.jns.2012.07.059. 2012 Aug 27.

22851346 NLM MEDLINE 20120801 20130320 20131115 1098-9056 (Electronic) 0734-0478 (Linking) 33 3 2012 Aug Telerehabilitation, virtual therapists, and acquired neurologic speech and language disorders. PG - 243-57 LID - 10.1055/s-0032-1320044 [doi] AB - Telerehabilitation (telerehab) offers cost-effective services that potenti ally

can improve access to care for those with acquired neurologic communicatio n disorders. However, regulatory issues including licensure, reimbursement, and threats to privacy and confidentiality hinder the routine implementation o f telerehab services into the clinical setting. Despite these barriers, rapi d technological advances and a growing body of research regarding the use of telerehab applications support its use. This article reviews the evidence related to acquired neurologic speech and language disorders in adults, focusing o n studies that have been published since 2000. Research studies have used te lerehab systems to assess and treat disorders including dysarthria, apraxia of spe ech, aphasia, and mild Alzheimer disease. They show that telerehab is a valid a nd reliable vehicle for delivering speech and language services. The studies represent a progression of technological advances in computing, Internet, and mobile technologies. They range on a continuum from working synchronously (in real-time) with a speech-language pathologist to working asynchronously (o ffline) with a stand-in virtual therapist. One such system that uses a virtual the rapist for the treatment of aphasia, the Web-ORLA (Rehabilitation Institute of Ch icago, Chicago, IL) system, is described in detail. Future directions for the advancement of telerehab for clinical practice are discussed. CI - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA. FAU - Cherney, Leora R AU - Cherney LR AD - Center for Aphasia Research and Treatment, Rehabilitation Institute of Chi cago, IL 60611, USA. Lcherney@ric.org FAU - van Vuuren, Sarel AU - van Vuuren S LA - eng GR - 1R01DC011754-01/DC/NIDCD NIH HHS/United States GR - R01 DC011754/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review DEP - 20120731 PL - United States TA - Semin Speech Lang JT - Seminars in speech and language JID - 8405117 SB - IM MH - Adult MH - Humans MH - Language Disorders/*rehabilitation MH - Speech Disorders/*rehabilitation MH - Speech Therapy/*methods MH - Telemedicine/*methods PMC - PMC3691350 MID - NIHMS482354

OID - NLM: NIHMS482354 OID - NLM: PMC3691350 EDAT- 2012/08/02 06:00 MHDA- 2013/03/21 06:00 CRDT- 2012/08/02 06:00 PHST- 2012/07/31 [epublish] AID - 10.1055/s-0032-1320044 [doi] PST - ppublish SO - Semin Speech Lang. 2012 Aug;33(3):243-57. doi: 10.1055/s-0032-1320044. Epu b 2012 Jul 31. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI apy ested within a randomized controlled trial. PG - 174-82 LID - 10.1177/0269215512450042 [doi] AB - OBJECTIVES: To explore trial participants' experiences of the process and outcomes of early, enhanced speech and language therapy after stroke with support from an employed visitor. DESIGN: Qualitative study nested within a random ized controlled trial. PARTICIPANTS: Twney-two people who, after stroke, had a diagnosis of aphasia (12), dysarthria (5) or both (5) and who participated in the ACT NoW study. SETTING: Eight English NHS usual care settings. METHOD: Ind ividual interviews. Thematic content analysis assisted by a bespoke data transform ation protocol for incorporating non-verbal and semantically ambiguous data. RES ULTS: Participants highly regarded regular and sustained contact with someone ou tside of immediate family/friends who engaged them in deliberate activities/communication in the early months after stroke. Participants identified differences in the process of intervention between speech and l anguage therapists and employed visitors. But no major discriminations were made b etween the impact or value of this contact according to whether provided by a spe ech and language therapist or employed visitor. Participant-defined criteria for effectiveness of contact included: impact on mood and confidence, self-recognition of progress and the meeting of individual needs. CONCLUSI ONS: As in the randomized controlled trial, participants reported no evidence of a dded 22837542 NLM MEDLINE 20130122 20130821 20131115 1477-0873 (Electronic) 0269-2155 (Linking) 27 2 2013 Feb Trial participants' experiences of early enhanced speech and language ther after stroke compared with employed visitor support: a qualitative study n

benefit of early communication therapy beyond that from attention control. The findings do not imply that regular contact with any non-professional can h ave beneficial effects for someone with aphasia or dysarthria in the early wee ks following a stroke. The study points to specific conditions that would hav e to be met for contact to have a positive effect. FAU - Young, Alys AU - Young A AD - School of Nursing Midwifery and Social Work, University of Manchester, Man chester Academic Health Sciences Centre, UK. alys.young@manchester.ac.uk FAU - Gomersall, Timothy AU - Gomersall T FAU - Bowen, Audrey AU - Bowen A CN - ACT NoW investigators LA - eng GR - Department of Health/United Kingdom PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20120726 PL - England TA - Clin Rehabil JT - Clinical rehabilitation JID - 8802181 SB - IM MH - Aged MH - Aged, 80 and over MH - Aphasia/etiology/psychology/*rehabilitation MH - Cohort Studies MH - Dysarthria/etiology/psychology/*rehabilitation MH - Female MH - Humans MH - *Language Therapy MH - Male MH - Middle Aged MH - Outcome and Process Assessment (Health Care) MH - *Patient Satisfaction MH - Qualitative Research MH - *Speech Therapy MH - Stroke/*complications/rehabilitation PMC - PMC3757994 OID - NLM: PMC3757994 EDAT- 2012/07/28 06:00 MHDA- 2013/08/22 06:00 CRDT- 2012/07/28 06:00 PHST- 2012/07/26 [aheadofprint] AID - 0269215512450042 [pii] AID - 10.1177/0269215512450042 [doi] PST - ppublish SO - Clin Rehabil. 2013 Feb;27(2):174-82. doi: 10.1177/0269215512450042. Epub 2 012 Jul 26. PMID- 22797843 OWN - NLM

STATDA DCOMLR IS IS VI DP TI fter PG LID LID AB n the tured

MEDLINE 20120716 20120927 20131115 1756-1833 (Electronic) 0959-535X (Linking) 345 2012 Effectiveness of enhanced communication therapy in the first four months a stroke for aphasia and dysarthria: a randomised controlled trial. e4407 10.1136/bmj.e4407 [doi] bmj.e4407 [pii] OBJECTIVE: To assess the effectiveness of enhanced communication therapy i first four months after stroke compared with an attention control (unstruc social contact). DESIGN: Externally randomised, pragmatic, parallel, super

iority trial with blinded outcome assessment. SETTING: Twelve UK hospital and com munity stroke services. PARTICIPANTS: 170 adults (mean age 70 years) randomised w ithin two weeks of admission to hospital with stroke (December 2006 to January 2 010) whom speech and language therapists deemed eligible, and 135 carers. INTERVENTIONS: Enhanced, agreed best practice, communication therapy speci fic to aphasia or dysarthria, offered by speech and language therapists according to participants' needs for up to four months, with continuity from hospital t o community. Comparison was with similarly resourced social contact (without communication therapy) from employed visitors. OUTCOME MEASURES: Primary o utcome was blinded, functional communicative ability at six months on the Therapy Outcome Measure (TOM) activity subscale. Secondary outcomes (unblinded, si x months): participants' perceptions on the Communication Outcomes After Str oke scale (COAST); carers' perceptions of participants from part of the Carer COAST; carers' wellbeing on Carers of Older People in Europe Index and quality of life items from Carer COAST; and serious adverse events. RESULTS: Therapist and visitor contact both had good uptake from service users. An average 22 con tacts (intervention or control) over 13 weeks were accepted by users. Impairment focused therapy was the approach most often used by the speech and languag e therapists. Visitors most often provided general conversation. In total, 8 1/85 of the intervention group and 72/85 of the control group completed the primar y outcome measure. Both groups improved on the TOM activity subscale. The es timated six months group difference was not statistically significant, with 0.25 ( 95% CI -0.19 to 0.69) points in favour of therapy. Sensitivity analyses that adju

sted for chance baseline imbalance further reduced this difference. Per protoco l analyses rejected a possible dilution of treatment effect from controls de clining their allocation and receiving usual care. There was no added benefit of t herapy on secondary outcome measures, subgroup analyses (such as aphasia), or ser ious adverse events, although the latter were less common after intervention (o dds ratio 0.42 (95% CI 0.16 to 1.1)). CONCLUSIONS: Communication therapy had n o added benefit beyond that from everyday communication in the first four months a fter stroke. Future research should evaluate reorganised services that support functional communication practice early in the stroke pathway. This projec t was funded by the NIHR Health Technology Assessment programme (project No 02/1 1/04) and is published in full in Health Technology Assessment 2012;16(26):1-160 . TRIAL REGISTRATION: ISRCTN78617680. FAU - Bowen, Audrey AU - Bowen A AD - HCD, Ellen Wilkinson Building, University of Manchester MAHSC, Manchester Academic Health Science Centre, Manchester M13 9PL, UK. audrey.bowen@manchester.ac.uk FAU - Hesketh, Anne AU - Hesketh A FAU - Patchick, Emma AU - Patchick E FAU - Young, Alys AU - Young A FAU - Davies, Linda AU - Davies L FAU - Vail, Andy AU - Vail A FAU - Long, Andrew F AU - Long AF FAU - Watkins, Caroline AU - Watkins C FAU - Wilkinson, Mo AU - Wilkinson M FAU - Pearl, Gill AU - Pearl G FAU - Ralph, Matthew A Lambon AU - Ralph MA FAU - Tyrrell, Pippa AU - Tyrrell P LA - eng SI - ISRCTN/ISRCTN78617680 GR - Department of Health/United Kingdom PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20120713 PL - England TA - BMJ

JT JID SB SB CIN CIN CIN CIN CIN MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTPST SO PMIDOWN STATDA IS IS VI IP DP TI nd

BMJ (Clinical research ed.) 8900488 AIM IM BMJ. 2012;345:e6014; author reply e6023. PMID: 22963945 BMJ. 2012;345:e4870. PMID: 22807163 BMJ. 2012;345:e6020; author reply e6023. PMID: 22963946 BMJ. 2012;345:e6022; author reply e6023. PMID: 22963947 J Neurol. 2012 Nov;259(11):2510-2. PMID: 23086178 Adult Aged Aged, 80 and over Aphasia/etiology/*rehabilitation Caregivers Communication Dysarthria/etiology/*rehabilitation Female Great Britain Humans Interpersonal Relations Language Therapy Male Middle Aged Outcome Assessment (Health Care)/*statistics & numerical data Patient Selection Practice Guidelines as Topic *Quality of Life Regression Analysis Severity of Illness Index *Speech Therapy Stroke/complications/*rehabilitation Treatment Outcome Visitors to Patients PMC3396461 NLM: PMC3396461 2012/07/17 06:00 2012/09/28 06:00 2012/07/17 06:00 epublish BMJ. 2012 Jul 13;345:e4407. doi: 10.1136/bmj.e4407. 22754604 NLM Publisher 20120703 1940-7742 (Print) 1940-7742 (Linking) 18 3 2011 Oct 1 Children with 7q11.23 Duplication Syndrome: Speech, Language, Cognitive, a

Behavioral Characteristics and their Implications for Intervention. PG - 108-116 AB - 7q11.23 duplication syndrome is a recently-documented genetic disorder ass ociated with severe speech delay, language delay, a characteristic facies, hypoton ia, developmental delay, and social anxiety. Developmentally appropriate nonve rbal

pragmatic abilities are demonstrated in socially comfortable situations. M otor speech disorder (Childhood Apraxia of Speech and/or dysarthria), oral apra xia, and/or phonological disorder or symptoms of these disorders are common as are characteristics consistent with expressive language disorder. Intensive speech/language therapy is critical for maximizing long-term outcomes. FAU - Velleman, Shelley L AU - Velleman SL AD - Department of Communication Sciences and Disorders, 402 Pomeroy, 489 Main Street, University of Vermont, Burlington VT 05405, shelley.velleman@uvm.edu , 802-656-3868. FAU - Mervis, Carolyn B AU - Mervis CB LA - ENG GR - R01 NS035102-15/NS/NINDS NIH HHS/United States GR - R37 HD029957/HD/NICHD NIH HHS/United States GR - R37 HD029957-20/HD/NICHD NIH HHS/United States PT - JOURNAL ARTICLE TA - Perspect Lang Learn Educ JT - Perspectives on language learning and education JID - 101514255 PMC - PMC3383616 MID - NIHMS356374 EDAT- 2012/07/04 06:00 MHDA- 2012/07/04 06:00 CRDT- 2012/07/04 06:00 AID - 10.1044/lle18.3.108 [doi] PST - ppublish SO - Perspect Lang Learn Educ. 2011 Oct 1;18(3):108-116. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 410 22752494 NLM MEDLINE 20120828 20130123 20140319 1708-8283 (Electronic) 0883-0738 (Linking) 27 9 2012 Sep Friedreich ataxia clinical outcome measures: natural history evaluation in

participants. PG - 1152-8 LID - 10.1177/0883073812448462 [doi] AB - Friedreich ataxia is an autosomal recessive neurodegenerative disorder characterized by ataxia, dysarthria, and areflexia. The authors report the progress of a large international noninterventional cohort (n = 410), trac king the natural history of disease progression using the neurologic examinatio n-based Friedreich Ataxia Rating Scale. The authors analyzed the rate of progressi on with cross-sectional analysis and longitudinal analysis over a 2-year period. T he Friedreich Ataxia Rating Scale captured disease progression when used at 1

and 2 years following initial evaluation, with a lower ratio of standard deviati on of change to mean change over 2 years of evaluation. However, modeling of dis ease progression identified substantial ceiling effects in the Friedreich Ataxi a Rating Scale, suggesting this measure is most useful in subjects before ma ximal deficit is approached. FAU - Regner, Sean R AU - Regner SR AD - Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. FAU - Wilcox, Nicholas S AU - Wilcox NS FAU - Friedman, Lisa S AU - Friedman LS FAU - Seyer, Lauren A AU - Seyer LA FAU - Schadt, Kim A AU - Schadt KA FAU - Brigatti, Karlla W AU - Brigatti KW FAU - Perlman, Susan AU - Perlman S FAU - Delatycki, Martin AU - Delatycki M FAU - Wilmot, George R AU - Wilmot GR FAU - Gomez, Christopher M AU - Gomez CM FAU - Bushara, Khalaf O AU - Bushara KO FAU - Mathews, Katherine D AU - Mathews KD FAU - Subramony, S H AU - Subramony SH FAU - Ashizawa, Tetsuo AU - Ashizawa T FAU - Ravina, Bernard AU - Ravina B FAU - Brocht, Alicia AU - Brocht A FAU - Farmer, Jennifer M AU - Farmer JM FAU - Lynch, David R AU - Lynch DR LA - eng GR - 2R13NS040925-14/NS/NINDS NIH HHS/United States GR - R13 NS040925/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20120629 PL - United States TA - J Child Neurol JT - Journal of child neurology JID - 8606714 RN - 0 (Iron-Binding Proteins)

RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTAID AID PST SO 2012 PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB of ar

0 (frataxin) IM Child Child, Preschool Cohort Studies Cross-Sectional Studies Disease Progression Female Friedreich Ataxia/complications/*diagnosis/genetics/*therapy Humans Iron-Binding Proteins/genetics Male Neurologic Examination Outcome Assessment (Health Care) Point Mutation/genetics Retrospective Studies Severity of Illness Index Trinucleotide Repeat Expansion/genetics PMC3674496 NIHMS473257 NLM: NIHMS473257 NLM: PMC3674496 2012/07/04 06:00 2013/01/24 06:00 2012/07/04 06:00 2012/06/29 [aheadofprint] 0883073812448462 [pii] 10.1177/0883073812448462 [doi] ppublish J Child Neurol. 2012 Sep;27(9):1152-8. doi: 10.1177/0883073812448462. Epub Jun 29. 22752490 NLM MEDLINE 20120828 20130123 20140319 1708-8283 (Electronic) 0883-0738 (Linking) 27 9 2012 Sep Cardiac transplantation in Friedreich ataxia. 1193-6 10.1177/0883073812448229 [doi] In this article, we describe a 14-year-old boy with a confirmed diagnosis Friedreich ataxia who underwent cardiac transplantation for left ventricul failure secondary to dilated cardiomyopathy with restrictive physiology. H

is neurological status prior to transplantation reflected early signs of neurological disease, with evidence of dysarthria, weakness, mild gait impairment, and limb ataxia. We review the ethical issues considered durin g the process leading to the decision to offer cardiac transplantation. FAU - Yoon, Grace AU - Yoon G

AD - Division of Clinical and Metabolic Genetics, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. grace.yoon@utoronto.ca FAU - Soman, Teesta AU - Soman T FAU - Wilson, Judith AU - Wilson J FAU - George, Kristen AU - George K FAU - Mital, Seema AU - Mital S FAU - Dipchand, Anne I AU - Dipchand AI FAU - McCabe, Jane AU - McCabe J FAU - Logan, William AU - Logan W FAU - Kantor, Paul AU - Kantor P LA - eng GR - 2R13NS040925-14/NS/NINDS NIH HHS/United States GR - R13 NS040925/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20120629 PL - United States TA - J Child Neurol JT - Journal of child neurology JID - 8606714 SB - IM MH - Friedreich Ataxia/*complications MH - Heart Diseases/*etiology/*surgery MH - Heart Transplantation/*methods MH - Humans PMC - PMC3671892 MID - NIHMS473277 OID - NLM: NIHMS473277 OID - NLM: PMC3671892 EDAT- 2012/07/04 06:00 MHDA- 2013/01/24 06:00 CRDT- 2012/07/04 06:00 PHST- 2012/06/29 [aheadofprint] AID - 0883073812448229 [pii] AID - 10.1177/0883073812448229 [doi] PST - ppublish SO - J Child Neurol. 2012 Sep;27(9):1193-6. doi: 10.1177/0883073812448229. Epub 2012 Jun 29. PMIDOWN STATDA DCOMIS IS VI IP 22652236 NLM MEDLINE 20121211 20131115 0150-9861 (Print) 0150-9861 (Linking) 39 5

DP - 2012 Dec TI - Reversible laminar signal intensity in deep cortical gray matter in T1-wei ghted images and FLAIR after mild acute hyperammonemic hepatic encephalopathy. PG - 350-3 LID - 10.1016/j.neurad.2012.03.002 [doi] LID - S0150-9861(12)00162-9 [pii] AB - Soporific acute hyperammonemic hepatic encephalopathy (aHE) can induce considerable changes in cerebral white and gray matter. This report descri bes a patient in the subacute phase of aHE grade I without disturbed consciousne ss and with reversible fine laminar cortical involvement on magnetic resonance im aging (MRI). The 59-year-old patient had esophageal varices bleeding due to prim ary biliary cirrhosis (ammonium blood level: 140 mmoL/L) and presented with se nsory Jacksonian seizures, dysarthria, and increased drowsiness and fatigue. MRI revealed patchy hyperintense (T2-weighted, T2w) white-matter lesions and bilateral signal intensities in the striatum (T1w). During a rise of ammon ium blood level to 220 mmoL/L, the patient had increased drowsiness, persisten t dysarthria and mild temporary hemiparesis without loss of consciousness. T wo weeks later, the patient was asymptomatic and blood ammonium level had rev erted to normal value. MRI at that time revealed bihemispheric fine laminar subc ortical hyperintensities on T2w and fluid-attenuated inversion recovery (FLAIR) im aging, and partially on T1w sequences, with no swelling or restricted diffusion; the hyperintensities were fully reversible a month later. Such a distinct cort ical signal increase not only on T2w images, but also on T1w, in a patient afte r a mild form of aHE is a new MR finding. CI - Copyright (c) 2012 Elsevier Masson SAS. All rights reserved. FAU - Treusch, N A AU - Treusch NA AD - Department of Neuroradiology, Johann-Wolfgang Goethe University Frankfurt, Frankfurt, Germany. nadja_treusch@klinikum-hanau.de FAU - van de Loo, S AU - van de Loo S FAU - Hattingen, E AU - Hattingen E LA - eng PT - Case Reports PT - Journal Article DEP - 20120529 PL - France TA - J Neuroradiol JT - Journal of neuroradiology. Journal de neuroradiologie JID - 7705086 RN - 0 (Gastrointestinal Agents) RN - 4618-18-2 (Lactulose) SB - IM MH - Cerebral Cortex/drug effects/*pathology

MH - Female MH - Gastrointestinal Agents/therapeutic use MH - Hepatic Encephalopathy/complications/*drug therapy/*pathology MH - Humans MH - Hyperammonemia/*drug therapy/etiology/*pathology MH - Lactulose/*therapeutic use MH - Magnetic Resonance Imaging/methods MH - Middle Aged MH - Neurons/drug effects/*pathology MH - Treatment Outcome EDAT- 2012/06/02 06:00 MHDA- 2013/11/16 06:00 CRDT- 2012/06/02 06:00 PHST- 2011/12/08 [received] PHST- 2012/03/01 [revised] PHST- 2012/03/02 [accepted] PHST- 2012/05/29 [aheadofprint] AID - S0150-9861(12)00162-9 [pii] AID - 10.1016/j.neurad.2012.03.002 [doi] PST - ppublish SO - J Neuroradiol. 2012 Dec;39(5):350-3. doi: 10.1016/j.neurad.2012.03.002. Ep ub 2012 May 29. PMIDOWN STATDA DCOMIS IS VI IP DP TI of ed controlled trial (the ACT NoW Study). PG - 1-160 LID - 10.3310/hta16260 [doi] AB - OBJECTIVE: To determine the clinical effectiveness, cost-effectiveness and service users' views of enhanced early communication therapy by speech and language (SL) therapists compared with attention control (AC). DESIGN: Suc cessful feasibility study followed by a randomised trial with economic evaluation, and nested qualitative study using 32 individual interviews. SETTING: Twelve E nglish NHS hospital and community stroke services. PARTICIPANTS: One hundred and seventy adults with aphasia or dysarthria admitted to hospital with stroke, Decemb er 2006 to January 2010. Eligibility determined by NHS SL therapists. Seventeen pe ople declined follow-up. INTERVENTIONS: A best-practice, flexible intervention by NHS SL therapists, up to three contacts per week for up to 16 weeks compared w ith a similar number of AC contacts by employed visitors. MAIN OUTCOME MEASURES: 22613690 NLM MEDLINE 20120522 20121018 2046-4924 (Electronic) 1366-5278 (Linking) 16 26 2012 May Clinical effectiveness, cost-effectiveness and service users' perceptions early, well-resourced communication therapy following a stroke: a randomis

Primary outcome was blinded, functional communicative ability 6 months pos t randomisation on the Therapy Outcome Measure activity subscale (TOM). Seco ndary outcomes were participants' perceptions on the Communication Outcomes Afte r Stroke scale (COAST); carers' perceptions of participants from part of the Carer COAST; carer well-being on Carers of Older People in Europe Index and quality-of-life items from Carer COAST. Serious adverse events (SAEs) were recorded. Economic evaluation: participants' utility (European Quality of Life-5 Dimensions), service use and cost data from medical records and carers, an d a discrete choice experiment. RESULTS: Intervention typically started after 2 weeks, providing 22 contacts. Both groups improved on the TOM. The estimat ed 6 months' group difference [95% confidence interval (CI)] was 0.25 (-0.19 to 0.69) points in favour of SL therapy. Sensitivity analyses adjusting for baselin e chance imbalance or not imputing values for decedents further reduced this difference. Per-protocol analyses rejected a possible dilution of therapy from controls refusing allocation and receiving NHS SL therapy. There was no ev idence of added benefit of therapy on any secondary outcome measure or SAEs, alth ough the latter were less frequent in the therapy group [odds ratio 0.42 (95% C I 0.16 to 1.1)]. Regardless of group allocation, interviewed participants reporte d positive impacts on their confidence and mood, identified drivers for chan ge and valued early and sustained contact. Health economic analysis indicated a h igh level of uncertainty. Early enhanced SL therapy for communication is likel y to be cost-effective only if decision-makers are prepared to pay >/= pound25,000 to gain one unit of utility. CONCLUSIONS: These findings exclude the possibil ity of a clinically significant difference of 0.5 points on the TOM. There was no evidence, on any measure, of added benefit of early communication therapy beyond that from AC. It is unclear whether therapy is more or less cost-effective than AC. Early, frequent contact was highly valued by users and had good uptake . Functional communication improved for both groups, plausibly due to natura l recovery and early and regular opportunity to practise everyday communicat ion with a professional (therapist/visitor). There is no evidence to recommend enhancing the provision of early communication therapy by a qualified SL therapist over and above usual care. SL therapy service reorganisation sho uld consider skill mix and timing within a stepped care model and should take place

within the context of a trial. FAU - Bowen, A AU - Bowen A AD - University of Manchester MAHSC (Manchester Academic Health Science Centre) , Manchester, UK. audrey.bowen@manchester.ac.uk FAU - Hesketh, A AU - Hesketh A FAU - Patchick, E AU - Patchick E FAU - Young, A AU - Young A FAU - Davies, L AU - Davies L FAU - Vail, A AU - Vail A FAU - Long, A AU - Long A FAU - Watkins, C AU - Watkins C FAU - Wilkinson, M AU - Wilkinson M FAU - Pearl, G AU - Pearl G FAU - Lambon Ralph, M AU - Lambon Ralph M FAU - Tyrrell, P AU - Tyrrell P CN - ACT NoW investigators LA - eng SI - ISRCTN/ISRCTN78617680 PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Health Technol Assess JT - Health technology assessment (Winchester, England) JID - 9706284 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Aphasia/therapy MH - Dysarthria/therapy MH - Feasibility Studies MH - Female MH - Great Britain MH - Humans MH - Interviews as Topic MH - Male MH - Middle Aged MH - Outcome Assessment (Health Care)/*methods MH - *Patient Satisfaction MH - Speech Therapy/*economics MH - State Medicine MH - Stroke/physiopathology/*rehabilitation EDAT- 2012/05/23 06:00 MHDA- 2012/10/19 06:00 CRDT- 2012/05/23 06:00 AID - 10.3310/hta16260 [doi]

PST - ppublish SO - Health Technol Assess. 2012 May;16(26):1-160. doi: 10.3310/hta16260. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB lly we describe the case of a 46-year-old patient with metastatic rectal carcinom a who received second-line therapy with irinotecan and developed isolated transi ent dysarthria (with normal MR imaging of the brain) following each administra tion of irinotecan. Neurological and logopedical evaluation revealed that the dysa rthria predominantly resulted from a reduced capacity in fine-tuning of motor fun ctions of the tip of the tongue and a minimal reduction in the power of speech at labiodental contact. As hypoglossal nerve activity has been reported to be especially susceptible to cholinergic stimulation and irinotecan can cause cholinergic side effects by binding to and inactivating acetylcholinestera se, we suspect this mechanism to be responsible for irinotecan-induced dysarthria . FAU - Dressel, Albertine J AU - Dressel AJ AD - Department of Medical Oncology, VU University Medical Center, Amsterdam, T he Netherlands. FAU - van der Mijn, Johannes C AU - van der Mijn JC FAU - Aalders, Ijke J AU - Aalders IJ FAU - Rinkel, Rico N P M AU - Rinkel RN FAU - van der Vliet, Hans J AU - van der Vliet HJ LA - eng PT - Journal Article DEP - 20120118 PL - Switzerland TA - Case Rep Oncol JT - Case reports in oncology JID - 101517601 PMC - PMC3290033 OID - NLM: PMC3290033 22379477 NLM PubMed-not-MEDLINE 20120301 20121002 20130529 1662-6575 (Electronic) 1662-6575 (Linking) 5 1 2012 Jan Irinotecan-induced dysarthria. 47-51 10.1159/000336156 [doi] Colorectal carcinomas are among the most common tumor types and are genera treated with palliative chemotherapy in case of metastatic disease. Here,

OTO OT OT OT EDATMHDACRDTPHSTAID AID PST SO 18.

NOTNLM Colorectal cancer Dysarthria Irinotecan 2012/03/02 06:00 2012/03/02 06:01 2012/03/02 06:00 2012/01/18 [epublish] 000336156 [doi] cro-0005-0047 [pii] ppublish Case Rep Oncol. 2012 Jan;5(1):47-51. doi: 10.1159/000336156. Epub 2012 Jan

PMID- 22295152 OWN - NLM STAT- MEDLINE DA - 20120201 DCOM- 20120524 LR - 20131014 IS - 1936-2625 (Electronic) IS - 1936-2625 (Linking) VI - 5 IP - 1 DP - 2012 TI - Rare case of a primary non-dural central nervous system low grade B-cell l ymphoma and literature review. PG - 89-95 AB - We present a case of a 70-year-old HIV negative man with a five-year histo ry of progressive dysnomia and new onset right extremity numbness, dysarthria, a nd blurry vision. On magnetic resonance imaging (MRI), an infiltrative enhanc ing tumor was noted. Follow up brain biopsy results revealed a small lymphocyt ic infiltrate with scattered plasma cells in a predominantly perivascular gro wth pattern. Flow-cytometric findings revealed a lambda monotypic B-cell popul ation. The morphology and the flow cytometric findings were consistent with invol vement by a low grade B-cell lymphoma. Subsequent positron emission tomography (P ET) studies along with bone marrow biopsy and serum protein electrophoresis sh owed no evidence of systemic disease. The above findings are consistent with invol vement by a non-dural extranodal marginal zone B-cell lymphoma (MZBCL) primary to the central nervous system (CNS). This is the first reported case of a primary CNS MZBCL with flow cytometric analysis. A review of literature on this rare e ntity is also included. FAU - Papanicolau-Sengos, Antonios AU - Papanicolau-Sengos A AD - Department of Pathology, University of California San Diego School of Medi cine

and Veteran Administration Medical Center of San Diego, 9500 Gilman Drive # 9113, La Jolla, CA 92093-9113, USA. FAU - Wang-Rodriguez, Jessica AU - Wang-Rodriguez J FAU - Wang, Huan-You AU - Wang HY FAU - Lee, Roland R AU - Lee RR FAU - Wong, Anna AU - Wong A FAU - Hansen, Lawrence A AU - Hansen LA FAU - Mahooti, Sepi AU - Mahooti S FAU - Rashidi, Hooman H AU - Rashidi HH LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20120101 PL - United States TA - Int J Clin Exp Pathol JT - International journal of clinical and experimental pathology JID - 101480565 SB - IM MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Central Nervous System Neoplasms/drug therapy/*pathology/physiopathology MH - Flow Cytometry MH - Humans MH - Lymphoma, B-Cell, Marginal Zone/drug therapy/*pathology/physiopathology MH - Male MH - Neoplasm Grading PMC - PMC3267491 OID - NLM: PMC3267491 OTO - NOTNLM OT - Primary Non-Dural Central Nervous System Low grade B-cell lymphoma OT - extranodal marginal zone lymphoma EDAT- 2012/02/02 06:00 MHDA- 2012/05/25 06:00 CRDT- 2012/02/02 06:00 PHST- 2011/11/20 [received] PHST- 2011/12/07 [accepted] PHST- 2012/01/01 [epublish] PST - ppublish SO - Int J Clin Exp Pathol. 2012;5(1):89-95. Epub 2012 Jan 1. PMIDOWN STATDA DCOMLR IS IS VI IP DP 22271664 NLM MEDLINE 20120227 20120409 20140319 1460-2156 (Electronic) 0006-8950 (Linking) 135 Pt 3 2012 Mar

TI PG LID AB d by of 2-3

A proposed staging system for amyotrophic lateral sclerosis. 847-52 10.1093/brain/awr351 [doi] Amyotrophic lateral sclerosis is a neurodegenerative disorder characterize progressive loss of upper and lower motor neurons, with a median survival

years. Although various phenotypic and research diagnostic classification systems exist and several prognostic models have been generated, there is no stagi ng system. Staging criteria for amyotrophic lateral sclerosis would help to p rovide a universal and objective measure of disease progression with benefits for patient care, resource allocation, research classifications and clinical t rial design. We therefore sought to define easily identified clinical milestone s that could be shown to occur at specific points in the disease course, reflect disease progression and impact prognosis and treatment. A tertiary referral centre clinical database was analysed, consisting of 1471 patients with amyotroph ic lateral sclerosis seen between 1993 and 2007. Milestones were defined as s ymptom onset (functional involvement by weakness, wasting, spasticity, dysarthria or dysphagia of one central nervous system region defined as bulbar, upper li mb, lower limb or diaphragmatic), diagnosis, functional involvement of a secon d region, functional involvement of a third region, needing gastrostomy and non-invasive ventilation. Milestone timings were standardized as proportio ns of time elapsed through the disease course using information from patients wh o had died by dividing time to a milestone by disease duration. Milestones occur red at predictable proportions of the disease course. Diagnosis occurred at 35% t hrough the disease course, involvement of a second region at 38%, a third region at 61%, need for gastrostomy at 77% and need for non-invasive ventilation at 80%. We therefore propose a simple staging system for amyotrophic lateral sclerosi s. Stage 1: symptom onset (involvement of first region); Stage 2A: diagnosis; Stage 2B: involvement of second region; Stage 3: involvement of third region; St age 4A: need for gastrostomy; and Stage 4B: need for non-invasive ventilation. Val idation of this staging system will require further studies in other populations, in population registers and in other clinic databases. The standardized times to milestones may well vary between different studies and populations, althou gh the stages themselves and their meanings are likely to remain unchanged. FAU - Roche, Jose C

AU - Roche JC AD - MRC Centre for Neurodegeneration Research, King's College London, Institut e of Psychiatry, London SE5 8AF, UK. FAU - Rojas-Garcia, Ricardo AU - Rojas-Garcia R FAU - Scott, Kirsten M AU - Scott KM FAU - Scotton, William AU - Scotton W FAU - Ellis, Catherine E AU - Ellis CE FAU - Burman, Rachel AU - Burman R FAU - Wijesekera, Lokesh AU - Wijesekera L FAU - Turner, Martin R AU - Turner MR FAU - Leigh, P Nigel AU - Leigh PN FAU - Shaw, Christopher E AU - Shaw CE FAU - Al-Chalabi, Ammar AU - Al-Chalabi A LA - eng GR - 089701/Wellcome Trust/United Kingdom GR - G0500289/Medical Research Council/United Kingdom GR - G0701923/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120123 PL - England TA - Brain JT - Brain : a journal of neurology JID - 0372537 SB - AIM SB - IM MH - Age of Onset MH - Aged MH - Amyotrophic Lateral Sclerosis/classification/diagnosis/*pathology MH - Cohort Studies MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Gastrostomy MH - Humans MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Models, Statistical MH - Patient Selection MH - Prognosis MH - Reproducibility of Results MH - Respiration, Artificial MH - Sex Factors MH - Survival Analysis PMC - PMC3286327 OID - NLM: PMC3286327 EDAT- 2012/01/25 06:00 MHDA- 2012/04/10 06:00

CRDTPHSTAID AID PST SO 23. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB resis

2012/01/25 06:00 2012/01/23 [aheadofprint] awr351 [pii] 10.1093/brain/awr351 [doi] ppublish Brain. 2012 Mar;135(Pt 3):847-52. doi: 10.1093/brain/awr351. Epub 2012 Jan 22247979 NLM MEDLINE 20120806 20121210 20131121 2000-1967 (Electronic) 0300-9734 (Linking) 117 3 2012 Aug A case of hypoglycemic hemiparesis and literature review. 347-51 10.3109/03009734.2011.652748 [doi] An 89-year-old man with diabetes treated with metformin 500 mg/day and glimepiride 4 mg/day was hospitalized because of hypoglycemic right hemipa and dysarthria (casual glucose value 1.8 mmol/L), which resolved quickly following administration of 40 mL of 40% dextrose. Hemiparesis is a rare s

ymptom (4.2%) of hypoglycemia. There are about 200 case reports of hypoglycemic hemiparesis. The average glucose level at which hemiparesis developed was 1.8 mmol/L. Right-sided hemiparesis predominated (R 66%; L 34%). On imaging st udies, abnormal findings were frequently observed in the internal capsule or sple nium of the corpus callosum. The mechanism of hemiparesis is not fully understood. The existence of cases in which hypoglycemia cannot be distinguished from stro ke on imaging studies suggests the importance of measurement of the blood glucos e level when the symptoms of stroke are first recognized. FAU - Yoshino, Tetsuhiro AU - Yoshino T AD - Division of Endocrinology, Metabolism and Nephrology Department of Interna l Medicine Keio University, School of Medicine, Shinjuku, Japan. tetta213@yahoo.co.jp FAU - Meguro, Shu AU - Meguro S FAU - Soeda, Yukie AU - Soeda Y FAU - Itoh, Arata AU - Itoh A FAU - Kawai, Toshihide AU - Kawai T FAU - Itoh, Hiroshi AU - Itoh H LA - eng PT - Case Reports

PT PT DEP PL TA JT JID RN RN RN RN SB MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTPHSTAID PST SO Epub PMIDOWN STATDA DCOMIS IS VI IP DP TI onian

Journal Article Review 20120117 England Ups J Med Sci Upsala journal of medical sciences 0332203 0 (Blood Glucose) 0 (Sulfonylurea Compounds) 9100L32L2N (Metformin) 93479-97-1 (glimepiride) IM Aged Aged, 80 and over Blood Glucose/analysis Diabetes Mellitus/drug therapy Humans Hypoglycemia/*complications Male Metformin/administration & dosage/therapeutic use Paresis/*diagnosis/etiology Sulfonylurea Compounds/administration & dosage/therapeutic use PMC3410296 NLM: PMC3410296 2012/01/18 06:00 2012/12/12 06:00 2012/01/18 06:00 2012/01/17 [aheadofprint] 10.3109/03009734.2011.652748 [doi] ppublish Ups J Med Sci. 2012 Aug;117(3):347-51. doi: 10.3109/03009734.2011.652748. 2012 Jan 17. 22142042 NLM MEDLINE 20111206 20120403 2146-8427 (Electronic) 1304-0855 (Linking) 9 6 2011 Dec Transient improvement of acquired hepatocerebral degeneration with parkins

symptoms after failed liver transplant: case report and literature review. PG - 363-9 AB - OBJECTIVES: Acquired (non-Wilsonian) hepato-cerebral degeneration is an infrequent neurologic disorder in patients with liver dysfunction and longstanding portal-systemic shunting. The clinical manifestations include dysarthria, ataxia, tremor, and cognitive dysfunction. Typically, patients with acquired hepatocerebral degeneration respond poorly to medical therapy as the underlying end-stage liver disease remains. Information regarding the effe ct of orthotopic liver transplant on acquired hepatocerebral degeneration, howev er, is limited and conflicting. MATERIALS AND METHODS: We conducted a review of

literature via a PubMed search to summarize the effect of orthotopic liver transplant on acquired hepatocerebral degeneration. RESULTS: We present a 56-year-old man with compensated hepatitis C cirrhosis who developed acqui red hepatocerebral degeneration with Parkinsonian symptoms refractory to conve ntional Parkinson medical therapy. Orthotopic liver transplant led to marked clini cal improvement of the acquired hepatocerebral degeneration. However, the pati ent developed recurrence of acquired hepatocerebral degeneration 6-week postorthotopic liver transplant as he developed graft failure from aggress ive progressive hepatitis C recurrence. Our review found a heterogeneous group of case series, suggesting that the experience with orthotopic liver transpla nt is variable. CONCLUSIONS: Our experience demonstrates that orthotopic liver transplant may lead to resolution of acquired hepatocerebral degeneration; however, acquired hepatocerebral degeneration may return with recurrent li ver disease. Future studies with long-term follow-up are needed. FAU - Chen, Yuanyuan AU - Chen Y AD - Department of Medicine, University of British Columbia, Vancouver, BC, Can ada. FAU - Haque, Mazhar AU - Haque M FAU - Yoshida, Eric M AU - Yoshida EM LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Turkey TA - Exp Clin Transplant JT - Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation JID - 101207333 RN - 0 (Antiparkinson Agents) RN - 0 (Antiviral Agents) SB - IM MH - Antiparkinson Agents/therapeutic use MH - Antiviral Agents/therapeutic use MH - End Stage Liver Disease/etiology/*surgery MH - Fatal Outcome MH - Graft Rejection/*etiology MH - Hepatitis C/*complications/diagnosis/drug therapy MH - Hepatolenticular Degeneration/diagnosis/etiology/*surgery MH - Humans MH - Liver Cirrhosis/etiology/*surgery MH - Liver Transplantation/*adverse effects MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Parkinsonian Disorders/diagnosis/drug therapy/etiology/*surgery MH - Recurrence MH - Treatment Failure EDAT- 2011/12/07 06:00

MHDACRDTPST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI PG AB cally in 180

2012/04/04 06:00 2011/12/07 06:00 ppublish Exp Clin Transplant. 2011 Dec;9(6):363-9. 22123476 NLM MEDLINE 20111129 20120425 1349-8029 (Electronic) 0470-8105 (Linking) 51 11 2011 Complications of subthalamic nucleus stimulation in Parkinson's disease. 749-55 Subthalamic nucleus deep brain stimulation (STN-DBS) is effective for medi refractory Parkinson's disease. We retrospectively analyzed complications consecutive patients who underwent bilateral STN-DBS. Surgery-related complications were symptomatic intracerebral hemorrhage in 2, chronic subd

ural hematoma in 1, and transient deterioration of medication-induced psychosis in 2 patients. Device-related complications involved device infection in 5, ski n erosion in 5, and implantable pulse generator malfunction in 2 patients. A ll of these patients required surgical repair. Surgery and device-related compli cations could be reduced with increased surgical experience and the introduction o f new surgical equipment and technology. Treatment or stimulation-related compli cations were intractable dyskinesia/dystonia in 11, problematic dysarthria in 7, a praxia of eyelid opening (ALO) in 11, back pain in 10, and restless leg syndrome in 6 patients. Neuropsychiatric complications were transient mood changes in so me, impulse control disorder in 2, severe depression related to excessive redu ction of dopaminergic medications in 2, rapid progression of dementia in 1, and suicide attempts in 2 patients. Most complications were mild and transient. Dysart hria and ALO were the most frequent permanent sequelae after STN-DBS. Treatment-related adverse events may be caused not only by the effect of stimulation effect but also excessive reduction of dopaminergic medication , or progression of the disease. In conclusion, STN-DBS seems to be a relativel y safe procedure. Although serious complications with permanent sequelae are rare , significant incidences of adverse effects occur. Physicians engaged in thi s treatment should have a comprehensive understanding of the probable compli cations

and how to avoid them. FAU - Umemura, Atsushi AU - Umemura A AD - Department of Neurosurgery, Nagoya City University Graduate School of Medi cine, Aichi. aume@med.nagoya-cu.ac.jp FAU - Oka, Yuichi AU - Oka Y FAU - Yamamoto, Kenichi AU - Yamamoto K FAU - Okita, Kenji AU - Okita K FAU - Matsukawa, Noriyuki AU - Matsukawa N FAU - Yamada, Kazuo AU - Yamada K LA - eng PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Apraxias/*etiology/pathology MH - Cohort Studies MH - Deep Brain Stimulation/*adverse effects MH - Dysarthria/*etiology MH - Dyskinesias/*etiology MH - Eyelid Diseases/etiology/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Mental Disorders/etiology MH - Middle Aged MH - Parkinson Disease/*therapy MH - Retrospective Studies EDAT- 2011/11/30 06:00 MHDA- 2012/04/26 06:00 CRDT- 2011/11/30 06:00 AID - JST.JSTAGE/nmc/51.749 [pii] PST - ppublish SO - Neurol Med Chir (Tokyo). 2011;51(11):749-55. PMID- 22078654 OWN - NLM STAT- MEDLINE DA - 20130507 DCOM- 20131219 IS - 1578-1968 (Electronic) IS - 0213-4853 (Linking) VI - 28 IP - 4 DP - 2013 May TI - Acute persistent dysarthria and dizziness with previous neurological study : is it enough to establish a diagnosis?

PG LID LID FAU AU FAU AU FAU AU FAU AU LA LA PT PT DEP PL TA JT JID RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTPHSTPHSTPHSTAID AID PST SO 1 Nov PMIDOWN STATDA DCOMLR IS IS VI IP -

250-2 10.1016/j.nrl.2011.09.001 [doi] S0213-4853(11)00394-X [pii] Fernandez Dominguez, J Fernandez Dominguez J Garcia Rodriguez, R Garcia Rodriguez R Vega, P Vega P Calleja Puerta, S Calleja Puerta S eng spa Case Reports Letter 20111109 Spain Neurologia Neurologia (Barcelona, Spain) 9005460 0 (Adrenergic beta-Antagonists) IM Adrenergic beta-Antagonists/therapeutic use Cerebral Angiography Dizziness/*diagnosis/*etiology/ultrasonography Dysarthria/*diagnosis/*etiology/ultrasonography Gait Disorders, Neurologic/diagnosis/etiology Humans Hypertension/complications/drug therapy Magnetic Resonance Angiography Male Middle Aged Nervous System Diseases/*complications/*diagnosis/ultrasonography Neurologic Examination Smoking/adverse effects Ultrasonography, Doppler, Transcranial 2011/11/15 06:00 2013/12/20 06:00 2011/11/15 06:00 2011/05/19 [received] 2011/09/12 [revised] 2011/09/15 [accepted] 2011/11/09 [aheadofprint] S0213-4853(11)00394-X [pii] 10.1016/j.nrl.2011.09.001 [doi] ppublish Neurologia. 2013 May;28(4):250-2. doi: 10.1016/j.nrl.2011.09.001. Epub 201 9. 22022010 NLM PubMed-not-MEDLINE 20111024 20111110 20130529 1998-3751 (Electronic) 0253-7613 (Linking) 43 5

DP - 2011 Sep TI - Quadriparesis and dysarthria due to tetrabenazine therapy in a child with rheumatic chorea. PG - 601-2 LID - 10.4103/0253-7613.84982 [doi] AB - Tetrabenazine (TBZ) is widely used to treat hyperkinetic movement disorder s in adults; however, published experience with the drug in children is limited . Common side effects of TBZ include drowsiness, sedation, weakness, Parkins onism, depression, and acute akathisia, all of which are reversible with decrease d doses. We report here a 7-year-old girl with rheumatic chorea who develope d acute akinesia of all four limbs and dysarthria due to TBZ therapy. Withdrawal o f the drug led to rapid improvement within 18 hours. FAU - Zaki, Syed Ahmed AU - Zaki SA AD - Department of Pediatrics, Lokmanya Tilak Municipal General Hospital and Me dical College, Sion, Mumbai - 400 022, India. FAU - Lad, Vijay AU - Lad V FAU - Shanbag, Preeti AU - Shanbag P LA - eng PT - Journal Article PL - India TA - Indian J Pharmacol JT - Indian journal of pharmacology JID - 7902477 PMC - PMC3195137 OID - NLM: PMC3195137 OTO - NOTNLM OT - Dysarthria OT - parkinsonism OT - quadriparesis OT - rheumatic chorea OT - tetrabenazine EDAT- 2011/10/25 06:00 MHDA- 2011/10/25 06:01 CRDT- 2011/10/25 06:00 PHST- 2011/01/28 [received] PHST- 2011/02/16 [revised] PHST- 2011/07/01 [accepted] AID - 10.4103/0253-7613.84982 [doi] AID - IJPharm-43-601 [pii] PST - ppublish SO - Indian J Pharmacol. 2011 Sep;43(5):601-2. doi: 10.4103/0253-7613.84982. PMIDOWN STATDA DCOMLR IS IS 22004192 NLM MEDLINE 20111129 20120413 20131212 1939-1277 (Electronic) 0096-1523 (Linking)

VI IP DP TI PG LID AB tive

37 6 2011 Dec Word recognition reflects dimension-based statistical learning. 1939-56 10.1037/a0025641 [doi] Speech processing requires sensitivity to long-term regularities of the na

language yet demands listeners to flexibly adapt to perturbations that ari se from talker idiosyncrasies such as nonnative accent. The present experiments investigate whether listeners exhibit dimension-based statistical learning of correlations between acoustic dimensions defining perceptual space for a g iven speech segment. While engaged in a word recognition task guided by a perce ptually unambiguous voice-onset time (VOT) acoustics to signal beer, pier, deer, o r tear, listeners were exposed incidentally to an artificial "accent" deviating fr om English norms in its correlation of the pitch onset of the following vowel (F0) to VOT. Results across four experiments are indicative of rapid, dimension -based statistical learning; reliance on the F0 dimension in word recognition was rapidly down-weighted in response to the perturbation of the correlation b etween F0 and VOT dimensions. However, listeners did not simply mirror the shortterm input statistics. Instead, response patterns were consistent with a linger ing influence of sensitivity to the long-term regularities of English. This su ggests that the very acoustic dimensions defining perceptual space are not fixed and, rather, are dynamically and rapidly adjusted to the idiosyncrasies of loca l experience, such as might arise from nonnative-accent, dialect, or dysarth ria. The current findings extend demonstrations of "object-based" statistical l earning across speech segments to include incidental, online statistical learning of regularities residing within a speech segment. FAU - Idemaru, Kaori AU - Idemaru K AD - Department of East Asian Languages and Literatures, University of Oregon, Eugene, OR 97403, USA. idemaru@uoregon.edu FAU - Holt, Lori L AU - Holt LL LA - eng GR - R01 DC004674/DC/NIDCD NIH HHS/United States GR - R01 DC004674-11/DC/NIDCD NIH HHS/United States GR - R01DC004674/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S.

DEP - 20111017 PL - United States TA - J Exp Psychol Hum Percept Perform JT - Journal of experimental psychology. Human perception and performance JID - 7502589 SB - IM MH - Acoustic Stimulation MH - Humans MH - *Learning MH - Psycholinguistics MH - Speech MH - *Speech Perception MH - Time Factors PMC - PMC3285244 MID - NIHMS356161 OID - NLM: NIHMS356161 OID - NLM: PMC3285244 EDAT- 2011/10/19 06:00 MHDA- 2012/04/14 06:00 CRDT- 2011/10/19 06:00 PHST- 2011/10/17 [aheadofprint] AID - 2011-23770-001 [pii] AID - 10.1037/a0025641 [doi] PST - ppublish SO - J Exp Psychol Hum Percept Perform. 2011 Dec;37(6):1939-56. doi: 10.1037/a0 025641. Epub 2011 Oct 17. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 21976947 NLM MEDLINE 20111006 20120202 20131016 1011-8942 (Print) 1011-8942 (Linking) 25 5 2011 Oct Ophthalmic artery obstruction and cerebral infarction following periocular injection of autologous fat. PG - 358-61 LID - 10.3341/kjo.2011.25.5.358 [doi] AB - We report a case of ophthalmic artery obstruction combined with brain infa rction following periocular autologous fat injection. The patient, a 44-year-old woman, visited our hospital for decreased visual acuity in her left eye and dysar thria one hour after receiving an autologous fat injection in the periocular are a. Her best corrected visual acuity for the concerned eye was no light perception . Also, a relative afferent pupillary defect was detected in this eye. The left fu ndus exhibited widespread retinal whitening with visible emboli in several reti nal arterioles. Diffusion-weighted magnetic resonance imaging of the brain sho wed a hyperintense lesion at the left insular cortex. Therefore, we diagnosed

ophthalmic artery obstruction and left middle cerebral artery infarction d ue to fat emboli. The patient was managed with immediate ocular massage, carbon dioxide, and oxygen therapy. Following treatment, dysarthria improved considerably but there was no improvement in visual acuity. FAU - Lee, Chang Mok AU - Lee CM AD - Department of Ophthalmology, Kangdong Sacred Heart Hospital, Hallym Univer sity College of Medicine, Seoul, Korea. FAU - Hong, In Hwan AU - Hong IH FAU - Park, Sung Pyo AU - Park SP LA - eng PT - Case Reports PT - Journal Article DEP - 20110920 PL - Korea (South) TA - Korean J Ophthalmol JT - Korean journal of ophthalmology : KJO JID - 8804513 SB - IM MH - Adult MH - Arterial Occlusive Diseases/diagnosis/*etiology MH - Female MH - Fluorescein Angiography MH - Follow-Up Studies MH - Fundus Oculi MH - Humans MH - Infarction, Middle Cerebral Artery/*complications/diagnosis MH - Magnetic Resonance Imaging MH - *Ophthalmic Artery MH - Orbit MH - Subcutaneous Fat/*transplantation MH - Transplantation, Autologous/adverse effects MH - Visual Acuity PMC - PMC3178774 OID - NLM: PMC3178774 OTO - NOTNLM OT - Abdominal fat OT - Cerebral infarction OT - Ophthalmic artery OT - Retinal artery occlusion EDAT- 2011/10/07 06:00 MHDA- 2012/02/03 06:00 CRDT- 2011/10/07 06:00 PHST- 2010/04/28 [received] PHST- 2010/08/16 [accepted] PHST- 2011/09/20 [epublish] AID - 10.3341/kjo.2011.25.5.358 [doi] PST - ppublish SO - Korean J Ophthalmol. 2011 Oct;25(5):358-61. doi: 10.3341/kjo.2011.25.5.358 . Epub 2011 Sep 20. PMIDOWN STATDA 21969916 NLM PubMed-not-MEDLINE 20111004

DCOM- 20111110 LR - 20120507 IS - 2042-0080 (Electronic) IS - 2042-0080 (Linking) VI - 2011 DP - 2011 TI - Assessment of prosodic communicative efficiency in Parkinson's disease as judged by professional listeners. PG - 129310 LID - 10.4061/2011/129310 [doi] AB - This study examines the impact of Parkinson's disease (PD) on communicativ e efficiency conveyed through prosody. A new assessment method for evaluatin g productive prosodic skills in Dutch speaking dysarthric patients was devis ed and tested on 36 individuals (18 controls, 18 PD patients). Three professional listeners judged the intended meanings in four communicative functions of Dutch prosody: Boundary Marking, Focus, Sentence Typing, and Emotional Prosody. Each function was tested through reading and imitation. Interrater agreement wa s calculated. Results indicated that healthy speakers, compared to PD patien ts, performed significantly better on imitation of Boundary Marking, Focus, an d Sentence Typing. PD patients with a moderate or severe dysarthria performe d significantly worse on imitation of Focus than on reading of Focus. No significant differences were found for Emotional Prosody. Judges agreed we ll on all tasks except Emotional Prosody. Future research will focus on elaborat ing the assessment and on developing a therapy programme paralleling the assessmen t. FAU - Martens, Heidi AU - Martens H AD - Rehabilitation Centre for Communication Disorders, Antwerp University Hosp ital, Wilrijkstraat 10, 2650 Edegem, Belgium. FAU - Van Nuffelen, Gwen AU - Van Nuffelen G FAU - Cras, Patrick AU - Cras P FAU - Pickut, Barbara AU - Pickut B FAU - De Letter, Miet AU - De Letter M FAU - De Bodt, Marc AU - De Bodt M LA - eng PT - Journal Article DEP - 20110928 PL - United States TA - Parkinsons Dis JT - Parkinson's disease JID - 101539877 PMC - PMC3182398

OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO 28.

NLM: PMC3182398 2011/10/05 06:00 2011/10/05 06:01 2011/10/05 06:00 2011/04/01 [received] 2011/07/03 [revised] 2011/07/23 [accepted] 2011/09/28 [epublish] 10.4061/2011/129310 [doi] ppublish Parkinsons Dis. 2011;2011:129310. doi: 10.4061/2011/129310. Epub 2011 Sep

PMID- 21953693 OWN - NLM STAT- MEDLINE DA - 20111123 DCOM- 20120405 LR - 20131016 IS - 1531-8257 (Electronic) IS - 0885-3185 (Linking) VI - 26 IP - 13 DP - 2011 Nov TI - Treatment of dysarthria following subthalamic nucleus deep brain stimulati on for Parkinson's disease. PG - 2434-6 LID - 10.1002/mds.23887 [doi] AB - BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is an established treatment for patients with Parkinson's disease (PD). Speech impairment is a frequent side effect of the surgery. This study examined t he efficacy of an intensive speech treatment, the Lee Silverman Voice Treatme nt (LSVT) on dysarthria after STN-DBS. METHODS: The LSVT was administered to 10 patients with STN-DBS (surgical group) and 10 patients without (medical gr oup). Patients were assessed before, immediately after, and 6 months following t he speech treatment using sustained phonation, a speech intelligibility scale , and monologue. Vocal loudness, speech intelligibility, and perceptual ratings were the primary outcome measures. RESULTS: Vocal loudness and perceptual score s improved significantly across tasks for the medical group only. Speech intelligibility did not significantly change for either group. Results in the surgical group were variable, with some patients deteriorating. CONCLUSION S: Treatment of dysarthria following STN-DBS needs further investigation beca use of the variable response to LSVT. CI - Copyright (c) 2011 Movement Disorder Society. FAU - Tripoliti, Elina AU - Tripoliti E AD - Sobell Department, Unit of Functional Neurosurgery, UCL, Institute of Neur

ology, FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA GR GR GR GR PT PT PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO 27. PMIDOWN STATDA Queen Square, London, United Kingdom. e.tripoliti@ion.ucl.ac.uk Strong, Laura Strong L Hickey, Freya Hickey F Foltynie, Tom Foltynie T Zrinzo, Ludvic Zrinzo L Candelario, Joseph Candelario J Hariz, Marwan Hariz M Limousin, Patricia Limousin P eng G-4070/Parkinson's UK/United Kingdom R01 NS040902-10/NS/NINDS NIH HHS/United States R01-NS40902/NS/NINDS NIH HHS/United States Department of Health/United Kingdom Comparative Study Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 20110927 United States Mov Disord Movement disorders : official journal of the Movement Disorder Society 8610688 IM Deep Brain Stimulation/*adverse effects *Dysarthria/etiology/physiopathology/therapy Humans Middle Aged Parkinson Disease/*therapy Severity of Illness Index Speech Therapy/*methods Subthalamic Nucleus/physiology/surgery Treatment Outcome PMC3223328 NIHMS308492 NLM: NIHMS308492 NLM: PMC3223328 2011/09/29 06:00 2012/04/06 06:00 2011/09/29 06:00 2010/07/14 [received] 2011/06/21 [revised] 2011/06/29 [accepted] 2011/09/27 [aheadofprint] 10.1002/mds.23887 [doi] ppublish Mov Disord. 2011 Nov;26(13):2434-6. doi: 10.1002/mds.23887. Epub 2011 Sep 21952405 NLM MEDLINE 20111123

DCOM- 20120224 LR - 20131016 IS - 1421-9972 (Electronic) IS - 1021-7762 (Linking) VI - 64 IP - 1 DP - 2012 TI - Effect of level of presentation to listeners on scaled speech intelligibil ity of speakers with dysarthria. PG - 26-33 LID - 10.1159/000328642 [doi] AB - OBJECTIVE: This study examined the effect of intensity level of presentati on on scaling of speech intelligibility in speakers with and without dysarthria. PATIENTS AND METHODS: A total of 50 utterances produced by speakers with dysarthria and healthy speakers were played to 60 listeners in four condit ions, which consisted of two different presentation levels ('high' vs. 'low') an d equalization of levels across utterances ('adjusted' vs. 'unadjusted'). Sp eech intelligibility was scaled by using a direct magnitude estimation techniqu e with and without modulus. RESULTS: A significant decrease in speech intelligibi lity was indicated when the stimuli were adjusted to have fixed intensity on th e most intense vocalic nuclei of each word, while no significant change was found between 'high' and 'low' presentation level conditions. CONCLUSION: The fi ndings suggest that an increase in presentation level alone does not result in significant improvement in speech intelligibility ratings. The results are discussed by considering clinical implications in conducting speech therap y with emphasis on intensity variation. FAU - Kim, Yunjung AU - Kim Y AD - Department of Communication Sciences and Disorders, Louisiana State Univer sity, Baton Rouge, LA, USA. FAU - Kuo, Christina AU - Kuo C LA - eng GR - DC00319/DC/NIDCD NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PL - Switzerland TA - Folia Phoniatr Logop JT - Folia phoniatrica et logopaedica : official organ of the International Association of Logopedics and Phoniatrics (IALP) JID - 9422792 SB - IM MH - Adult MH - Dysarthria/etiology/*psychology MH - Humans MH - *Loudness Perception MH - Multiple System Atrophy/complications MH - Observer Variation

MH MH MH MH MH PMC OID EDATMHDACRDTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI DP TI -

Parkinson Disease/complications *Speech Intelligibility Stroke/complications Voice Quality Young Adult PMC3221263 NLM: PMC3221263 2011/09/29 06:00 2012/03/01 06:00 2011/09/29 06:00 2011/09/22 [aheadofprint] 000328642 [pii] 10.1159/000328642 [doi] ppublish Folia Phoniatr Logop. 2012;64(1):26-33. doi: 10.1159/000328642.

21918728 NLM PubMed-not-MEDLINE 20110915 20111110 20130513 2042-0080 (Electronic) 2042-0080 (Linking) 2011 2011 A cognitive-perceptual approach to conceptualizing speech intelligibility deficits and remediation practice in hypokinetic dysarthria. PG - 150962 LID - 10.4061/2011/150962 [doi] AB - Hypokinetic dysarthria is a common manifestation of Parkinson's disease, w hich negatively influences quality of life. Behavioral techniques that aim to i mprove speech intelligibility constitute the bulk of intervention strategies for this population, as the dysarthria does not often respond vigorously to medical interventions. Although several case and group studies generally support t he efficacy of behavioral treatment, much work remains to establish a rigorou s evidence base. This absence of definitive research leaves both the speech-language pathologist and referring physician with the task of deter mining the feasibility and nature of therapy for intelligibility remediation in P D. The purpose of this paper is to introduce a novel framework for medical practi tioners in which to conceptualize and justify potential targets for speech remedia tion. The most commonly targeted deficits (e.g., speaking rate and vocal loudnes s) can be supported by this approach, as well as underutilized and novel treatmen t targets that aim at the listener's perceptual skills. FAU - Lansford, Kaitlin L AU - Lansford KL AD - Motor Speech Disorders Laboratory, Department of Speech and Hearing Scienc e, Arizona State University, P.O. Box 870102, Tempe, AZ 85287-0102, USA.

FAU AU FAU AU FAU AU LA GR GR PT DEP PL TA JT JID PMC OID EDATMHDACRDTPHSTPHSTPHSTPHSTAID PST SO 12. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB ith a 1

Liss, Julie M Liss JM Caviness, John N Caviness JN Utianski, Rene L Utianski RL eng R01 DC006859/DC/NIDCD NIH HHS/United States R01 DC006859-08/DC/NIDCD NIH HHS/United States Journal Article 20110912 United States Parkinsons Dis Parkinson's disease 101539877 PMC3171761 NLM: PMC3171761 2011/09/16 06:00 2011/09/16 06:01 2011/09/16 06:00 2011/04/09 [received] 2011/06/14 [revised] 2011/07/13 [accepted] 2011/09/12 [epublish] 10.4061/2011/150962 [doi] ppublish Parkinsons Dis. 2011;2011:150962. doi: 10.4061/2011/150962. Epub 2011 Sep 21837541 NLM MEDLINE 20111206 20120329 20131016 1573-7373 (Electronic) 0167-594X (Linking) 106 2 2012 Jan Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. 431-5 10.1007/s11060-011-0678-2 [doi] We observed a patient with subcutaneous seeding from gliomatosis cerebri w low-grade histopathology. A 33-year-old woman with neurofibromatosis type presented with progressive headache, diplopia, dysphagia, and a rightward instability. On neurological examination dysarthria, gait ataxia, and left

-sided central facial and hypoglossal palsies were determined. MRI of the brain demonstrated diffuse, infiltrative non-enhancing lesions in the pons, both cerebellar hemispheres, the parahippocampal gyrus, and the thalamus. A stereotactic biopsy demonstrated an astrocytoma WHO grade 2. These characteristics confirmed gliomatosis cerebri. Three months later, the pat ient presented with hydrocephalus and a subcutaneous swelling directly undernea th the surgical scar. The subcutaneous swelling was removed and the hydrocephalus was

treated by ventriculoperitoneal shunting. Histopathological examination co nfirmed a subcutaneous manifestation of low-grade oligoastrocytoma. Gliomatosis ce rebri with low-grade histology can seed subcutaneously. FAU - Buis, Dennis R AU - Buis DR FAU - van der Valk, Paul AU - van der Valk P FAU - De Witt Hamer, Philip C AU - De Witt Hamer PC LA - eng PT - Case Reports PT - Letter DEP - 20110812 PL - United States TA - J Neurooncol JT - Journal of neuro-oncology JID - 8309335 SB - IM MH - Adult MH - Biopsy MH - Brain Neoplasms/complications/pathology/*surgery MH - Female MH - Humans MH - Neoplasm Grading MH - *Neoplasm Seeding MH - Neoplasms, Neuroepithelial/complications/pathology/*surgery MH - Neurofibromatosis 1/complications MH - Neuronavigation/*adverse effects MH - Scalp/pathology MH - Subcutaneous Tissue/*pathology PMC - PMC3230756 OID - NLM: PMC3230756 EDAT- 2011/08/13 06:00 MHDA- 2012/03/30 06:00 CRDT- 2011/08/13 06:00 PHST- 2011/04/17 [received] PHST- 2011/07/30 [accepted] PHST- 2011/08/12 [aheadofprint] AID - 10.1007/s11060-011-0678-2 [doi] PST - ppublish SO - J Neurooncol. 2012 Jan;106(2):431-5. doi: 10.1007/s11060-011-0678-2. Epub 2011 Aug 12. PMIDOWN STATDA DCOMIS IS VI IP DP TI ed by 21701107 NLM MEDLINE 20110624 20111122 1349-8029 (Electronic) 0470-8105 (Linking) 51 6 2011 Partially thrombosed vertebral artery aneurysm with wall enhancement treat

stent-assisted coil embolization. PG - 431-3

AB - A 50-year-old man presented with a 2-month history of dysarthria caused by a partially thrombosed vertebral artery (VA) aneurysm. Magnetic resonance im aging showed enhancement of the thickened wall and angiography detected staining . Stent-assisted coil embolization with protection of the parent artery pate ncy was performed. The patient's clinical course was unremarkable and shrinking of the aneurysm was obtained. The stent-assisted coil embolization promoted intra-aneurysm flow disruption and stabilized the wall environment, sugges ting another strategy for the treatment of partially thrombosed VA aneurysm. FAU - Abe, Toshi AU - Abe T AD - Department of Radiology, Kurume University School of Medicine, Fukuoka, Ja pan. toshiabe@med.kurume-u.ac.jp FAU - Hagihara, Naoshi AU - Hagihara N FAU - Hirohata, Masaru AU - Hirohata M FAU - Uchiyama, Yusuke AU - Uchiyama Y FAU - Tanaka, Norimitsu AU - Tanaka N FAU - Hayabuchi, Naofumi AU - Hayabuchi N LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 SB - IM MH - Cerebral Angiography MH - Embolization, Therapeutic/*methods MH - Humans MH - Intracranial Aneurysm/pathology/*therapy MH - Intracranial Thrombosis/pathology/*therapy MH - Magnetic Resonance Angiography MH - Male MH - Middle Aged MH - *Stents MH - Treatment Outcome MH - Vertebral Artery/*pathology EDAT- 2011/06/28 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/06/25 06:00 AID - JST.JSTAGE/nmc/51.431 [pii] PST - ppublish SO - Neurol Med Chir (Tokyo). 2011;51(6):431-3. PMIDOWN STATDA DCOM21673467 NLM MEDLINE 20110615 20111014

IS IS VI IP DP TI e

1349-7235 (Electronic) 0918-2918 (Linking) 50 12 2011 Recurrent Hashimoto's encephalopathy, showing spontaneous remission: a cas

report. PG - 1309-12 AB - Hashimoto's encephalopathy (HE) is a rare condition associated with Hashim oto's thyroiditis (HT). It is characterized by neurological/psychiatric symptoms , high levels of anti-thyroid antibodies, non-specific radiological examinations or electroencephalogram abnormalities, and responsiveness to corticosteroid treatment. We describe the case of a man with HE who showed decreased ment ality, cognitive impairment, dysarthria, and gait disturbance. The initial attack was improved rapidly by corticosteroid treatment. When the symptoms recurred i n 7 months, the patient achieved spontaneous remission without corticosteroid treatment. The recognition of the condition was essential for the prognosi s and treatment of this rare disease. FAU - Li, Lin AU - Li L AD - Department of Endocrinology, the Affiliated Sir Run Run Shaw Hospital, Sch ool of Medicine, Zhejiang University, China. lihongheyi@126.com FAU - Zheng, Fen-Ping AU - Zheng FP FAU - Wang, Guoxing AU - Wang G FAU - Li, Hong AU - Li H LA - eng PT - Case Reports PT - Journal Article DEP - 20110615 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Autoantibodies) RN - Hashimoto's encephalitis SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Aged MH - Autoantibodies/blood MH - Brain/pathology MH - Brain Diseases/diagnosis/drug therapy/*etiology MH - Cognition Disorders/etiology MH - Dysarthria/etiology MH - Electroencephalography MH - Gait Disorders, Neurologic/etiology MH - Hashimoto Disease/diagnosis/drug therapy/*etiology MH - Humans

MH MH MH MH MH EDATMHDACRDTPHSTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI DP TI d

Magnetic Resonance Imaging Male Prognosis Recurrence Remission, Spontaneous 2011/06/16 06:00 2011/10/15 06:00 2011/06/16 06:00 2011/06/15 [epublish] JST.JSTAGE/internalmedicine/50.4966 [pii] ppublish Intern Med. 2011;50(12):1309-12. Epub 2011 Jun 15. 21619691 NLM MEDLINE 20110627 20111018 20131017 1750-1172 (Electronic) 1750-1172 (Linking) 6 2011 Autosomal dominant cerebellar ataxia type I: a review of the phenotypic an

genotypic characteristics. PG - 33 LID - 10.1186/1750-1172-6-33 [doi] AB - Type I autosomal dominant cerebellar ataxia (ADCA) is a type of spinocereb ellar ataxia (SCA) characterized by ataxia with other neurological signs, includ ing oculomotor disturbances, cognitive deficits, pyramidal and extrapyramidal dysfunction, bulbar, spinal and peripheral nervous system involvement. The global prevalence of this disease is not known. The most common type I ADCA is SC A3 followed by SCA2, SCA1, and SCA8, in descending order. Founder effects no doubt contribute to the variable prevalence between populations. Onset is usuall y in adulthood but cases of presentation in childhood have been reported. Clini cal features vary depending on the SCA subtype but by definition include ataxi a associated with other neurological manifestations. The clinical spectrum r anges from pure cerebellar signs to constellations including spinal cord and per ipheral nerve disease, cognitive impairment, cerebellar or supranuclear ophthalmol ogic signs, psychiatric problems, and seizures. Cerebellar ataxia can affect vi rtually any body part causing movement abnormalities. Gait, truncal, and limb atax ia are often the most obvious cerebellar findings though nystagmus, saccadic abnormalities, and dysarthria are usually associated. To date, 21 subtypes have been identified: SCA1-SCA4, SCA8, SCA10, SCA12-SCA14, SCA15/16, SCA17-SCA2 3,

SCA25, SCA27, SCA28 and dentatorubral pallidoluysian atrophy (DRPLA). Type I ADCA can be further divided based on the proposed pathogenetic mechanism into 3 subclasses: subclass 1 includes type I ADCA caused by CAG repeat expansion s such as SCA1-SCA3, SCA17, and DRPLA, subclass 2 includes trinucleotide repeat expansions that fall outside of the protein-coding regions of the disease gene including SCA8, SCA10 and SCA12. Subclass 3 contains disorders caused by s pecific gene deletions, missense mutation, and nonsense mutation and includes SCA1 3, SCA14, SCA15/16, SCA27 and SCA28. Diagnosis is based on clinical history, physical examination, genetic molecular testing, and exclusion of other di seases. Differential diagnosis is broad and includes secondary ataxias caused by d rug or toxic effects, nutritional deficiencies, endocrinopathies, infections and post-infection states, structural abnormalities, paraneoplastic conditions and certain neurodegenerative disorders. Given the autosomal dominant pattern of inheritance, genetic counseling is essential and best performed in special ized genetic clinics. There are currently no known effective treatments to modi fy disease progression. Care is therefore supportive. Occupational and physic al therapy for gait dysfunction and speech therapy for dysarthria is essentia l. Prognosis is variable depending on the type of ADCA and even among kindred s. FAU - Whaley, Nathaniel Robb AU - Whaley NR AD - Tri State Mountain Neurology, 105 Woodlawn Dr, Johnson City, TN 27604, USA . FAU - Fujioka, Shinsuke AU - Fujioka S FAU - Wszolek, Zbigniew K AU - Wszolek ZK LA - eng GR - 1RC2 NS070276/NS/NINDS NIH HHS/United States GR - P50 NS 072187/NS/NINDS NIH HHS/United States GR - R01 NS057567/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20110528 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - Spinocerebellar ataxia 13 SB - IM MH - Genotype MH - Humans MH - Phenotype MH - Spinocerebellar Degenerations/*genetics/*pathology PMC - PMC3123548

OID EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI uals

NLM: PMC3123548 2011/05/31 06:00 2011/10/19 06:00 2011/05/31 06:00 2009/07/07 [received] 2011/05/28 [accepted] 2011/05/28 [aheadofprint] 1750-1172-6-33 [pii] 10.1186/1750-1172-6-33 [doi] epublish Orphanet J Rare Dis. 2011 May 28;6:33. doi: 10.1186/1750-1172-6-33. 21386044 NLM MEDLINE 20111004 20120227 20131121 1558-9102 (Electronic) 1092-4388 (Linking) 54 5 2011 Oct Changes to articulatory kinematics in response to loudness cues in individ

with Parkinson's disease. PG - 1247-59 LID - 10.1044/1092-4388(2011/10-0024) [doi] AB - PURPOSE: Individuals with Parkinson's disease (PD) exhibit differences in displacement and velocity of the articulators as compared with older adult s. The purpose of the current study was to examine effects of 3 loudness cues on articulatory movement patterns in individuals with PD. METHOD: Nine indivi duals diagnosed with idiopathic PD and 9 age- and sex-matched healthy controls p roduced sentences in 4 conditions: (a) comfortable loudness, (b) targeting 10 dB a bove comfortable, (c) twice as loud as comfortable, and (d) in background noise . Lip and jaw kinematics and acoustic measurements were obtained. RESULTS: Both groups significantly increased sound pressure level (SPL) in the loud conditions as compared with the comfortable condition. For the loud conditions, both gro ups had the highest SPL in the background noise and the 10 dB conditions, and the lowest SPL in the twice as loud condition. Control participants produced the larg est opening displacement in the background noise condition and the smallest op ening displacement in the twice as loud condition. Conversely, individuals with PD produced the largest opening displacement in the twice as loud condition a nd the smallest opening displacement in the background noise condition. CONCLUSIO NS: Control participants and individuals with PD responded to cues to increase loudness in different ways. Changes in SPL may explain differences in kine

matics FAU AU AD FAU AU LA GR GR PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTAID AID PST SO PMIDOWN STATDA DCOMIS for the control participants, but they do not explain such differences for individuals with PD. Darling, Meghan Darling M Purdue University, West Lafayette, IN, USA Huber, Jessica E Huber JE eng 1R03DC05731/DC/NIDCD NIH HHS/United States R03 DC005731/DC/NIDCD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural 20110308 United States J Speech Lang Hear Res Journal of speech, language, and hearing research : JSLHR 9705610 IM Acoustic Stimulation Aged Aged, 80 and over Articulation Disorders/*complications/physiopathology Biomechanical Phenomena Case-Control Studies Cues Dysarthria/etiology/physiopathology Female Humans *Jaw Lip Male Matched-Pair Analysis Movement/*physiology Parkinson Disease/*complications/physiopathology Reaction Time/physiology Reference Values *Speech Acoustics Speech Articulation Tests *Speech Production Measurement PMC3433496 NIHMS401371 NLM: NIHMS401371 NLM: PMC3433496 2011/03/10 06:00 2012/03/01 06:00 2011/03/10 06:00 2011/03/08 [aheadofprint] 1092-4388_2011_10-0024 [pii] 10.1044/1092-4388(2011/10-0024) [doi] ppublish J Speech Lang Hear Res. 2011 Oct;54(5):1247-59. doi: 10.1044/1092-4388(2011/10-0024). Epub 2011 Mar 8. 21358160 NLM MEDLINE 20110301 20130729 1349-8029 (Electronic)

IS - 0470-8105 (Linking) VI - 51 IP - 2 DP - 2011 TI - Possible neuro-Sweet disease mimicking brain tumor in the medulla oblongat a--case report. PG - 140-3 AB - A 62-year-old male presented with a rare case of possible neuro-Sweet Dise ase (NSD) mimicking brain tumor in the medulla oblongata, manifesting as numbn ess in the bilateral upper and lower extremities, gait disturbance, dysarthria, a nd swallowing disturbance which gradually deteriorated over 3 months. Magneti c resonance imaging showed a mass lesion in the medulla oblongata, extending to the upper cervical cord with rim enhancement by gadolinium. The preoperative diagnosis was brain tumor, such as glioma, or inflammatory disease. His neurological symptoms gradually deteriorated, so biopsy was performed thro ugh the midline suboccipital approach. Histological examination showed infiltratio n of inflammatory cells, mainly lymphocytes and macrophages. Human leukocyte an tigen typing showed Cw1 and B54 which strongly suggested possible NSD. Steroid p ulse therapy was started after surgery and the clinical symptoms improved. Neurosurgeons should be aware of inflammatory disorders such as NSD mimick ing brain tumor. FAU - Akiba, Chihiro AU - Akiba C AD - Department of Neurosurgery, Juntendo University Shizuoka Hospital, Izunoku ni, Shizuoka, Japan. FAU - Esaki, Takanori AU - Esaki T FAU - Ando, Maya AU - Ando M FAU - Furuya, Tsuyoshi AU - Furuya T FAU - Noda, Kazuyuki AU - Noda K FAU - Nakao, Yasuaki AU - Nakao Y FAU - Yamamoto, Takuji AU - Yamamoto T FAU - Okuma, Yasuyuki AU - Okuma Y FAU - Mori, Kentaro AU - Mori K LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775

SB MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI lytic

IM Brain Neoplasms/diagnosis/*pathology/physiopathology Diagnosis, Differential Humans Inflammation/etiology/pathology/physiopathology Male Medulla Oblongata/*pathology/physiopathology Middle Aged Sweet Syndrome/*complications/diagnosis 2011/03/02 06:00 2013/07/31 06:00 2011/03/02 06:00 JST.JSTAGE/nmc/51.140 [pii] ppublish Neurol Med Chir (Tokyo). 2011;51(2):140-3. 21350199 NLM MEDLINE 20110329 20110531 20140220 1524-4628 (Electronic) 0039-2499 (Linking) 42 4 2011 Apr Home time is extended in patients with ischemic stroke who receive thrombo

therapy: a validation study of home time as an outcome measure. PG - 1046-50 LID - 10.1161/STROKEAHA.110.601302 [doi] AB - BACKGROUND AND PURPOSE: "Home time" (HT) refers to the number of days over the first 90 after stroke onset that a patient spends residing in their own ho me or a relative's home versus any institutional care. It is an accessible and obj ective parameter, free from subjective bias, with potential as an outcome measure in acute stroke trials. We sought to validate HT and assess treatment respons iveness using independent data. METHODS: We estimated HT in the Stroke Acute Ische mic NXY Treatment (SAINT) I neuroprotection trial. We compared outcomes between thrombolyzed (T) and nonthrombolyzed comparators (C) using HT and the modi fied Rankin Scale. For our primary analysis, we adjusted for baseline covariate s that significantly influence HT and in sensitivity analyses considered all vari ables that differed between groups at baseline. We report ordinal logistic regre ssion and analysis of covariance with 95% CIs. We describe the relationship of H T with baseline National Institutes of Health Stroke Scale and its components and with Day 90 modified Rankin Scale and Barthel Index. RESULTS: SAINT I included 1699 patients from 23 countries, of whom 28.7% received alteplase. HT correlate

d with age, baseline severity, alteplase use, side of ischemic lesion, presence o f diabetes, and country of patient enrollment (each P<0.05). We found an association between use of alteplase with better adjusted outcomes by eith er measure (OR for extended HT, 1.36; 95% CI, 1.08 to 1.72; P=0.009; analysis of covariance P=0.007 with a 5.5-day advantage; OR for more favorable modifie d Rankin Scale, 1.6; 95% CI, 1.28 to 2.00; P<0.0001; Cochran-Mantel-Haenszel P=0.046). HT was significantly associated with baseline National Institute s of Health Stroke Scale and each component of the National Institutes of Healt h Stroke Scale except level of consciousness, dysarthria, and ataxia. HT was significantly associated with Day 90 modified Rankin Scale and Barthel Ind ex. CONCLUSIONS: HT is a responsive measure for use in multinational acute str oke trials. Its inclusion as a complementary outcome is reasonable. We propose treatment effects are adjusted for age, baseline National Institutes of He alth Stroke Scale, side of stroke lesion, country of enrollment, and the presen ce of diabetes. FAU - Mishra, Nishant K AU - Mishra NK AD - Acute Stroke Unit, University of Glasgow, University Department of Medicin e and Therapeutics, Gardiner Institute, Western Infirmary & Faculty of Medicine, University of Glasgow, Glasgow, UK G11 6NT. 0808124@clinmed.gla.ac.uk FAU - Shuaib, Ashfaq AU - Shuaib A FAU - Lyden, Patrick AU - Lyden P FAU - Diener, Hans-Christoph AU - Diener HC FAU - Grotta, James AU - Grotta J FAU - Davis, Stephen AU - Davis S FAU - Davalos, Antoni AU - Davalos A FAU - Ashwood, Tim AU - Ashwood T FAU - Wasiewski, Warren AU - Wasiewski W FAU - Lees, Kennedy R AU - Lees KR CN - Stroke Acute Ischemic NXY Treatment I Trialists LA - eng GR - CZB/4/595/Chief Scientist Office/United Kingdom PT - Clinical Trial, Phase I PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PT - Validation Studies DEP - 20110224

PL - United States TA - Stroke JT - Stroke; a journal of cerebral circulation JID - 0235266 RN - 0 (Fibrinolytic Agents) SB - IM MH - Aged MH - Brain Ischemia/*drug therapy/mortality/nursing MH - Cohort Studies MH - Female MH - Fibrinolytic Agents/therapeutic use MH - Home Care Services MH - Hospitalization MH - Humans MH - *Length of Stay/trends MH - Male MH - Middle Aged MH - Outcome Assessment (Health Care)/*methods/trends MH - Recovery of Function/drug effects/physiology MH - Stroke/*drug therapy/mortality/nursing MH - Thrombolytic Therapy/*methods MH - Time Factors MH - Treatment Outcome EDAT- 2011/02/26 06:00 MHDA- 2011/06/01 06:00 CRDT- 2011/02/26 06:00 PHST- 2011/02/24 [aheadofprint] AID - STROKEAHA.110.601302 [pii] AID - 10.1161/STROKEAHA.110.601302 [doi] PST - ppublish SO - Stroke. 2011 Apr;42(4):1046-50. doi: 10.1161/STROKEAHA.110.601302. Epub 20 11 Feb 24. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID FAU AU AD NY FAU AU FAU AU FAU AU FAU 21336801 NLM MEDLINE 20110808 20111130 20131121 1937-6995 (Electronic) 1556-9039 (Linking) 7 3 2011 Sep "Chasing the dragon"--heroin-associated spongiform leukoencephalopathy. 240-2 10.1007/s13181-011-0139-5 [doi] Kass-Hout, Tareq Kass-Hout T Department of Neurology, State University of New York at Buffalo, Buffalo, 14209, USA. Kass-Hout, Omar Kass-Hout O Darkhabani, M Ziad Darkhabani MZ Mokin, Maxim Mokin M Mehta, Bijal

AU FAU AU LA PT PT PL TA JT f

Mehta B Radovic, Vladan Radovic V eng Case Reports Journal Article United States J Med Toxicol Journal of medical toxicology : official journal of the American College o Medical Toxicology 101284598 0 (Smoke) 70D95007SX (Heroin) IM Administration, Inhalation Brain/pathology Cerebellum/pathology Dysarthria/chemically induced/psychology Heroin/*adverse effects Heroin Dependence/*complications Humans Leukoencephalopathies/*chemically induced Magnetic Resonance Imaging Male Nystagmus, Pathologic/chemically induced Smoke Substance Abuse, Intravenous/*complications Young Adult PMC3550206 NLM: PMC3550206 2011/02/22 06:00 2011/12/13 00:00 2011/02/22 06:00 10.1007/s13181-011-0139-5 [doi] ppublish J Med Toxicol. 2011 Sep;7(3):240-2. doi: 10.1007/s13181-011-0139-5. 21273753 NLM MEDLINE 20110128 20110523 1349-8029 (Electronic) 0470-8105 (Linking) 51 1 2011 Inflammatory myofibroblastic tumor of the cerebellar hemisphere--case repo 79-81 A 60-year-old man presented with a rare cerebellar inflammatory myofibrobl tumor (IMT) manifesting as gait disturbance and dysarthria. Brain magnetic resonance imaging demonstrated an intra-axial round-shaped isointense mass homogeneously enhanced with gadolinium in the right cerebellar hemisphere,

JID RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI rt. PG AB astic

as well as perifocal edema extending to the brain stem and right thalamus. Th e tumor was elastic hard and was resected en bloc with a clear margin. Histologica

l examination revealed IMT with spindle cells and collagen, but negative for anaplastic lymphoma kinase expression. IMT most commonly affects the lung, but may involve many other parts of the body. There is some debate regarding t he disease entity of IMT in the central nervous system (IMT-CNS) because of i ts rarity and high frequency of recurrence. IMT-CNS is an important different ial diagnosis among tumor-like intracranial lesions and total resection is req uired. FAU - Kato, Koichi AU - Kato K AD - Department of Neurosurgery, Tokyo Rosai Hospital, Tokyo, Japan. kkato@tokyoh.rofuku.go.jp FAU - Moteki, Yosuke AU - Moteki Y FAU - Nakagawa, Masanori AU - Nakagawa M FAU - Kadoyama, Shigeru AU - Kadoyama S FAU - Ujiie, Hiroshi AU - Ujiie H LA - eng PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 SB - IM MH - Brain Edema/diagnosis/pathology/surgery MH - Cerebellar Diseases/*diagnosis/pathology/*surgery MH - Cerebellum/pathology/surgery MH - Diagnosis, Differential MH - Dysarthria/etiology MH - Gait Disorders, Neurologic/etiology MH - Granuloma, Plasma Cell/*diagnosis/pathology/surgery MH - Humans MH - *Image Processing, Computer-Assisted MH - *Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - *Tomography, X-Ray Computed EDAT- 2011/01/29 06:00 MHDA- 2011/05/24 06:00 CRDT- 2011/01/29 06:00 AID - JST.JSTAGE/nmc/51.79 [pii] PST - ppublish SO - Neurol Med Chir (Tokyo). 2011;51(1):79-81. PMIDOWN STATDA DCOMIS IS VI IP 21206184 NLM MEDLINE 20110105 20110502 1349-8029 (Electronic) 0470-8105 (Linking) 50 12

DP - 2010 TI - Paradoxical association of moyamoya syndrome with large middle cerebral ar tery aneurysm and subarachnoid hemorrhage. Case report. PG - 1088-91 AB - A 69-year-old woman was admitted to our hospital because of fluctuating dysarthria during the past 2 months. Magnetic resonance imaging revealed o ld cerebral infarction of the left cerebral hemisphere with acute subarachnoi d hemorrhage in the left sylvian fissure. Cerebral angiography showed a larg e saccular aneurysm, 14 mm in diameter, at the bifurcation of the left middl e cerebral artery (MCA) in association with moyamoya vasculopathy with atherosclerosis, including steno-occlusive changes at the bilateral termin al internal carotid arteries and abnormal net-like vessels at the base of the brain. She underwent microsurgical neck clipping of the large aneurysm followed b y superficial temporal artery-MCA anastomosis without complication. Intraope rative findings showed no evidence of aneurysm rupture, suggesting that the subar achnoid hemorrhage was due to the intrinsic pathology of moyamoya vasculopathy. Th e postoperative course was uneventful, and the patient was discharged withou t neurological deficit. Association of moyamoya syndrome with large MCA aneu rysm is extremely rare, and formation of large aneurysm at the vascular territory of an occluded vessel is apparently unique. FAU - Endo, Hidenori AU - Endo H AD - Department of Neurosurgery, Kohnan Hospital, Sendai, Miyagi, Japan. FAU - Fujimura, Miki AU - Fujimura M FAU - Inoue, Takashi AU - Inoue T FAU - Shimizu, Hiroaki AU - Shimizu H FAU - Tominaga, Teiji AU - Tominaga T LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 SB - IM MH - Aged MH - Cerebral Angiography MH - Cerebral Revascularization/*methods MH - Female MH - Humans MH - Intracranial Aneurysm/pathology/*surgery MH - Microsurgery

MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI ory

Middle Cerebral Artery/*pathology/surgery Moyamoya Disease/*complications/pathology Subarachnoid Hemorrhage/*etiology/therapy Tomography, X-Ray Computed Treatment Outcome 2011/01/06 06:00 2011/05/03 06:00 2011/01/06 06:00 JST.JSTAGE/nmc/50.1088 [pii] ppublish Neurol Med Chir (Tokyo). 2010;50(12):1088-91. 21203459 NLM MEDLINE 20110104 20110705 20131020 1932-6203 (Electronic) 1932-6203 (Linking) 5 12 2010 Perinatal asphyxia affects rat auditory processing: implications for audit

perceptual impairments in neurodevelopmental disorders. PG - e15326 LID - 10.1371/journal.pone.0015326 [doi] AB - Perinatal asphyxia, a naturally and commonly occurring risk factor in birt hing, represents one of the major causes of neonatal encephalopathy with long te rm consequences for infants. Here, degraded spectral and temporal responses t o sounds were recorded from neurons in the primary auditory cortex (A1) of a dult rats exposed to asphyxia at birth. Response onset latencies and durations were increased. Response amplitudes were reduced. Tuning curves were broader. D egraded successive-stimulus masking inhibitory mechanisms were associated with a r educed capability of neurons to follow higher-rate repetitive stimuli. The archit ecture of peripheral inner ear sensory epithelium was preserved, suggesting that recorded abnormalities can be of central origin. Some implications of thes e findings for the genesis of language perception deficits or for impaired l anguage expression recorded in developmental disorders, such as autism spectrum disorders, contributed to by perinatal asphyxia, are discussed. FAU - Strata, Fabrizio AU - Strata F AD - Department of Neuroscience, Section of Physiology, University of Parma, Pa rma, Italy. fab@phy.ucsf.edu FAU - Stoianov, Ivilin P AU - Stoianov IP FAU - de Villers-Sidani, Etienne AU - de Villers-Sidani E

FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA GR PT PT PT DEP PL TA JT JID RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDACRDTPHSTPHSTPHSTAID PST SO PMIDOWN STATDA DCOMLR IS VI DP -

Bonham, Ben Bonham B Martone, Tiziana Martone T Kenet, Tal Kenet T Chang, Edward F Chang EF Vincenti, Vincenzo Vincenti V Merzenich, Michael M Merzenich MM eng MH77970/MH/NIMH NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 20101223 United States PLoS One PloS one 101285081 Auditory perceptual impairment IM Acoustic Stimulation/adverse effects Animals Animals, Newborn Asphyxia Neonatorum/*complications Auditory Cortex/physiology Developmental Disabilities/complications/etiology Disease Models, Animal Dysarthria/complications/etiology Electrophysiology Evoked Potentials, Auditory, Brain Stem/physiology Humans Infant, Newborn Neurons Rats Time Factors PMC3009724 NLM: PMC3009724 2011/01/05 06:00 2011/07/06 06:00 2011/01/05 06:00 2010/08/12 [received] 2010/11/06 [accepted] 2010/12/23 [epublish] 10.1371/journal.pone.0015326 [doi] epublish PLoS One. 2010 Dec 23;5(12):e15326. doi: 10.1371/journal.pone.0015326. 22696689 NLM MEDLINE 20120614 20130906 20131115 1757-790X (Electronic) 2011 2011

TI LID LID AB n can series

Epilepsy in a patient with ataxia caused by vitamin E deficiency. 10.1136/bcr.01.2011.3728 [doi] bcr0120113728 [pii] Ataxia due to vitamin E deficiency is important because disease progressio be stopped by supplementary therapy. A limited number of studies and case suggest that the disease is mainly confined to the cerebellum and spinal c

ord tract and seems to be more common in North African countries. We report a patient from North Norway with progressive ataxia from the age of 5, bilateral dro pfoot, Babinski's sign, dysarthria and early epilepsy. Two mutations, 513insTT an d p.Arg134x, were detected. When treatment was initiated 25 years after onse t of symptoms, the patient was bound to the wheel chair. No further progression of pareses, ataxia or epileptic seizures has been observed in a 3-year follow -up period. This case indicates that cerebral involvement may be present in pa tients with a lack of vitamin E. If this observation is confirmed, a further expl oration of clinical presentation, anatomic involvement and geographic distribution of the disease is warranted. FAU - Muller, Kai Ivar AU - Muller KI AD - Department of Neurology, University Hospital of North Norway, Tromso, Norw ay. kai.ivar.muller@unn.no FAU - Bekkelund, Svein Ivar AU - Bekkelund SI LA - eng PT - Case Reports PT - Journal Article DEP - 20110503 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Ataxia/diagnosis/*etiology MH - Child, Preschool MH - Diagnosis, Differential MH - Electroencephalography MH - Epilepsy/diagnosis/*etiology MH - Female MH - Humans MH - Vitamin E Deficiency/*complications/diagnosis PMC - PMC3089935 OID - NLM: PMC3089935 EDAT- 2011/01/01 00:00 MHDA- 2013/09/07 06:00 CRDT- 2012/06/15 06:00 AID - bcr.01.2011.3728 [pii] AID - 10.1136/bcr.01.2011.3728 [doi] PST - epublish

SO - BMJ Case Rep. 2011 May 3;2011. pii: bcr0120113728. doi: 10.1136/bcr.01.201 1.3728. PMID- 21125300 OWN - NLM STAT- MEDLINE DA - 20110329 DCOM- 20110721 LR - 20131021 IS - 1432-0932 (Electronic) IS - 0940-6719 (Linking) VI - 20 IP - 4 DP - 2011 Apr TI - Endoscopic transnasal resection of the odontoid: case series and clinical course. PG - 661-6 LID - 10.1007/s00586-010-1629-x [doi] AB - The transoral route is the gold standard for odontoid resection. Results a re satisfying though surgery can be challenging for patients and surgeons due to its invasiveness. A less invasive transnasal approach could provide a sufficie nt extent of resection with less collateral damage. The technique of transnas al endoscopic odontoid resection is demonstrated by a case series of three pa tients. A fully endoscopic transnasal odontoid resection was conducted by use of C T-based neuronavigation. A complete odontoid resection succeeded in all patients. Symptoms such as dysarthria, swallowing disturbance, salivary retention, myelopathic gait disturbances, neck pain, and tetraparesis improved in all patients markedly. Transnasal endoscopic odontoid resection is a feasible alternative to the transoral technique. It leaves the oropharynx intact, w hich could result in lower approach related complications especially in patient s with bulbar symptoms. FAU - Gempt, Jens AU - Gempt J AD - Neurochirurgische Klinik und Poliklinik, Klinikum rechts der Isar, Technis che Universitat Munchen, Ismaningerstr. 22, 81675 Munich, Germany. Jens.Gempt@lrz.tum.de FAU - Lehmberg, Jens AU - Lehmberg J FAU - Grams, Astrid E AU - Grams AE FAU - Berends, Lars AU - Berends L FAU - Meyer, Bernhard AU - Meyer B FAU - Stoffel, Michael AU - Stoffel M LA - eng PT - Case Reports PT - Journal Article DEP - 20101202 PL - Germany

TA - Eur Spine J JT - European spine journal : official publication of the European Spine Societ y, the European Spinal Deformity Society, and the European Section of the Cervica l Spine Research Society JID - 9301980 SB - IM MH - Aged MH - Arthritis, Rheumatoid/*surgery MH - Central Nervous System Neoplasms/*surgery MH - Cervical Vertebrae/surgery MH - Decompression, Surgical/methods MH - Deglutition Disorders/prevention & control MH - Endoscopy/adverse effects/*methods MH - Female MH - Humans MH - Middle Aged MH - Neuronavigation/*methods MH - Neurosurgical Procedures/*methods MH - Odontoid Process/*surgery MH - Spinal Fusion/methods MH - Treatment Outcome PMC - PMC3065609 OID - NLM: PMC3065609 EDAT- 2010/12/03 06:00 MHDA- 2011/07/22 06:00 CRDT- 2010/12/03 06:00 PHST- 2010/06/18 [received] PHST- 2010/11/07 [accepted] PHST- 2010/09/01 [revised] PHST- 2010/12/02 [aheadofprint] AID - 10.1007/s00586-010-1629-x [doi] PST - ppublish SO - Eur Spine J. 2011 Apr;20(4):661-6. doi: 10.1007/s00586-010-1629-x. Epub 20 10 Dec 2. PMIDOWN STATDA DCOMLR IS VI DP TI PG AB . The patient underwent speech therapy and showed significant improvement over a short period of time. The favorable outcome of the present study highlights the role of speech therapy in such a case, where it often remains un-emphasized. FAU - Vir, Dharam AU - Vir D AD - Department of Otolaryngology and Head and Neck Surgery, Post Graduate Inst 21119998 NLM MEDLINE 20101201 20110624 20131021 1937-8688 (Electronic) 4 2010 Neurological manifestations in speech after snake bite: a rare case. 13 The patient was admitted after reporting a snake bite from which he later developed neurological signs and symptoms among which a flaccid dysarthria

itute FAU AU FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID RN RN RN RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC OID OTO OT OT OT OT EDATMHDACRDTPHSTPHSTPHSTPST SO PMIDOWN STATDA of Medical Education and Research, Chandigarh, India. Sachin Sachin Gupta, Dipti Gupta D Modi, Munish Modi M Panda, Naresh Panda N eng Case Reports Journal Article 20100324 Uganda Pan Afr Med J The Pan African medical journal 101517926 0 (Anti-Bacterial Agents) 0 (Antihypertensive Agents) 0 (Antivenins) 0 (Bungarotoxins) 0 (Tetanus Toxoid) IM Animals Anti-Bacterial Agents/administration & dosage Antihypertensive Agents/administration & dosage Antivenins/*therapeutic use Bungarotoxins/*poisoning *Bungarus Dysarthria/*etiology/therapy Humans Male Middle Aged Nervous System Diseases/etiology Quadriplegia/etiology Snake Bites/*complications/drug therapy Speech Therapy/methods Tetanus Toxoid/administration & dosage Treatment Outcome PMC2984309 NLM: PMC2984309 NOTNLM Snake bite dysarthria flaccid dysarthria speech therapy 2010/12/02 06:00 2011/06/28 06:00 2010/12/02 06:00 2009/09/04 [received] 2010/02/17 [accepted] 2010/03/24 [epublish] epublish Pan Afr Med J. 2010 Mar 24;4:13. 20966389 NLM MEDLINE 20110404

DCOMLR IS IS VI IP DP TI -

20110729 20131021 1558-9102 (Electronic) 1092-4388 (Linking) 54 2 2011 Apr Prevalence and phenotype of childhood apraxia of speech in youth with galactosemia. PG - 487-519 LID - 10.1044/1092-4388(2010/10-0068) [doi] AB - PURPOSE: In this article, the authors address the hypothesis that the seve re and persistent speech disorder reported in persons with galactosemia meets contemporary diagnostic criteria for childhood apraxia of speech (CAS). A positive finding for CAS in this rare metabolic disorder has the potential to impact treatment of persons with galactosemia and inform explanatory persp ectives on CAS in neurological, neurodevelopmental, and idiopathic contexts. METHO D: Thirty-three youth with galactosemia and significant prior or persistent s peech sound disorder were assessed in their homes in 17 states. Participants com pleted a protocol yielding information on their cognitive, structural, sensorimot or, language, speech, prosody, and voice status and function. RESULTS: Eight o f the 33 participants (24%) met contemporary diagnostic criteria for CAS. Two participants, 1 of whom was among the 8 with CAS, met criteria for ataxic or hyperkinetic dysarthria. Groupwise findings for the remaining 24 participa nts are consistent with a classification category termed Motor Speech Disorder-Not Otherwise Specified (Shriberg, Fourakis et al., 2010a). CONCLUSION: The au thors estimate the prevalence of CAS in galactosemia at 18 per hundred-180 times the estimated risk for idiopathic CAS. Findings support the need to study risk factors for the high occurrence of motor speech disorders in galactosemia despite early compliant dietary management. FAU - Shriberg, Lawrence D AU - Shriberg LD AD - Waisman Center, Madison, WI, USA. shriberg@waisman.wisc.edu FAU - Potter, Nancy L AU - Potter NL FAU - Strand, Edythe A AU - Strand EA LA - eng GR - DC000496/DC/NIDCD NIH HHS/United States GR - HD03352/HD/NICHD NIH HHS/United States GR - R01 DC000496-22/DC/NIDCD NIH HHS/United States GR - R01 DC000496-23/DC/NIDCD NIH HHS/United States GR - R01 DC000496-24/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20101021 PL - United States

TA JT JID SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI cy

J Speech Lang Hear Res Journal of speech, language, and hearing research : JSLHR 9705610 IM Adolescent Adult Apraxias/*diagnosis/*epidemiology Child Child, Preschool Cognition Galactosemias/diet therapy/*epidemiology/genetics Genes, Recessive Genotype Humans Phenotype Phonetics Prevalence Risk Factors Speech Disorders/*diagnosis/*epidemiology Speech Production Measurement Young Adult PMC3070858 NIHMS238267 NLM: NIHMS238267 NLM: PMC3070858 2010/10/23 06:00 2011/07/30 06:00 2010/10/23 06:00 2010/10/21 [aheadofprint] 1092-4388_2010_10-0068 [pii] 10.1044/1092-4388(2010/10-0068) [doi] ppublish J Speech Lang Hear Res. 2011 Apr;54(2):487-519. doi: 10.1044/1092-4388(2010/10-0068). Epub 2010 Oct 21. 20938199 NLM MEDLINE 20110621 20111024 20131021 1421-9972 (Electronic) 1021-7762 (Linking) 63 4 2011 The impact of rate reduction and increased loudness on fundamental frequen

characteristics in dysarthria. PG - 178-86 LID - 10.1159/000316315 [doi] AB - OBJECTIVE: This study examined the extent to which articulatory rate reduc tion and increased loudness were associated with adjustments in utterance-level measures of fundamental frequency (F(0)) variability for speakers with dys arthria and healthy controls that have been shown to impact on intelligibility in previously published studies. More generally, the current study sought to compare and contrast how a slower-than-normal rate and increased vocal loudness im

pact on a variety of utterance-level F(0) characteristics for speakers with dysart hria and healthy controls. PATIENTS AND METHODS: Eleven speakers with Parkinson 's disease, 15 speakers with multiple sclerosis, and 14 healthy control speak ers were audio recorded while reading a passage in habitual, loud, and slow conditions. Magnitude production was used to elicit variations in rate and loudness. Acoustic measures of duration, intensity and F(0) were obtained. RESULTS AND CONCLUSIONS: For all speaker groups, a slower-than-normal articulatory rate and increased vocal loudness had distinct effects on F(0 ) relative to the habitual condition, including a tendency for measures of F (0) variation to be greater in the loud condition and reduced in the slow cond ition. These results suggest implications for the treatment of dysarthria. CI - Copyright (c) 2010 S. Karger AG, Basel. FAU - Tjaden, Kris AU - Tjaden K AD - Department of Communicative Disorders and Sciences, University at Buffalo, NY, USA. Tjaden@buffalo.edu FAU - Wilding, Greg AU - Wilding G LA - eng GR - R01DC004689/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20101012 PL - Switzerland TA - Folia Phoniatr Logop JT - Folia phoniatrica et logopaedica : official organ of the International Association of Logopedics and Phoniatrics (IALP) JID - 9422792 SB - IM MH - Adult MH - Aged MH - *Behavior Therapy MH - Dysarthria/etiology/*physiopathology/therapy MH - Female MH - Humans MH - Male MH - Middle Aged MH - Multiple Sclerosis/complications/physiopathology MH - Observer Variation MH - Parkinson Disease/complications/physiopathology MH - Reproducibility of Results MH - Sound Spectrography MH - *Speech Acoustics MH - *Speech Intelligibility MH - Time Factors PMC - PMC2982854 OID - NLM: PMC2982854 EDAT- 2010/10/13 06:00 MHDA- 2011/10/25 06:00 CRDT- 2010/10/13 06:00 PHST- 2010/10/12 [aheadofprint] AID - 000316315 [pii]

AID - 10.1159/000316315 [doi] PST - ppublish SO - Folia Phoniatr Logop. 2011;63(4):178-86. doi: 10.1159/000316315. Epub 2010 Oct 12. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI ghted 20926409 NLM MEDLINE 20110103 20110127 20131121 1465-3621 (Electronic) 0368-2811 (Linking) 41 1 2011 Jan Early detection of 5-FU-induced acute leukoencephalopathy on diffusion-wei

MRI. PG - 121-4 LID - 10.1093/jjco/hyq157 [doi] AB - A 59-year-old man treated with 5-fluorouracil and cisplatin for advanced oesophageal cancer presented abnormal behaviour and subsequently developed impairment of cognitive function, dysphagia and dysarthria on the fifth da y of the treatment. Although brain computed tomography revealed no abnormal fin dings, brain magnetic resonance imaging using diffusion-weighted imaging clearly revealed the presence of a high signal intensity in the deep white matter of the bilateral cerebral hemispheres, including the corpus callosum symmetricall y. A diagnosis of acute leukoencephalopathy was reached based on these findings . His clinical symptoms normalized four days after the discontinuation of the chemotherapy. Improvement in magnetic resonance imaging findings was delay ed compared with that of clinical symptoms; however, the high signal intensit y detected in the deep white matter had disappeared completely five months a fter the onset of symptoms. Early detection of drug-induced leukoencephalopathy is important as the clinical symptoms can be reversed by early discontinuatio n of the causative drug. Diffusion-weighted magnetic resonance imaging is a use ful modality for the early detection and definitive diagnosis of this characte ristic encephalopathy. FAU - Akitake, Reiko AU - Akitake R AD - Department of Gastroenterology and Hepatology, Graduate School of Medicine , Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan. FAU - Miyamoto, Shin'ichi AU - Miyamoto S FAU - Nakamura, Fumiyasu AU - Nakamura F

FAU AU FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID RN RN SB MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTAID AID PST SO 0 Oct PMIDOWN STATDA DCOMLR IS IS VI IP DP TI f a

Horimatsu, Takahiro Horimatsu T Ezoe, Yasumasa Ezoe Y Muto, Manabu Muto M Chiba, Tsutomu Chiba T eng Case Reports Journal Article 20101006 England Jpn J Clin Oncol Japanese journal of clinical oncology 0313225 0 (Antimetabolites, Antineoplastic) U3P01618RT (Fluorouracil) IM Acute Disease Antimetabolites, Antineoplastic/administration & dosage/*adverse effects Brain/*pathology *Diffusion Magnetic Resonance Imaging Early Diagnosis Esophageal Neoplasms/drug therapy Fluorouracil/administration & dosage/*adverse effects Humans Leukoencephalopathies/*chemically induced/*diagnosis/pathology Male Middle Aged 2010/10/12 06:00 2011/01/29 06:00 2010/10/08 06:00 2010/10/06 [aheadofprint] hyq157 [pii] 10.1093/jjco/hyq157 [doi] ppublish Jpn J Clin Oncol. 2011 Jan;41(1):121-4. doi: 10.1093/jjco/hyq157. Epub 201 6. 20846979 NLM MEDLINE 20100917 20110207 20131022 1748-880X (Electronic) 0007-1285 (Linking) 83 994 2010 Oct Bilateral hypertrophic olivary degeneration following surgical resection o

posterior fossa epidermoid cyst. PG - e211-5 LID - 10.1259/bjr/27446907 [doi] AB - Hypertrophic olivary degeneration is a result of a primary lesion damaging the dento-rubro-olivary pathway. It is a transynaptic form of degeneration and

is unique, causing hypertrophy rather than atrophy of the inferior olivary nu cleus. We report a case of bilateral hypertrophic olivary degeneration following surgical excision of a posterior fossa epidermoid cyst and review the rele vant literature. FAU - Vaidhyanath, R AU - Vaidhyanath R AD - Department of Radiology, University Hospitals of Leicester, Leicester Roya l Infirmary, Infirmary Square, Leicester LE1 5WW, UK. ram.vaidhyanath@uhl-tr .nhs.uk FAU - Thomas, A AU - Thomas A FAU - Messios, N AU - Messios N LA - eng PT - Case Reports PT - Journal Article PT - Review PL - England TA - Br J Radiol JT - The British journal of radiology JID - 0373125 SB - AIM SB - IM MH - Dysarthria/etiology/therapy MH - Epidermal Cyst/complications/*pathology MH - Humans MH - Hypertrophy/pathology MH - Male MH - Middle Aged MH - Olivary Nucleus/*pathology MH - Postoperative Complications MH - Speech Therapy MH - Tomography, X-Ray Computed PMC - PMC3473740 OID - NLM: PMC3473740 EDAT- 2010/09/18 06:00 MHDA- 2011/02/08 06:00 CRDT- 2010/09/18 06:00 AID - 83/994/e211 [pii] AID - 10.1259/bjr/27446907 [doi] PST - ppublish SO - Br J Radiol. 2010 Oct;83(994):e211-5. doi: 10.1259/bjr/27446907. PMID- 20814000 OWN - NLM STAT- MEDLINE DA - 20101026 DCOM- 20101116 LR - 20131023 IS - 1524-4628 (Electronic) IS - 0039-2499 (Linking) VI - 41 IP - 11 DP - 2010 Nov TI - Strokes with minor symptoms: an exploratory analysis of the National Insti tute of

Neurological Disorders and Stroke recombinant tissue plasminogen activator trials. PG - 2581-6 LID - 10.1161/STROKEAHA.110.593632 [doi] AB - BACKGROUND AND PURPOSE: The pivotal National Institute of Neurological Dis orders and Stroke recombinant tissue plasminogen activator trials excluded patien ts with ischemic stroke with specific minor presentations or rapidly improving sym ptoms. The recombinant tissue plasminogen activator product label notes that its use for minor neurological deficit or rapidly improving stroke symptoms has not be en evaluated. As a result, patients with low National Institutes of Health St roke Scale scores are not commonly treated in clinical practice. We sought to f urther characterize the patients with minor stroke who were included in the Natio nal Institute of Neurological Disorders and Stroke trials. METHODS: Minor stro kes were defined as National Institutes of Health Stroke Scale score </= 5 at baseline for this retrospective analysis, because this subgroup is most co mmonly excluded from treatment in clinical practice and trials. Clinical stroke syndromes were defined based on prespecified National Institutes of Health Stroke Scale item score clusters. Clinical outcomes were reviewed generally and w ithin these cluster subgroups. RESULTS: Only 58 cases had National Institutes of Health Stroke Scale scores of 0 to 5 in the National Institute of Neurological Di sorders and Stroke trials (42 recombinant tissue plasminogen activator and 16 plac ebo), and 2971 patients were excluded from the trials due to "rapidly improving" or "minor symptoms" as the primary reason. No patients were enrolled with iso lated motor symptoms, isolated facial droop, isolated ataxia, dysarthria, isolat ed sensory symptoms, or with only symptoms/signs not captured by the National Institutes of Health Stroke Scale score (ie, National Institutes of Health Stroke Scale=0). There were </= 3 patients with each of the other isolated defici ts enrolled in the trial. CONCLUSIONS: The National Institute of Neurological Disorders and Stroke trials excluded a substantial number of strokes with minor presentations, those that were included were small in number, and conclusi ons about outcomes based on specific syndromes cannot be drawn. Further prospe ctive, systematic study of this subgroup is needed. FAU - Khatri, Pooja AU - Khatri P AD - Department of Neurology, University of Cincinnati, Cincinnati, OH 45267-05 25, USA. pooja.khatri@uc.edu

FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA GR GR GR GR GR GR GR GR GR GR GR GR GR GR PT PT PT DEP PL TA JT JID RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC

Kleindorfer, Dawn O Kleindorfer DO Yeatts, Sharon D Yeatts SD Saver, Jeffrey L Saver JL Levine, Steven R Levine SR Lyden, Patrick D Lyden PD Moomaw, Charles J Moomaw CJ Palesch, Yuko Y Palesch YY Jauch, Edward C Jauch EC Broderick, Joseph P Broderick JP eng 1R0 1 HL096944/HL/NHLBI NIH HHS/United States 1R0 1 NS052417/NS/NINDS NIH HHS/United States K23 NS059843-04/NS/NINDS NIH HHS/United States K23 NS059843-05/NS/NINDS NIH HHS/United States K23NS059843/NS/NINDS NIH HHS/United States P50 NS044378/NS/NINDS NIH HHS/United States R01 NS062778/NS/NINDS NIH HHS/United States R01 NS062835/NS/NINDS NIH HHS/United States U 01 NS052220/NS/NINDS NIH HHS/United States U01 NS 44364/NS/NINDS NIH HHS/United States U01 NS054630-04/NS/NINDS NIH HHS/United States U01 NS059041/NS/NINDS NIH HHS/United States U01 NS061861/NS/NINDS NIH HHS/United States U01, NS059041/NS/NINDS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 20100902 United States Stroke Stroke; a journal of cerebral circulation 0235266 0 (Recombinant Proteins) EC 3.4.21.68 (Tissue Plasminogen Activator) IM Aged Cognition Disorders/prevention & control Disability Evaluation Female Humans Male Middle Aged *National Institute of Neurological Disorders and Stroke Recombinant Proteins/*therapeutic use Retrospective Studies Severity of Illness Index Stroke/*drug therapy Tissue Plasminogen Activator/*therapeutic use Treatment Outcome United States PMC2964419

MID - NIHMS237407 OID - NLM: NIHMS237407 OID - NLM: PMC2964419 EDAT- 2010/09/04 06:00 MHDA- 2010/11/17 06:00 CRDT- 2010/09/04 06:00 PHST- 2010/09/02 [aheadofprint] AID - STROKEAHA.110.593632 [pii] AID - 10.1161/STROKEAHA.110.593632 [doi] PST - ppublish SO - Stroke. 2010 Nov;41(11):2581-6. doi: 10.1161/STROKEAHA.110.593632. Epub 20 10 Sep 2. PMID- 20739256 OWN - NLM STAT- MEDLINE DA - 20100826 DCOM- 20101109 LR - 20131121 IS - 1760-1703 (Print) IS - 1760-1703 (Linking) VI - 8 IP - 3 DP - 2010 Sep TI - [Multiple system atrophy]. PG - 179-91 LID - 10.1684/pnv.2010.0212 [doi] AB - Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder of unknown etiology. It is the most frequent disorder among atypical parkinsonism wit h an estimated prevalence of 2 to 5 per 100 000 inhabitants. The clinical sympt oms are rapidly progressing with a mean survival ranging between 6 to 9 years. The diagnosis is based on consensus criteria that have been revised in 2008. T he diagnostic criteria allow defining "possible", "probable" and "definite" M SA. The latter requires post mortem confirmation of striatonigral and olivopontocerebellar degeneration with alpha-synuclein containing glial cytoplasmic inclusions. The diagnosis of "possible" and "probable" MSA is based on the variable presence and severity of parkinsonism, cerebellar dysfunct ion, autonomic failure and pyramidal signs. According to the revised criteria, atrophy of putamen, pons, middle cerebellar peduncle (MCP) or cerebellum on brain magnetic resonance imaging are considered to be additional features for th e diagnosis of "possible" MSA. T2-weighted brain imaging may further reveal a putaminal hypointensity, a hyperintense lateral putaminal rim, the so call ed "hot cross bun sign" and MCP hyperintensities. Cardiovascular examination, urod ynamic testing and anal sphincter electromyography may be helpful for the diagnos is of autonomic failure. Some patients may respond to levodopa, but usually to a lesser

extent than those suffering from Parkinson's disease, and high doses are a lready required in early disease stages. No specific therapy is available for cer ebellar dysfunction, while effective treatments exist for urinary and cardiovascul ar autonomic failure. Physical therapy may help to improve the difficulties o f gait and stance, and to prevent their complications. In later disease stages, s peech therapy becomes necessary for the treatment of dysarthria and dysphagia. Percutaneous gastrostomy is sometimes necessary in patients with severe dysphagia. Beyond these strategies, psychological support, social care and occupational therapy to adapt the environment to the patient's disability are prerequisites for improving the quality of life in MSA patients. FAU - Damon-Perriere, Nathalie AU - Damon-Perriere N AD - Service de neurologie, Centre de reference national maladie rare atrophie multisystematisee, CHU de Bordeaux, Hopital du Haut-Leveque, Pessac. FAU - Tison, Francois AU - Tison F FAU - Meissner, Wassilios G AU - Meissner WG LA - fre PT - English Abstract PT - Journal Article PT - Review TT - L'atrophie multisystematisee. PL - France TA - Psychol Neuropsychiatr Vieil JT - Psychologie & neuropsychiatrie du vieillissement JID - 101203421 RN - 0 (Antiparkinson Agents) RN - 46627O600J (Levodopa) SB - IM MH - Aged MH - Antiparkinson Agents/therapeutic use MH - Atrophy MH - Brain/pathology MH - Combined Modality Therapy MH - Cross-Sectional Studies MH - Humans MH - Levodopa/therapeutic use MH - Magnetic Resonance Imaging MH - Multiple System Atrophy/*diagnosis/epidemiology/therapy MH - Neurologic Examination MH - Palliative Care MH - Parkinsonian Disorders/diagnosis/epidemiology/therapy MH - Physical Therapy Modalities MH - Prognosis MH - Survival Rate EDAT- 2010/08/27 06:00 MHDA- 2010/11/10 06:00 CRDT- 2010/08/27 06:00 AID - pnv.2010.0212 [pii] AID - 10.1684/pnv.2010.0212 [doi] PST - ppublish SO - Psychol Neuropsychiatr Vieil. 2010 Sep;8(3):179-91. doi: 10.1684/pnv.2010. 0212.

PMIDOWN STATDA DCOMLR IS IS VI IP DP TI n's

20699346 NLM MEDLINE 20110202 20110526 20131023 1558-9102 (Electronic) 1092-4388 (Linking) 54 1 2011 Feb The intonation-syntax interface in the speech of individuals with Parkinso

disease. PG - 19-32 LID - 10.1044/1092-4388(2010/09-0079) [doi] AB - PURPOSE: This study examined the effect of Parkinson's disease (PD) on the intonational marking of final and nonfinal syntactic boundaries and invest igated whether the effect of PD on intonation was sex specific. METHOD: Eight wom en and 8 men with PD and 16 age- and sex-matched control participants read a pass age at comfortable pitch, rate, and loudness. Nuclear tones from final and nonfin al syntactic boundaries in clauses and lists were extracted. Measures of fund amental frequency (F0) were made on each tone contour. RESULTS: Individuals with P D demonstrated impaired differentiation of syntactic boundary finality/nonfi nality with contour direction. They produced a lower proportion of falling contou rs in final boundaries and a higher proportion of falling contours in nonfinal boundaries than did control participants. Although not mediated by syntax, the effect of PD on F0 standard deviation (F0 SD) and pitch range in semitones (PRST) was sex specific. Women with PD produced greater F0 SD and PRST than did m en with PD and women without PD. Men with PD produced lower PRST than did men with out PD. CONCLUSIONS: Impaired intonational marking of syntactic boundaries likely contributes to dysprosody and reduced communicative effectiveness in PD. T he effect of PD on intonation was sex specific. The results are not fully exp lained by PD-related motor execution impairments. FAU - MacPherson, Megan K AU - MacPherson MK AD - Purdue University, Department of Speech, Language, and Hearing Sciences, 1 353 Heavilon Hall, 500 Oval Drive, West Lafayette, IN 47907-2038, USA. FAU - Huber, Jessica E AU - Huber JE FAU - Snow, David P AU - Snow DP LA - eng GR - R03 DC005731/DC/NIDCD NIH HHS/United States

GR GR PT PT PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB yped h a

R03DC05731/DC/NIDCD NIH HHS/United States T32DC00030/DC/NIDCD NIH HHS/United States Comparative Study Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 20100810 United States J Speech Lang Hear Res Journal of speech, language, and hearing research : JSLHR 9705610 IM Acoustic Stimulation Aged Aged, 80 and over Dysarthria/*diagnosis/*etiology Female Humans Linguistics Male Middle Aged Models, Biological Parkinson Disease/*complications *Phonetics Sex Characteristics Speech Production Measurement Voice Quality PMC3441058 NIHMS401366 NLM: NIHMS401366 NLM: PMC3441058 2010/08/12 06:00 2011/05/27 06:00 2010/08/12 06:00 2010/08/10 [aheadofprint] 1092-4388_2010_09-0079 [pii] 10.1044/1092-4388(2010/09-0079) [doi] ppublish J Speech Lang Hear Res. 2011 Feb;54(1):19-32. doi: 10.1044/1092-4388(2010/09-0079). Epub 2010 Aug 10. 20619422 NLM MEDLINE 20100804 20101122 20131023 1878-5883 (Electronic) 0022-510X (Linking) 296 1-2 2010 Sep 15 Capsular warning syndrome caused by middle cerebral artery stenosis. 115-20 10.1016/j.jns.2010.06.003 [doi] The capsular warning syndrome is a term used to describe recurrent stereot lacunar transient ischemic attacks (TIAs). This syndrome is associated wit high risk of developing a completed stroke. The presumed mechanism for thi

s syndrome is angiopathy of a lenticulostriate artery. We describe the case of a 33-year-old man who presented with the capsular warning syndrome who was successfully treated with angioplasty. The patient's capsular warning synd rome manifested as recurrent episodes of transient left hemiparesis. Symptoms r ecurred one to three times daily despite treatment with antithrombotics. Cerebral angiography demonstrated stenosis of the right middle cerebral artery (MCA ) with decreased flow to a dominant lenticulostriate artery. Angioplasty of the r ight middle cerebral artery increased flow to the lenticulostriate artery and t he TIAs resolved following the procedure. In select cases intracranial angioplasty , may be an effective treatment for patients with capsular warning syndrome. CI - 2010 Elsevier B.V. All rights reserved. FAU - Lee, Jun AU - Lee J AD - Department Neurology and Neurological Sciences and the Stanford Stroke Cen ter, Stanford University Medical Center, 701 Welch Road, Suite 325, Palo Alto, Stanford, CA 94304, USA. FAU - Albers, Gregory W AU - Albers GW FAU - Marks, Michael P AU - Marks MP FAU - Lansberg, Maarten G AU - Lansberg MG LA - eng GR - K23 NS051372-04/NS/NINDS NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100708 PL - Netherlands TA - J Neurol Sci JT - Journal of the neurological sciences JID - 0375403 SB - IM MH - Adult MH - Angioplasty MH - Cerebral Angiography MH - Dysarthria/etiology MH - Humans MH - Infarction, Middle Cerebral Artery/*complications/surgery MH - Ischemic Attack, Transient/*etiology/surgery MH - Magnetic Resonance Imaging MH - Male MH - Neurosurgical Procedures MH - Paresis/etiology MH - Recurrence MH - Tomography, Emission-Computed, Single-Photon PMC - PMC2932463 MID - NIHMS222337 OID - NLM: NIHMS222337 OID - NLM: PMC2932463 EDAT- 2010/07/14 06:00

MHDACRDTPHSTPHSTPHSTPHSTAID AID PST SO Epub

2010/12/14 06:00 2010/07/13 06:00 2010/03/19 [received] 2010/05/26 [revised] 2010/06/02 [accepted] 2010/07/08 [aheadofprint] S0022-510X(10)00251-0 [pii] 10.1016/j.jns.2010.06.003 [doi] ppublish J Neurol Sci. 2010 Sep 15;296(1-2):115-20. doi: 10.1016/j.jns.2010.06.003. 2010 Jul 8.

PMID- 20576654 OWN - NLM STAT- MEDLINE DA - 20100819 DCOM- 20101213 IS - 1569-9285 (Electronic) IS - 1569-9285 (Linking) VI - 11 IP - 3 DP - 2010 Sep TI - Giant cell arteritis presenting as dysarthria and mediastinal mass. PG - 337-9 LID - 10.1510/icvts.2010.239772 [doi] AB - Giant cell arteritis, Takayasu arteritis, and Horton disease are rare, idi opathic diseases that cause chronic inflammation and obliteration of large arterie s, mainly the aorta and its major branches. Histological examination reveals multinucleated giants cells and clinical presentation is characterized by general symptoms and/or symptoms related to stenosis or occlusion of vessels. A ca se of a 50-year-old woman with neurological symptoms, cervicothoracic tumour with severe stenosis of the right subclavian artery and complete occlusion of common c arotid artery is presented. FAU - Ramos, Ricard AU - Ramos R AD - Department of Thoracic Surgery, Hospital Universitari de Bellvitge, L'Hosp italet de Llobregat, Barcelona, Spain. ricardramos@ub.edu FAU - Vila, Ramon AU - Vila R FAU - Penin, Rosa AU - Penin R FAU - Cairols, Marc-Antoni AU - Cairols MA LA - eng PT - Case Reports PT - Journal Article DEP - 20100624 PL - England TA - Interact Cardiovasc Thorac Surg JT - Interactive cardiovascular and thoracic surgery JID - 101158399 SB - IM

MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTAID AID PST SO -

Blood Vessel Prosthesis Implantation Brachiocephalic Trunk/*pathology/surgery Carotid Stenosis/etiology Dysarthria/*etiology/surgery Female Giant Cell Arteritis/complications/*diagnosis/surgery Humans Mediastinal Neoplasms/*etiology/pathology/surgery Middle Aged Sternotomy Subclavian Steal Syndrome/etiology Tomography, X-Ray Computed Treatment Outcome Vascular Neoplasms/*etiology/pathology/surgery 2010/06/26 06:00 2010/12/14 06:00 2010/06/26 06:00 2010/06/24 [aheadofprint] icvts.2010.239772 [pii] 10.1510/icvts.2010.239772 [doi] ppublish Interact Cardiovasc Thorac Surg. 2010 Sep;11(3):337-9. doi: 10.1510/icvts.2010.239772. Epub 2010 Jun 24.

PMID- 20562764 OWN - NLM STAT- MEDLINE DA - 20100621 DCOM- 20100827 LR - 20131121 IS - 0353-5053 (Print) IS - 0353-5053 (Linking) VI - 22 IP - 2 DP - 2010 Jun TI - Premorbid combat related ptsd in Huntington's disease - Case report. PG - 286-8 AB - Huntington's disease (HD) is a neurodegenerative, autosomal dominant disea se that manifests with a triad of symptom clusters including movement disorder, co gnitive impairment and psychiatric symptoms. We present a patient with HD who, pri or to developing neurological signs and symptoms, had been exposed to war trauma and had developed posttraumatic stress disorder. Fifteen years later he manife sted with dysarthria, difficulties with swallowing and involuntary movement. Wh at brought him to psychiatrist was a heteroanamnestically noticed change in personality with irritable mood, impulsivity, aggressive outbursts in beha vior and delusional ideation. Therapy was stared with haloperidol, but patient developed severe extrapiramidal side effects. Subsequent treatment with olanzapine, diazepam and omega 3 fatty acids lead to mood stabilization an d better impulse control with even some improvement in motoric symptoms. To our knowledge, this is the first case report on combat related PTSD as psychia tric

disorder manifested prior to HD. We discuss a possible influence of psycho logical stress disorder on severity of psychiatric symptoms in the HD. The importa nce of personalized approach in both psychopharmacological and psychotherapeutica l treatment of patients with HD is emphasized. If the influence of environme ntal stress on the psychiatric phenotype of the disease should be confirmed by clinical trials and further studies, both screening methods and interventi ons aimed to reduce psychological stress in carriers of Huntington gene could be considered. FAU - Skocic, Milena AU - Skocic M AD - Department of Psychiatry, University Hospital Centre Zagreb, 10000 Zagreb, Croatia. milena.skocic@gmail.com FAU - Dujmovic, Josip AU - Dujmovic J FAU - Jevtovic, Sasa AU - Jevtovic S FAU - Jakovljevic, Miro AU - Jakovljevic M LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Psychiatr Danub JT - Psychiatria Danubina JID - 9424753 RN - 0 (Antipsychotic Agents) RN - 0 (Fatty Acids, Omega-3) RN - 12794-10-4 (Benzodiazepines) RN - 132539-06-1 (olanzapine) RN - Q3JTX2Q7TU (Diazepam) SB - IM MH - Alleles MH - Antipsychotic Agents/therapeutic use MH - Atrophy MH - Benzodiazepines/therapeutic use MH - Cerebral Cortex/pathology MH - Chromosomes, Human, Pair 4/genetics MH - Combat Disorders/*diagnosis/genetics/*psychology/therapy MH - Combined Modality Therapy MH - Comorbidity MH - Diagnosis, Differential MH - Diazepam/therapeutic use MH - Fatty Acids, Omega-3/therapeutic use MH - Genetic Testing MH - Humans MH - Huntington Disease/*diagnosis/genetics/*psychology/therapy MH - Magnetic Resonance Imaging MH - Male MH - Medulla Oblongata/pathology MH - Middle Aged MH - Neurologic Examination MH - Patient Care Team MH - Psychotherapy MH - Risk Factors

MH MH MH EDATMHDACRDTPST SO -

Social Environment Stress Disorders, Post-Traumatic/*diagnosis/genetics/*psychology/therapy Trinucleotide Repeats 2010/06/22 06:00 2010/08/28 06:00 2010/06/22 06:00 ppublish Psychiatr Danub. 2010 Jun;22(2):286-8.

PMID- 20525256 OWN - NLM STAT- MEDLINE DA - 20100713 DCOM- 20101101 LR - 20131121 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 5 DP - 2010 TI - Niemann-Pick disease type C. PG - 16 LID - 10.1186/1750-1172-5-16 [doi] AB - Niemann-Pick C disease (NP-C) is a neurovisceral atypical lysosomal lipid storage disorder with an estimated minimal incidence of 1/120,000 live births. The broad clinical spectrum ranges from a neonatal rapidly fatal disorder to an adul t-onset chronic neurodegenerative disease. The neurological involvement defines th e disease severity in most patients but is typically preceded by systemic si gns (cholestatic jaundice in the neonatal period or isolated spleno- or hepatosplenomegaly in infancy or childhood). The first neurological sympto ms vary with age of onset: delay in developmental motor milestones (early infantil e period), gait problems, falls, clumsiness, cataplexy, school problems (lat e infantile and juvenile period), and ataxia not unfrequently following init ial psychiatric disturbances (adult form). The most characteristic sign is ver tical supranuclear gaze palsy. The neurological disorder consists mainly of cere bellar ataxia, dysarthria, dysphagia, and progressive dementia. Cataplexy, seizur es and dystonia are other common features. NP-C is transmitted in an autosomal re cessive manner and is caused by mutations of either the NPC1 (95% of families) or the NPC2 genes. The exact functions of the NPC1 and NPC2 proteins are still un clear. NP-C is currently described as a cellular cholesterol trafficking defect b ut in the brain, the prominently stored lipids are gangliosides. Clinical examin ation should include comprehensive neurological and ophthalmological evaluations . The primary laboratory diagnosis requires living skin fibroblasts to demonstra

te accumulation of unesterified cholesterol in perinuclear vesicles (lysosome s) after staining with filipin. Pronounced abnormalities are observed in abou t 80% of the cases, mild to moderate alterations in the remainder ("variant" biochemical phenotype). Genotyping of patients is useful to confirm the di agnosis in the latter patients and essential for future prenatal diagnosis. The differential diagnosis may include other lipidoses; idiopathic neonatal he patitis and other causes of cholestatic icterus should be considered in neonates, and conditions with cerebellar ataxia, dystonia, cataplexy and supranuclear ga ze palsy in older children and adults. Symptomatic management of patients is crucial. A first product, miglustat, has been granted marketing authorizat ion in Europe and several other countries for specific treatment of the neurologi cal manifestations. The prognosis largely correlates with the age at onset of the neurological manifestations. FAU - Vanier, Marie T AU - Vanier MT AD - Institut National de la Sante et de la Recherche Medicale, Unite 820, Facu lte de Medecine Lyon-Est Claude Bernard, 7 Rue G, Paradin, F-69008, Lyon, France. marie-t.vanier@inserm.fr LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20100603 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (miglustat) RN - 19130-96-2 (1-Deoxynojirimycin) RN - 97C5T2UQ7J (Cholesterol) SB - IM MH - 1-Deoxynojirimycin/analogs & derivatives/therapeutic use MH - Age Factors MH - Age of Onset MH - Biological Transport/genetics/*physiology MH - Cholesterol/*blood MH - Genetic Association Studies MH - Genetic Testing MH - Humans MH - Niemann-Pick Disease, Type C/drug therapy/genetics/metabolism/*pathology PMC - PMC2902432 OID - NLM: PMC2902432 EDAT- 2010/06/08 06:00 MHDA- 2010/11/03 06:00 CRDT- 2010/06/08 06:00 PHST- 2009/10/07 [received] PHST- 2010/06/03 [accepted] PHST- 2010/06/03 [aheadofprint] AID - 1750-1172-5-16 [pii]

AID - 10.1186/1750-1172-5-16 [doi] PST - epublish SO - Orphanet J Rare Dis. 2010 Jun 3;5:16. doi: 10.1186/1750-1172-5-16. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID AB ncing ative and has important implications for health care providers. Communication se rves many roles for older people, not only establishing and maintaining social affiliations but also providing access to health care services. Health car e providers should be aware of potential communication disorders and make pr ovision for quiet environments, reading materials at appropriate literacy levels, and longer appointments for people with communication difficulties. FAU - Yorkston, Kathryn M AU - Yorkston KM AD - Division of Speech Pathology, Department of Rehabilitation Medicine, Unive rsity of Washington, Box 356490, Seattle, WA 98195-6490, USA. yorkston@u.washing ton.edu <yorkston@u.washington.edu> FAU - Bourgeois, Michelle S AU - Bourgeois MS FAU - Baylor, Carolyn R AU - Baylor CR LA - eng GR - H133B080024/PHS HHS/United States GR - R03 DC010044-01/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review PL - United States TA - Phys Med Rehabil Clin N Am JT - Physical medicine and rehabilitation clinics of North America JID - 9102787 SB - IM MH - Aged MH - Aged, 80 and over MH - Aging/*physiology/psychology MH - *Communication MH - Communication Barriers 20494279 NLM MEDLINE 20100524 20100824 20131023 1558-1381 (Electronic) 1047-9651 (Linking) 21 2 2010 May Communication and aging. 309-19 10.1016/j.pmr.2009.12.011 [doi] People with communication disorders form a diverse group with some experie long-standing disorders and others the onset of new disorders in old age. Regardless of age at onset, the burden of communication disorders is cumul

MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH RF PMC MID OID OID EDATMHDACRDTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI erior

Communication Disorders/diagnosis/epidemiology/*rehabilitation Dysarthria/diagnosis/rehabilitation Female Geriatric Assessment Health Behavior Health Services Accessibility Humans Incidence *Interpersonal Relations Male Memory Disorders/diagnosis/rehabilitation Professional-Patient Relations *Quality of Life Risk Assessment Sensation Disorders/diagnosis/rehabilitation 50 PMC3074568 NIHMS280809 NLM: NIHMS280809 NLM: PMC3074568 2010/05/25 06:00 2010/08/25 06:00 2010/05/25 06:00 S1047-9651(09)00119-3 [pii] 10.1016/j.pmr.2009.12.011 [doi] ppublish Phys Med Rehabil Clin N Am. 2010 May;21(2):309-19. doi: 10.1016/j.pmr.2009.12.011. 20472980 NLM MEDLINE 20100517 20100901 20110729 0300-9173 (Print) 0300-9173 (Linking) 47 2 2010 [A case of myasthenia gravis with an invasive thymoma infiltrating the sup

vena cava and right atrium and causing lung metastasis]. PG - 158-61 AB - A 72-year-old woman was admitted to a local hospital with general fatigue, ptosis and dysarthria. Her anti-AchR antibody titer was high, so myasthenia gravi s was diagnosed. She was given a cholinesterase inhibitor, but her symptoms did not improve. CT and MRI scans revealed a mass in the anterior mediastinum infiltrating the superior vena cava (SVC) and the right atrium (RA) . The diagnosis was an invasive thymoma extending into the SVC and the RA. Moreo ver, there was a mass in the right middle lobe of her lung, which was suspected to be the result of metastasis of the thymoma. She was transferred to our hospit al for medication and surgery for the invasive thymoma. Urgent surgery was perfor med

without preoperative therapy, because the tumor was nearly obstructing her tricuspid valve. An expanded thymomectomy and a right middle lobectomy wer e performed. As the tumor had infiltrated into the SVC, the SVC was replaced with an artificial graft. The clinicopathological diagnosis of thymoma (Masaoka Stage IVb) was given. The patient had a myasthenic crisis for several weeks afte r surgery, so her breathing was controlled by an artificial respirator. Her symptoms improved after treatment with steroids, tacrolimus and a cholines terase inhibitor. Although major surgery was required to prevent tumor embolism, the patient survived. Careful observation is necessary to detect signs of rela pse of invasive thymoma. FAU - Ochi, Masayuki AU - Ochi M AD - Department of Geriatric Medicine, Medicine and Bioscience, Graduate School of Medicine, Ehime University. FAU - Toi, Takayuki AU - Toi T FAU - Kamogawa, Kenji AU - Kamogawa K FAU - Nagai, Tokihisa AU - Nagai T FAU - Taguchi, Keiko AU - Taguchi K FAU - Kawano, Yuji AU - Kawano Y FAU - Igase, Michiya AU - Igase M FAU - Kohara, Katsuhiko AU - Kohara K FAU - Miki, Tetsuro AU - Miki T LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Nihon Ronen Igakkai Zasshi JT - Nihon Ronen Igakkai zasshi. Japanese journal of geriatrics JID - 7507332 SB - IM MH - Aged MH - Female MH - Heart Atria/*pathology MH - Humans MH - Lung Neoplasms/*secondary MH - Myasthenia Gravis/*complications MH - Thymoma/*complications/*pathology MH - Thymus Neoplasms/*complications/*pathology MH - Vena Cava, Superior/*pathology EDAT- 2010/05/18 06:00 MHDA- 2010/09/02 06:00 CRDT- 2010/05/18 06:00 AID - JST.JSTAGE/geriatrics/47.158 [pii]

PST - ppublish SO - Nihon Ronen Igakkai Zasshi. 2010;47(2):158-61. PMID- 20460952 OWN - NLM STAT- MEDLINE DA - 20100714 DCOM- 20101019 LR - 20131023 IS - 1423-0372 (Electronic) IS - 1011-6125 (Linking) VI - 88 IP - 4 DP - 2010 TI - Patient-specific model-based investigation of speech intelligibility and m ovement during deep brain stimulation. PG - 224-33 LID - 10.1159/000314357 [doi] AB - BACKGROUND/AIMS: Deep brain stimulation (DBS) is widely used to treat moto r symptoms in patients with advanced Parkinson's disease. The aim of this st udy was to investigate the anatomical aspects of the electric field in relation to effects on speech and movement during DBS in the subthalamic nucleus. METH ODS: Patient-specific finite element models of DBS were developed for simulatio n of the electric field in 10 patients. In each patient, speech intelligibility and movement were assessed during 2 electrical settings, i.e. 4 V (high) and 2 V (low). The electric field was simulated for each electrical setting. RESUL TS: Movement was improved in all patients for both high and low electrical set tings. In general, high-amplitude stimulation was more consistent in improving th e motor scores than low-amplitude stimulation. In 6 cases, speech intelligibility was impaired during high-amplitude electrical settings. Stimulation of part of the fasciculus cerebellothalamicus from electrodes positioned medial and/or po sterior to the center of the subthalamic nucleus was recognized as a possible caus e of the stimulation-induced dysarthria. CONCLUSION: Special attention to stimulation-induced speech impairments should be taken in cases when activ e electrodes are positioned medial and/or posterior to the center of the subthalamic nucleus. CI - 2010 S. Karger AG, Basel. FAU - Astrom, Mattias AU - Astrom M AD - Department of Biomedical Engineering, Linkoping University, Linkoping, Swe den. matas@imt.liu.se FAU - Tripoliti, Elina AU - Tripoliti E FAU - Hariz, Marwan I

AU - Hariz MI FAU - Zrinzo, Ludvic U AU - Zrinzo LU FAU - Martinez-Torres, Irene AU - Martinez-Torres I FAU - Limousin, Patricia AU - Limousin P FAU - Wardell, Karin AU - Wardell K LA - eng GR - G-4070/Parkinson's UK/United Kingdom GR - R01-NS40902/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20100512 PL - Switzerland TA - Stereotact Funct Neurosurg JT - Stereotactic and functional neurosurgery JID - 8902881 SB - IM MH - Aged MH - Brain Mapping MH - *Deep Brain Stimulation MH - Electrodes, Implanted MH - Female MH - Humans MH - Male MH - Middle Aged MH - Models, Neurological MH - Movement/*physiology MH - Parkinson Disease/*therapy MH - Speech Intelligibility/*physiology MH - Stereotaxic Techniques MH - Subthalamic Nucleus/*physiopathology/surgery PMC - PMC3214825 OID - NLM: PMC3214825 EDAT- 2010/05/13 06:00 MHDA- 2010/10/20 06:00 CRDT- 2010/05/13 06:00 PHST- 2009/10/01 [received] PHST- 2010/03/04 [accepted] PHST- 2010/05/12 [aheadofprint] AID - 000314357 [pii] AID - 10.1159/000314357 [doi] PST - ppublish SO - Stereotact Funct Neurosurg. 2010;88(4):224-33. doi: 10.1159/000314357. Epu b 2010 May 12. PMIDOWN STATDA DCOMIS IS VI IP DP 20453409 NLM MEDLINE 20100510 20100811 1349-7235 (Electronic) 0918-2918 (Linking) 49 9 2010

TI PG AB a.

- Clinical study of the responsible lesion for dysarthria in the cerebellum. - 861-4 - There have been few reports describing the lesion for cerebellar dysarthri We compared MRI findings of 4 reported patients (including our previously rep orted patient) to that of our patient who showed ataxic speech and ataxic gait. The lesions of 4 patients involved lobulus quadrangularis and lobulus simplex, and the lesion of the present patient involved lobulus semilunaris superior an d lobulus simplex. Since lobulus simplex and lobulus quadrangularis were inv olved in many patients, we speculated that the cerebellar dysarthria of the pres ent patient was due to the damage of these areas in the upper cerebellum. FAU - Ogawa, Katsuhiko AU - Ogawa K AD - Division of Neurology, Department of Medicine, Nihon University, School of Medicine, Tokyo. ogawak@med.nihon-u.ac.jp FAU - Yoshihashi, Hirokazu AU - Yoshihashi H FAU - Suzuki, Yutaka AU - Suzuki Y FAU - Kamei, Satoshi AU - Kamei S FAU - Mizutani, Tomohiko AU - Mizutani T LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20100430 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM MH - Aged MH - Cerebellum/blood supply/*pathology MH - Cerebral Infarction/*complications/diagnosis MH - Dysarthria/diagnosis/*etiology/therapy MH - Female MH - Follow-Up Studies MH - Humans MH - Magnetic Resonance Imaging/methods MH - Neurologic Examination MH - Severity of Illness Index RF - 14 EDAT- 2010/05/11 06:00 MHDA- 2010/08/12 06:00 CRDT- 2010/05/11 06:00 PHST- 2010/04/30 [epublish] AID - JST.JSTAGE/internalmedicine/49.2913 [pii] PST - ppublish SO - Intern Med. 2010;49(9):861-4. Epub 2010 Apr 30. PMID- 20376593 OWN - NLM

STATDA DCOMLR IS IS VI IP DP TI -

MEDLINE 20100811 20101029 20131121 1556-9039 (Print) 1556-9039 (Linking) 6 2 2010 Jun Clinical effects and toxicokinetic evaluation following massive topiramate ingestion. PG - 135-8 LID - 10.1007/s13181-010-0065-y [doi] AB - Topiramate is used to treat a variety of neurologic and psychiatric diseas es due to its benign safety profile. Data regarding the toxicity and toxicokineti cs of topiramate in acute overdose are limited. A case of massive, acute ingesti on resulting in the highest reported topiramate level is presented, including toxicokinetic evaluation. A 37-year-old woman presented with coma unrespon sive to naloxone following topiramate ingestion. She had normal vital signs withou t respiratory depression. She was intubated for airway protection, given 3.5 mg lorazepam IV for facial and neck muscle twitching, and transferred to our facility. No additional sedation was required for 18 h on the ventilator. Following mental status improvement, the patient was extubated. Confusion, dysarthria, and imbalance resolved over the next 2 days. Nonanion gap meta bolic acidosis persisted for 3 days. Peak serum topiramate level was 356.6 micro g/ml (reference range, 5-20 microg/ml). Massive topiramate ingestion led to pro longed coma with normal vital signs and nonanion gap metabolic acidosis. Coma of this severity has not been previously reported. Serum half-life, which has not been studied after overdose, was 16 h. Despite the large ingestion and signific ant presenting symptoms, the patient recovered fully with supportive intensive care alone. Massive acute topiramate ingestion may lead to nonanion gap metabol ic acidosis and prolonged coma which resolves with intensive supportive care. Toxicokinetic data following large, suicidal ingestion of topiramate were similar to previously published pharmacokinetic information. FAU - Lynch, Michael J AU - Lynch MJ AD - Division of Medical Toxicology, Department of Emergency Medicine, Universi ty of Pittsburgh Medical Center, Pittsburgh, PA, USA. lyncmj@upmc.edu FAU - Pizon, Anthony F AU - Pizon AF FAU - Siam, Mohamed G AU - Siam MG FAU - Krasowski, Matthew D AU - Krasowski MD

LA GR GR GR PT PT PT PL TA JT f

eng K08 GM074238-03/GM/NIGMS NIH HHS/United States K08 GM074238-04/GM/NIGMS NIH HHS/United States K08-GM074238/GM/NIGMS NIH HHS/United States Case Reports Journal Article Research Support, N.I.H., Extramural United States J Med Toxicol Journal of medical toxicology : official journal of the American College o Medical Toxicology 101284598 0 (Anticonvulsants) 0 (Hypnotics and Sedatives) 0H73WJJ391 (topiramate) 30237-26-4 (Fructose) O26FZP769L (Lorazepam) IM Acidosis/chemically induced Adult Anticonvulsants/*pharmacokinetics/*poisoning/therapeutic use Bipolar Disorder/drug therapy/psychology Blood Gas Analysis Coma/chemically induced Drug Overdose Female Fructose/*analogs & derivatives/pharmacokinetics/poisoning/therapeutic use Gas Chromatography-Mass Spectrometry Humans Hypnotics and Sedatives/therapeutic use Intensive Care Lorazepam/therapeutic use Respiration, Artificial PMC2916051 NIHMS198235 NLM: NIHMS198235 NLM: PMC2916051 2010/04/09 06:00 2010/10/30 06:00 2010/04/09 06:00 10.1007/s13181-010-0065-y [doi] ppublish J Med Toxicol. 2010 Jun;6(2):135-8. doi: 10.1007/s13181-010-0065-y.

JID RN RN RN RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI -

20363963 NLM MEDLINE 20100528 20100820 20131121 1708-8283 (Electronic) 0883-0738 (Linking) 25 6 2010 Jun Hyperperfusion on magnetic resonance imaging in acute chemotherapy-related leukoencephalopathy. PG - 776-9 LID - 10.1177/0883073809346349 [doi]

AB - Acute chemotherapy-related leukoencephalopathy can present similar to acut e stroke with symptoms including aphasia, dysarthria, and hemiplegia. Differentiation based on clinical appearance is challenging, and physician s must distinguish between the 2 conditions rapidly to institute appropriate ther apies. An 8-year-old male with acute lymphoblastic leukemia receiving chemotherap y, including intrathecal methotrexate, presented to our emergency center with 2 hours of expressive aphasia and flaccid right hemiplegia. Emergent magneti c resonance imaging (MRI) was obtained, demonstrating diffusion restriction within bilateral corona radiata and centrum semiovale. Magnetic resonance perfusi on revealed mildly increased perfusion, a finding inconsistent with ischemic stroke and previously unreported in acute chemotherapy-related leukoencephalopath y without necrosis. This increased perfusion conclusively eliminated stroke from the clinical differential. Magnetic resonance perfusion imaging proved val uable to rapidly distinguish acute chemotherapy-related leukoencephalopathy from ischemia, and the evaluation of perfusion alterations in this disorder may provide further insight into the pathophysiology of this entity. FAU - El-Hakam, Lisa Michael AU - El-Hakam LM AD - Department of Pediatrics, Section of Pediatric Neurology and Developmental Neuroscience, Baylor College of Medicine, Texas Children's Hospital, Houst on, Texas 77030, USA. hakam@bcm.edu FAU - Ramocki, Melissa Beth AU - Ramocki MB FAU - Riviello, James John AU - Riviello JJ FAU - Illner, Anna AU - Illner A LA - eng GR - 5K08NS062711-02/NS/NINDS NIH HHS/United States GR - K08 NS062711/NS/NINDS NIH HHS/United States GR - K08 NS062711-02/NS/NINDS NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20100402 PL - United States TA - J Child Neurol JT - Journal of child neurology JID - 8606714 RN - 0 (Polyethylene Glycols) RN - 0 (pegaspargase) RN - 04079A1RDZ (Cytarabine) RN - 57-22-7 (Vincristine) RN - EC 3.5.1.1 (Asparaginase) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Acute Disease

MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTPHSTAID AID PST SO 2010 PMIDOWN STATDA DCOMLR IS IS VI DP TI s

Antineoplastic Combined Chemotherapy Protocols/*adverse effects Asparaginase/adverse effects Child Cytarabine/adverse effects Diagnosis, Differential Humans Leukoencephalopathies/*chemically induced/*diagnosis Magnetic Resonance Imaging Male Methotrexate/adverse effects Polyethylene Glycols/adverse effects Precursor Cell Lymphoblastic Leukemia-Lymphoma/*drug therapy Stroke/diagnosis Vincristine/adverse effects PMC2936236 NIHMS233333 NLM: NIHMS233333 NLM: PMC2936236 2010/04/07 06:00 2010/08/21 06:00 2010/04/06 06:00 2010/04/02 [aheadofprint] 0883073809346349 [pii] 10.1177/0883073809346349 [doi] ppublish J Child Neurol. 2010 Jun;25(6):776-9. doi: 10.1177/0883073809346349. Epub Apr 2. 20233583 NLM MEDLINE 20100611 20110125 20131024 1872-6240 (Electronic) 0006-8993 (Linking) 1341 2010 Jun 23 Targeted exercise therapy for voice and swallow in persons with Parkinson'

disease. PG - 3-11 LID - 10.1016/j.brainres.2010.03.029 [doi] AB - Sensorimotor deficits affecting voice and swallowing ability can have a devastating impact on the quality of life of people with Parkinson disease (PD). Recent scientific findings in animal models of PD pinpoint targeted exerci se therapy as a potential treatment to reduce neurochemical loss and decrease parkinsonian symptoms. Although there may be beneficial effects, targeted exercise therapy is not a standard component of therapy for the cranial sensiromotor deficits seen in PD. In this paper, we review the scientific evidence for targeted training for voice and swallowing deficits. The lite rature search revealed 19 publications that included targeted training for voice and only one publication that included targeted training for swallowing. We su mmarize 3 main findings: (1) targeted training may be associated with lasting chan

ges in voice behavior; (2) targeted training of sensorimotor actions with anatomi cal or functional overlap with voice and swallowing may improve voice and swallow ing to some degree, but it is unknown whether these effects endure over time; and (3) evidence regarding cranial sensorimotor interventions for Parkinson diseas e is sparse. We concluded that targeted training for voice and swallow is a pro mising but understudied intervention for cranial sensorimotor deficits associated with PD and posit that animal models can be useful in designing empirically bas ed studies that further the science on targeted training. CI - Copyright 2010 Elsevier B.V. All rights reserved. FAU - Russell, John A AU - Russell JA AD - University of Wisconsin School of Medicine and Public Health, Otolaryngolo gy Head and Neck Surgery, USA. russell@surgery.wisc.edu FAU - Ciucci, Michelle R AU - Ciucci MR FAU - Connor, Nadine P AU - Connor NP FAU - Schallert, Timothy AU - Schallert T LA - eng GR - P30 DC010754-01/DC/NIDCD NIH HHS/United States GR - R01 DC008149-01A1/DC/NIDCD NIH HHS/United States GR - R01 DC008149-05/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Review DEP - 20100315 PL - Netherlands TA - Brain Res JT - Brain research JID - 0045503 SB - IM MH - Animals MH - Deglutition/*physiology MH - Deglutition Disorders/etiology/physiopathology/*therapy MH - Dysarthria/etiology/physiopathology/*therapy MH - Dysphonia/etiology/physiopathology/*therapy MH - Exercise Therapy/*methods MH - Humans MH - Parkinson Disease/complications/physiopathology/*therapy MH - Phonation/*physiology RF - 80 PMC - PMC2908992 MID - NIHMS200160 OID - NLM: NIHMS200160 OID - NLM: PMC2908992 EDAT- 2010/03/18 06:00 MHDA- 2011/01/28 06:00 CRDT- 2010/03/18 06:00 PHST- 2010/02/17 [received] PHST- 2010/03/08 [accepted] PHST- 2010/03/15 [aheadofprint]

AID - S0006-8993(10)00573-1 [pii] AID - 10.1016/j.brainres.2010.03.029 [doi] PST - ppublish SO - Brain Res. 2010 Jun 23;1341:3-11. doi: 10.1016/j.brainres.2010.03.029. Epu b 2010 Mar 15. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI l 20228603 NLM MEDLINE 20100315 20100610 20131121 1349-7235 (Electronic) 0918-2918 (Linking) 49 6 2010 Central pontine and extrapontine myelinolysis that developed during alcoho

withdrawal, without hyponatremia, in a chronic alcoholic. PG - 615-8 AB - Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are osmotic demyelination syndrome. A 45-year-old man with a history of alcoho lism visited the ER with dysarthria and dysphagia for 2 days. These symptoms oc curred 3 days after he had stopped drinking alcohol. The neurological symptoms progressed to anarthria, pseudobulbar palsy and gait disturbance. During admission, the electrolyte studies were within the normal range. Diffusion-weighted images revealed high signal intensities in the pons, th alamus and basal ganglia. Apparent diffusion coefficient image showed low signal intensities in the pontine lesion, but isosignal intensities in the extrap ontine lesion. The symptoms gradually improved after 1 month with only conservati ve treatment. The 1 month-follow-up MRI showed significant reduction of the p revious extrapontine lesions. These findings suggest that cytotoxic edema is centr al to the pathogenesis of CPM, but vasogenic edema plays an important role in th e pathogenesis of EPM occurring during alcohol withdrawal. FAU - An, Jae Young AU - An JY AD - Department of Neurology, College of Medicine, The Catholic University of K orea, Seoul, Korea. nrjyan@gmail.com FAU - Park, Sung Kyung AU - Park SK FAU - Han, Si Ryung AU - Han SR FAU - Song, In Uk AU - Song IU LA - eng PT - Case Reports PT - Journal Article DEP - 20100315 PL - Japan

TA JT JID RN SB MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTAID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI derly

Intern Med Internal medicine (Tokyo, Japan) 9204241 3K9958V90M (Ethanol) IM Alcoholism/*complications/therapy Edema/complications Ethanol/*adverse effects Humans Male Middle Aged Myelinolysis, Central Pontine/diagnosis/*etiology Prognosis Substance Withdrawal Syndrome/*complications 2010/03/17 06:00 2010/06/11 06:00 2010/03/16 06:00 2010/03/15 [epublish] JST.JSTAGE/internalmedicine/49.3069 [pii] ppublish Intern Med. 2010;49(6):615-8. Epub 2010 Mar 15. 20203552 NLM MEDLINE 20100305 20100525 1827-1596 (Electronic) 0375-9393 (Linking) 76 3 2010 Mar Importance of perioperative monitoring of cerebral tissue saturation in el

patients: an interesting case. PG - 232-5 AB - The authors describe the case of an elderly diabetic patient with a hip fr acture who developed neurocognitive dysfunction and dysarthria preoperatively. Up on arrival in the operating room, the monitoring of cerebral oxygenation by near-infrared spectroscopy (NIRS) showed cerebral desaturation (44% on the left hemisphere and 46% on the right). Cerebral oximetry values were stabilized during the surgery after administration of crystalloid fluids and vasoconstrictiv e drugs and were ameliorated significantly after administration of two units of bl ood. The patient's cerebral saturation was 60% on the left and 58% on the right hemisphere after the end of surgery and he was in normal neurological stat us. Observations underlined the importance of preoperative evaluation of cereb ral tissue oxygenation by non-invasive cerebral NIRS in elderly diabetic patie nts who develop hypovolemia and anemia due to major fracture. FAU - Tzimas, P AU - Tzimas P AD - Department of Anesthesiology and Postoperative Intensive Care, University

of FAU AU FAU AU FAU AU LA PT PT DEP PL TA JT JID SB MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTPHSTAID PST SO PMIDOWN STATDA DCOMIS IS VI DP TI Ioannina - School of Medicine, Ioannina, Greece. petzimas@gmail.com Liarmakopoulou, A Liarmakopoulou A Arnaoutoglou, H Arnaoutoglou H Papadopoulos, G Papadopoulos G eng Case Reports Journal Article 20091130 Italy Minerva Anestesiol Minerva anestesiologica 0375272 IM Aged Brain Chemistry/*physiology Hip Fractures/surgery Humans Hypoxia, Brain/*diagnosis/prevention & control Male *Monitoring, Intraoperative Orthopedic Procedures Oxygen Consumption/*physiology Spectroscopy, Near-Infrared 2010/03/06 06:00 2010/05/26 06:00 2010/03/06 06:00 2009/11/30 [aheadofprint] R02095512 [pii] ppublish Minerva Anestesiol. 2010 Mar;76(3):232-5. Epub 2009 Nov 30.

20184678 NLM PubMed-not-MEDLINE 20100226 20100714 1757-1626 (Electronic) 1757-1626 (Linking) 2 2009 Reversible oropharyngeal dysphagia secondary to cricopharyngeal sphincter achalasia in a patient with myasthenia gravis: a case report. PG - 6565 LID - 10.1186/1757-1626-0002-0000006565 [doi] AB - Bulbar weakness and fatigue resulting in dysphagia and dysarthria is commo n in myasthenia gravis. In chronic MG it is often assumed that these symptoms h erald an exacerbation of the patient's disease and doses of cholinergic agents a nd immunomodulatory therapies may be increased, along with initiation of plas ma exchange. A case is presented in which dysphagia was refractory to standar d MG therapy, leading to the subsequent discovery of cricopharyngeal sphincter achalasia as the primary cause of the patient's symptoms rather than an as

sumed myasthenia gravis exacerbation. The patient's dysphagia resolved after eso phageal dilatation. Cricopharyngeal sphincter achalasia is a common disorder produ cing dysphagia in the elderly and needs to be considered in the evaluation of a myasthenic patient with worsening dysphagia when standard myasthenia gravi s therapy fails. Discussion of myasthenia gravis, cholinergic therapy and cricopharyngeal sphincter achalasia is undertaken. Clinicians are encourag ed to consider non-neurologic causes of worsening dysphagia in the myasthenic pa tient. FAU - Rison, Richard A AU - Rison RA AD - University of Southern California, Keck School of Medicine, Los Angeles Co unty Medical Center, Presbyterian Intercommunity Hospital, Neurology Consultant s Medical Group, Whittier, CA, USA. rison@usc.edu LA - eng PT - Journal Article DEP - 20090807 PL - England TA - Cases J JT - Cases journal JID - 101474272 PMC - PMC2827116 OID - NLM: PMC2827116 EDAT- 2010/02/27 06:00 MHDA- 2010/02/27 06:01 CRDT- 2010/02/27 06:00 PHST- 2009/03/14 [received] PHST- 2009/07/23 [accepted] PHST- 2009/08/07 [aheadofprint] AID - 1757-1626-0002-0000006565 [pii] AID - 10.1186/1757-1626-0002-0000006565 [doi] PST - epublish SO - Cases J. 2009 Aug 7;2:6565. doi: 10.1186/1757-1626-0002-0000006565. PMIDOWN STATDA DCOMLR IS IS VI DP TI PG LID AB opment and deterioration of several autoimmune conditions, including myasthenia g ravis. CASE PRESENTATION: We report the case of a 60-year-old Caucasian man who presented with acute onset of dysarthria and dysphagia initially attribute 20170525 NLM PubMed-not-MEDLINE 20100309 20110714 20120516 1752-1947 (Electronic) 1752-1947 (Linking) 4 2010 Statin-associated weakness in myasthenia gravis: a case report. 61 10.1186/1752-1947-4-61 [doi] INTRODUCTION: Myasthenia gravis is a commonly undiagnosed condition in the elderly. Statin medications can cause weakness and are linked to the devel

d to a brain stem stroke. Oculobulbar and limb weakness progressed until myasthen ia gravis was diagnosed and treated, and until statin therapy was finally wit hdrawn. CONCLUSION: Myasthenia gravis may be underappreciated as a cause of acute bulbar weakness among the elderly. Statin therapy appeared to have contributed to the weakness in our patient who was diagnosed with myasthenia gravis. FAU - Keogh, Michael J AU - Keogh MJ AD - Department of Stroke Medicine, United Lincolnshire Hospitals Trust, Lincol n County Hospital, Lincoln, LN2 5QY, UK. mikekeogh@doctors.org.uk. FAU - Findlay, John M AU - Findlay JM FAU - Leach, Simon AU - Leach S FAU - Bowen, John AU - Bowen J LA - eng PT - Journal Article DEP - 20100220 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 PMC - PMC2834677 OID - NLM: PMC2834677 EDAT- 2010/02/23 06:00 MHDA- 2010/02/23 06:01 CRDT- 2010/02/23 06:00 PHST- 2008/01/29 [received] PHST- 2010/02/20 [accepted] PHST- 2010/02/20 [aheadofprint] AID - 1752-1947-4-61 [pii] AID - 10.1186/1752-1947-4-61 [doi] PST - epublish SO - J Med Case Rep. 2010 Feb 20;4:61. doi: 10.1186/1752-1947-4-61. PMIDOWN STATDA DCOMIS IS VI IP DP TI PG LID AB nial r history of radiation therapy presented with complaints of increased drowsi ness, 20160365 NLM MEDLINE 20100217 20100511 1998-4138 (Electronic) 1998-4138 (Linking) 5 4 2009 Oct-Dec Three distinct co-existent primary brain tumors in a patient. 293-6 10.4103/0973-1482.59914 [doi] A rare case of simultaneous occurrence of three entirely distinct intracra tumors is described. A 55-year-old male with no evidence of phacomatoses o

headaches, and dysarthria. Investigations revealed an olfactory groove meningioma, a glioblastoma multiforme in the left medial temporal lobe, an d a diffuse glioma in the brain stem. Occurrence of multiple varieties of tumo rs at the same time is extremely rare. Theories that explain their occurrences including the role of common carcinogens, autocrine growth factors, and tu mor suppressor genes are discussed. FAU - Iyer, Veena R AU - Iyer VR AD - Department of Radiology, Seth G. S. Medical College and King Edward Memori al Hospital, Parel, Mumbai, India. FAU - Sanghvi, Darshana A AU - Sanghvi DA FAU - Shenoy, Asha AU - Shenoy A FAU - Goel, Atul AU - Goel A LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Cancer Res Ther JT - Journal of cancer research and therapeutics JID - 101249598 SB - IM MH - Brain Neoplasms/*pathology/surgery MH - Brain Stem Neoplasms/pathology/surgery MH - Glioblastoma/*pathology/surgery MH - Glioma/*pathology/surgery MH - Humans MH - Magnetic Resonance Imaging MH - Male MH - Meningeal Neoplasms/*pathology/surgery MH - Meningioma/*pathology/surgery MH - Middle Aged MH - Neoplasms, Multiple Primary/*pathology/surgery MH - Temporal Lobe/pathology/surgery EDAT- 2010/02/18 06:00 MHDA- 2010/05/12 06:00 CRDT- 2010/02/18 06:00 AID - JCanResTher_2009_5_4_293_59914 [pii] AID - 10.4103/0973-1482.59914 [doi] PST - ppublish SO - J Cancer Res Ther. 2009 Oct-Dec;5(4):293-6. doi: 10.4103/0973-1482.59914. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 20063431 NLM MEDLINE 20100323 20100810 20131217 1531-8257 (Electronic) 0885-3185 (Linking) 25 4 2010 Mar 15 Measuring the rate of progression in Friedreich ataxia: implications for c

linical trial design. PG - 426-32 LID - 10.1002/mds.22912 [doi] AB - Friedreich ataxia is an autosomal recessive neurodegenerative disorder characterized by ataxia of all four limbs, dysarthria, and arreflexia. A v ariety of measures are currently used to quantify disease progression, including the Friedreich Ataxia Rating Scale, examiner-rated functional disability scale s, self-reported activities of daily living and performance measures such as the timed 25-foot walk, 9-hole pegboard test, PATA speech test, and low-contra st letter acuity vision charts. This study examines the rate of disease progr ession over one and two years in a cohort of 236 Friedreich ataxia patients using these scales and performance measure composites. The Friedreich Ataxia Rating Sc ale and performance-measure composites captured disease progression, with a greate r sensitivity to change over 2 years than over 1 year. The measures differed in their sensitivity to change and in possible bias. These results help to es tablish norms for progression in FRDA that can be useful in measuring the long-ter m success of therapeutic agents and defining sample-size calculations for double-blind clinical trials. FAU - Friedman, Lisa S AU - Friedman LS AD - Department of Neurology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA. FAU - Farmer, Jennifer M AU - Farmer JM FAU - Perlman, Susan AU - Perlman S FAU - Wilmot, George AU - Wilmot G FAU - Gomez, Christopher M AU - Gomez CM FAU - Bushara, Khalaf O AU - Bushara KO FAU - Mathews, Katherine D AU - Mathews KD FAU - Subramony, S H AU - Subramony SH FAU - Ashizawa, Tetsuo AU - Ashizawa T FAU - Balcer, Laura J AU - Balcer LJ FAU - Wilson, Robert B AU - Wilson RB FAU - Lynch, David R AU - Lynch DR LA - eng GR - NS041547/NS/NINDS NIH HHS/United States GR - NS050733/NS/NINDS NIH HHS/United States

GR GR GR GR GR PT PT PT PL TA JT JID RN RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTAID PST SO -

NS45986/NS/NINDS NIH HHS/United States R01 NSO043264/PHS HHS/United States RC1 NS068897/NS/NINDS NIH HHS/United States RC1 NS068897-01/NS/NINDS NIH HHS/United States U54 NS053672/NS/NINDS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States Mov Disord Movement disorders : official journal of the Movement Disorder Society 8610688 0 (DNA, Mitochondrial) 0 (Iron-Binding Proteins) 0 (frataxin) IM Activities of Daily Living Adult Aged Clinical Trials as Topic DNA, Mitochondrial/genetics Disease Progression Double-Blind Method Female Friedreich Ataxia/*diagnosis/genetics/*physiopathology Humans Iron-Binding Proteins/genetics Male Middle Aged Neurologic Examination Point Mutation/genetics Severity of Illness Index Speech Disorders/diagnosis Trinucleotide Repeats/genetics Walking Young Adult PMC2954653 NIHMS217747 NLM: NIHMS217747 NLM: PMC2954653 2010/01/12 06:00 2010/08/11 06:00 2010/01/12 06:00 10.1002/mds.22912 [doi] ppublish Mov Disord. 2010 Mar 15;25(4):426-32. doi: 10.1002/mds.22912.

PMID- 20046527 OWN - NLM STAT- MEDLINE DA - 20100104 DCOM- 20100401 LR - 20130825 IS - 1976-2437 (Electronic) IS - 0513-5796 (Linking) VI - 51 IP - 1 DP - 2010 Jan TI - Cerebellar nocardiosis and myopathy from long-term corticosteroids for idi opathic

thrombocytopenia. PG - 131-7 LID - 10.3349/ymj.2010.51.1.131 [doi] AB - Infection of the central nervous system with Nocardia sp. usually manifest s as supratentorial abscesses. Supratentorial and cerebellar abscesses from inf ection with Nocardia sp. following immunosuppression with long-term corticosteroi ds for idiopathic thrombocytopenia (ITP) have not been reported. An 83 years-old, human immunodeficiency virus (HIV)-negative, polymorbid male with ITP for which he required corticosteroids since age 53 years developed tiredness, dyspnoea, hemoptysis, abdominal pain, and progressive gait disturbance. Imaging stud ies of the lung revealed an enhancing tumour in the right upper lobe with central and peripheral necrosis, multiple irregularly contoured hyperdensities over bo th lungs, and right-sided pleural effusions. Sputum culture grew Nocardia sp. Neurological diagnostic work-up revealed dysarthria, dysphagia, ptosis, hypoacusis, tremor, dysdiadochokinesia, proximal weakness of the lower lim bs, diffuse wasting, and stocking-type sensory disturbances. The neurological deficits were attributed to an abscess in the upper cerebellar vermis, myo pathy from corticosteroids, and polyneuropathy. Meropenem for 37 days and trimethoprime-sulfamethoxazole for 3 months resulted in a reduction of the pulmonary, but not the cerebral lesions. Therefore, sultamicillin was begu n, but without success. Long-term therapy with corticosteroids for ITP may induce not only steroid myopathy but also immune-incompetence with the development of pulmonary and cerebral nocardiosis. Cerebral nocardiosis may not sufficien tly respond to long-term antibiotic therapy why switching to alternative antib iotics or surgery may be necessary. FAU - Frank, Marlies AU - Frank M AD - First Medical Department, Hospital Rudolfstiftung,Vienna, Austria. FAU - Woschnagg, Herbert AU - Woschnagg H FAU - Molzer, Gunther AU - Molzer G FAU - Finsterer, Josef AU - Finsterer J LA - eng PT - Case Reports PT - Journal Article DEP - 20091229 PL - Korea (South) TA - Yonsei Med J JT - Yonsei medical journal JID - 0414003 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/*adverse effects/*therapeutic use MH - Aged, 80 and over

MH - Cerebellar Diseases/*chemically induced/*diagnosis/pathology MH - Humans MH - Immunosuppression MH - Male MH - Muscular Diseases/*chemically induced/pathology MH - Nocardia Infections/*diagnosis MH - Purpura, Thrombocytopenic, Idiopathic/*drug therapy PMC - PMC2799960 OID - NLM: PMC2799960 OTO - NOTNLM OT - Infection OT - antibiotics OT - brain abscess OT - immunosuppression OT - opportunistic OT - steroid myopathy EDAT- 2010/01/05 06:00 MHDA- 2010/04/02 06:00 CRDT- 2010/01/05 06:00 PHST- 2008/01/22 [received] PHST- 2008/05/09 [revised] PHST- 2008/05/14 [accepted] PHST- 2009/12/29 [epublish] AID - 10.3349/ymj.2010.51.1.131 [doi] PST - ppublish SO - Yonsei Med J. 2010 Jan;51(1):131-7. doi: 10.3349/ymj.2010.51.1.131. Epub 2 009 Dec 29. PMIDOWN STATDA DCOMLR IS IS VI DP TI 20021666 NLM MEDLINE 20100112 20100224 20131121 1471-2377 (Electronic) 1471-2377 (Linking) 9 2009 Tetrabenazine as anti-chorea therapy in Huntington disease: an open-label continuation study. Huntington Study Group/TETRA-HD Investigators. PG - 62 LID - 10.1186/1471-2377-9-62 [doi] AB - BACKGROUND: Tetrabenazine (TBZ) selectively depletes central monoamines by reversibly binding to the type-2 vesicular monoamine transporter. A previo us double blind study in Huntington disease (HD) demonstrated that TBZ effect ively suppressed chorea, with a favorable short-term safety profile (Neurology 2006;66:366-372). The objective of this study was to assess the long-term safety and effectiveness of TBZ for chorea in HD. METHODS: Subjects who completed the 13-week, double blind protocol were invited to participate in this open la bel extension study for up to 80 weeks. Subjects were titrated to the best ind ividual dose or a maximum of 200 mg/day. Chorea was assessed using the Total Maxim al Chorea (TMC) score from the Unified Huntington Disease Rating Scale. RESUL

TS: Of the 75 participants, 45 subjects completed 80 weeks. Three participants terminated due to adverse events (AEs) including depression, delusions wit h associated previous suicidal behavior, and vocal tics. One subject died du e to breast cancer. The other 26 subjects chose not to continue on with each en suing extension for various reasons. When mild and unrelated AEs were excluded, the most commonly reported AEs (number of subjects) were sedation/somnolence ( 18), depressed mood (17), anxiety (13), insomnia (10), and akathisia (9). Parki nsonism and dysarthria [corrected] scores were significantly increased at week 80 compared to baseline. At week 80, chorea had significantly improved from b aseline with a mean reduction in the TMC score of 4.6 (SD 5.5) units. The mean dos age at week 80 was 63.4 mg (range 12.5-175 mg). CONCLUSIONS: TBZ effectively supp resses HD-related chorea for up to 80 weeks. Patients treated chronically with TB Z should be monitored for parkinsonism, dysphagia and other side effects inc luding sleep disturbance, depression, anxiety, and akathisia. TRIAL REGISTRATION: Clinicaltrials.gov registration number (initial study): NCT00219804. FAU - Frank, Samuel AU - Frank S AD - samuel.frank@bmc.org LA - eng SI - ClinicalTrials.gov/NCT00219804 PT - Clinical Trial PT - Journal Article PT - Randomized Controlled Trial DEP - 20091218 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 RN - 0 (Adrenergic Uptake Inhibitors) RN - Z9O08YRN8O (Tetrabenazine) SB - IM EIN - BMC Neurol. 2011;11:18 MH - Adrenergic Uptake Inhibitors/*therapeutic use MH - Adult MH - Aged MH - Disability Evaluation MH - Double-Blind Method MH - Drug Evaluation/methods MH - Follow-Up Studies MH - Humans MH - Huntington Disease/*drug therapy MH - Middle Aged MH - Severity of Illness Index MH - Tetrabenazine/*therapeutic use MH - Time Factors MH - Treatment Outcome PMC - PMC2804668 OID - NLM: PMC2804668

EDATMHDACRDTPHSTPHSTPHSTAID AID PST SO PMIDOWN STATDA DCOMIS IS VI IP DP TI the

2009/12/22 06:00 2010/02/25 06:00 2009/12/22 06:00 2009/03/20 [received] 2009/12/18 [accepted] 2009/12/18 [aheadofprint] 1471-2377-9-62 [pii] 10.1186/1471-2377-9-62 [doi] epublish BMC Neurol. 2009 Dec 18;9:62. doi: 10.1186/1471-2377-9-62. 20007722 NLM MEDLINE 20100813 20101130 1936-959X (Electronic) 0195-6108 (Linking) 31 7 2010 Aug Transient hemiglossal denervation during acute internal capsule infarct in

setting of dysarthria-clumsy hand syndrome. PG - 1266-7 LID - 10.3174/ajnr.A1874 [doi] AB - A case of MR imaging-documented transient unilateral tongue denervation presenting during acute internal capsule infarction is described. Understa nding the corticolingual pathway innervation of the hypoglossal nucleus is essen tial for explaining these findings. Awareness of the findings in this case will facilitate appropriate diagnosis, provide neuroanatomic explanation, and p revent misdiagnosis. FAU - Titelbaum, David S AU - Titelbaum DS AD - Department of Radiology, Shields Health Care, Brockton, Massachusetts 0230 1, USA. dtitelbaum@shields.com FAU - Sodha, N B AU - Sodha NB FAU - Moonis, M AU - Moonis M LA - eng PT - Case Reports PT - Journal Article DEP - 20091210 PL - United States TA - AJNR Am J Neuroradiol JT - AJNR. American journal of neuroradiology JID - 8003708 SB - IM MH - Acute Disease MH - Aged MH - Cerebral Infarction/*complications/pathology MH - Dysarthria/*etiology/pathology MH - Efferent Pathways/pathology MH - Hand/innervation MH - Humans

MH - Hypoglossal Nerve Diseases/*etiology/pathology MH - Internal Capsule/*pathology MH - Magnetic Resonance Imaging MH - Male MH - Movement Disorders/*etiology/pathology MH - Tongue/*innervation MH - Transcranial Magnetic Stimulation EDAT- 2009/12/17 06:00 MHDA- 2010/12/14 06:00 CRDT- 2009/12/17 06:00 PHST- 2009/12/10 [aheadofprint] AID - ajnr.A1874 [pii] AID - 10.3174/ajnr.A1874 [doi] PST - ppublish SO - AJNR Am J Neuroradiol. 2010 Aug;31(7):1266-7. doi: 10.3174/ajnr.A1874. Epu b 2009 Dec 10. PMID- 19948755 OWN - NLM STAT- MEDLINE DA - 20100212 DCOM- 20100421 LR - 20130826 IS - 1558-9102 (Electronic) IS - 1092-4388 (Linking) VI - 53 IP - 1 DP - 2010 Feb TI - Formant centralization ratio: a proposal for a new acoustic measure of dys arthric speech. PG - 114-25 LID - 10.1044/1092-4388(2009/08-0184) [doi] AB - PURPOSE: The vowel space area (VSA) has been used as an acoustic metric of dysarthric speech, but with varying degrees of success. In this study, the authors aimed to test an alternative metric to the VSA-the formant central ization ratio (FCR), which is hypothesized to more effectively differentiate dysar thric from healthy speech and register treatment effects. METHOD: Speech recordi ngs of 38 individuals with idiopathic Parkinson's disease and dysarthria (19 of w hom received 1 month of intensive speech therapy [Lee Silverman Voice Treatmen t; LSVT LOUD]) and 14 healthy control participants were acoustically analyzed. Vow els were extracted from short phrases. The same vowel-formant elements were us ed to construct the FCR, expressed as (F2u + F2a + F1i + F1u) / (F2i + F1a), the VSA, expressed as ABS([F1i x (F2a - F2u) + F1a x (F2u - F2i) + F1u x (F2i - F2a )] / 2), a logarithmically scaled version of the VSA (LnVSA), and the F2i /F2u ratio. RESULTS: Unlike the VSA and the LnVSA, the FCR and F2i/F2u ratio robustly differentiated dysarthric from healthy speech and were not gender sensitiv e. All metrics effectively registered treatment effects and were strongly correla

ted with each other. CONCLUSION: Albeit preliminary, the present findings indi cate that the FCR is a sensitive, valid, and reliable acoustic metric for distinguishing dysarthric from unimpaired speech and for monitoring treatm ent effects, probably because of reduced sensitivity to interspeaker variabili ty and enhanced sensitivity to vowel centralization. - Sapir, Shimon - Sapir S - Department of Communication Sciences and Disorders, Faculty of Social Welf and Health Sciences, University of Haifa, Haifa, Israel. sapir@research.haifa. ac.il FAU - Ramig, Lorraine O AU - Ramig LO FAU - Spielman, Jennifer L AU - Spielman JL FAU - Fox, Cynthia AU - Fox C LA - eng GR - R01 DC001150-17/DC/NIDCD NIH HHS/United States GR - R01 DC1150/DC/NIDCD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20091130 PL - United States TA - J Speech Lang Hear Res JT - Journal of speech, language, and hearing research : JSLHR JID - 9705610 SB - IM MH - Aged MH - Dysarthria/*diagnosis/therapy MH - Female MH - Humans MH - Male MH - Parkinson Disease/diagnosis/therapy MH - Phonetics MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Sex Factors MH - *Speech MH - *Speech Acoustics MH - Speech Production Measurement/*methods MH - Speech Therapy MH - Time Factors MH - Treatment Outcome PMC - PMC2821466 MID - NIHMS136610 OID - NLM: NIHMS136610 OID - NLM: PMC2821466 EDAT- 2009/12/02 06:00 MHDA- 2010/04/22 06:00 CRDT- 2009/12/02 06:00 PHST- 2009/11/30 [aheadofprint] AID - 1092-4388_2009_08-0184 [pii] AID - 10.1044/1092-4388(2009/08-0184) [doi] PST - ppublish SO - J Speech Lang Hear Res. 2010 Feb;53(1):114-25. doi: FAU AU AD are

10.1044/1092-4388(2009/08-0184). Epub 2009 Nov 30. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 19908293 NLM MEDLINE 20100115 20100217 20131121 1545-5017 (Electronic) 1545-5009 (Linking) 54 3 2010 Mar Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside. PG - 416-23 LID - 10.1002/pbc.22326 [doi] AB - BACKGROUND: Central nervous system (CNS) complications of Langerhans cell histiocytosis (LCH) include mass lesions and a neurodegenerative (ND) synd rome with ataxia, dysarthria, dysmetria, learning and behavior difficulties and /or characteristic changes on brain MRIs. Hydrocephalus has rarely been report ed in LCH. LCH lesions of the orbit, mastoid and temporal bones ("CNS-Risk" lesi ons) and diabetes insipidus predispose patients to ND-CNS-LCH. Treatment option s have been limited and only a case series using trans-retinoic acid (ATRA) and intravenous immunoglobulin (IVIG) have been published. METHODS: We have us ed cytosine arabinoside (ARA-C) with or without vincristine to treat eight pa tients with ND-CNS LCH. Patients: Seven male children and one young adult male wi th clinical and radiologic ND-CNS-LCH were treated with a regimen of vincrist ine 1.5 mg/m(2) on day 1 and ARA-C 100 mg/m(2) daily for 5 days or ARA-C alone mon thly for 4-19 months. Seven patients were evaluated with an ataxia rating scale (ARS) and all with serial MRIs of the brain. RESULTS: Five of seven patients had decreases in their ARS scores and/or decreased T2 hyperintense lesions on MRI images. Grade 2 neutropenia was the most frequent adverse event. Vincristine-associated neuropathy occurred in two patients. Hydrocephalus caused symptoms and signs that confounded the diagnosis and management of ND-CNSLCH in all four patients affected with both. CONCLUSIONS: Subtle changes in neuro logic function may be complicated by hydrocephalus. Vcr/ARA-C or ARA-C were an effective therapies for some ND-CNS LCH patients. A clinical trial using t his and possibly other modalities such as IVIG or ATRA should be done. CI - Copyright 2009 Wiley-Liss, Inc. FAU - Allen, Carl E AU - Allen CE AD - Texas Children's Cancer Center/Hematology Service, Houston, TX 77030, USA. FAU - Flores, Ricardo

AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU FAU AU LA GR GR GR PT PL TA JT JID RN RN SB CIN MH MH MH MH MH MH MH MH MH MH MH MH MH PMC MID OID OID EDATMHDACRDTAID PST SO PMIDOWN STATDA -

Flores R Rauch, Ronald Rauch R Dauser, Robert Dauser R Murray, Jeffrey C Murray JC Puccetti, Diane Puccetti D Hsu, David A Hsu DA Sondel, Paul Sondel P Hetherington, Maxine Hetherington M Goldman, Stan Goldman S McClain, Kenneth L McClain KL eng L40 AI076739/AI/NIAID NIH HHS/United States L40 AI076739-03/AI/NIAID NIH HHS/United States L40 AI076739-04A1/AI/NIAID NIH HHS/United States Journal Article United States Pediatr Blood Cancer Pediatric blood & cancer 101186624 04079A1RDZ (Cytarabine) 57-22-7 (Vincristine) IM Pediatr Blood Cancer. 2010 Jul 15;55(1):215-6. PMID: 20310001 Central Nervous System Diseases/complications/*drug therapy Child Child, Preschool Cytarabine/*therapeutic use Histiocytosis, Langerhans-Cell/complications/*drug therapy Humans Hydrocephalus/complications/*drug therapy Infant Magnetic Resonance Imaging Male Neurodegenerative Diseases/complications/*drug therapy Retrospective Studies Vincristine/*therapeutic use PMC3444163 NIHMS310852 NLM: NIHMS310852 NLM: PMC3444163 2009/11/13 06:00 2010/02/18 06:00 2009/11/13 06:00 10.1002/pbc.22326 [doi] ppublish Pediatr Blood Cancer. 2010 Mar;54(3):416-23. doi: 10.1002/pbc.22326. 19830227 NLM PubMed-not-MEDLINE 20091015

DCOMLR IS IS VI DP TI -

20110714 20120516 1752-1947 (Electronic) 1752-1947 (Linking) 3 2009 Distal migration of a floating carotid thrombus in a patient using oral contraceptives: a case report. PG - 8389 LID - 10.4076/1752-1947-3-8389 [doi] AB - INTRODUCTION: We report the case of a patient with distal migration of a f loating carotid thrombus caused by oral contraceptives. CASE PRESENTATION: A 48-ye ar-old woman using oral contraceptives suffered from dysarthria and gait disturba nce. Examinations, including ultrasound and cerebral arteriogram, revealed a fl oating thrombus at the left carotid bifurcation with no stenosis. Despite antithr ombotic therapy, the floating carotid thrombus migrated to the ipsilateral middle cerebral artery, resulting in a severe stroke. CONCLUSION: Some floating t hrombi are resistant to conservative therapy and have a risk of distal migration, which may cause a major stroke in the acute stage. FAU - Watanabe, Masaki AU - Watanabe M FAU - Mori, Takahisa AU - Mori T FAU - Imai, Keisuke AU - Imai K FAU - Izumoto, Hajime AU - Izumoto H FAU - Hirano, Teruyuki AU - Hirano T FAU - Uchino, Makoto AU - Uchino M LA - eng PT - Journal Article DEP - 20090714 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 PMC - PMC2737801 OID - NLM: PMC2737801 EDAT- 2009/10/16 06:00 MHDA- 2009/10/16 06:01 CRDT- 2009/10/16 06:00 PHST- 2008/06/20 [received] PHST- 2009/01/22 [accepted] PHST- 2009/07/14 [epublish] AID - 10.4076/1752-1947-3-8389 [doi] PST - epublish SO - J Med Case Rep. 2009 Jul 14;3:8389. doi: 10.4076/1752-1947-3-8389. PMID- 19829680 OWN - NLM STAT- MEDLINE

DA DCOMIS IS VI IP DP TI tem

20091015 20110330 1671-167X (Print) 1671-167X (Linking) 41 5 2009 Oct 18 [Tumor-like inflammatory demyelinating diseases of the central nervous sys

with relapse onset: a case report and review]. PG - 588-9 AB - Here we report the clinical, radiological and neuropathological findings o f a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-month history of inflammatory pseudotumor and no other significant medical history, who pre sented to our hospital recurrent relapse numbness and weakness of his right extre mities, dysarthria and memory deterioration. Brain magnetic resonance imaging(MRI) showed mass focal lesion in white matter of left parietal lobes. The biopsy showe d numerous infiltrating macrophages and lymphocytes within the perivascular. The patient responded clinically to corticosteroid and intravenous immunoglobulin(IVIG) therapy. According to the results of the biopsy and t he MRI , a diagnosis of inflammatory pseudotumor of the central nervous system wa s made. The vascular dysfunction may act in the pathogenesis of inflammatory pseud otumor of the central nervous system. FAU - Qi, Xue Liang AU - Qi XL AD - Department of Neurology, Peking University First Hospital, Beijing 100034, China. FAU - Chen, Jing AU - Chen J FAU - Zheng, Ri Liang AU - Zheng RL FAU - Li, Ying AU - Li Y FAU - Huang, Yi Ning AU - Huang YN FAU - Yuan, Yun AU - Yuan Y LA - chi PT - Case Reports PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Central Nervous System Diseases/*pathology MH - Demyelinating Diseases/diagnosis/*pathology

MH MH MH MH MH MH EDATMHDACRDTPST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI PG LID FAU AU AD FAU AU FAU AU LA PT PT PL TA JT JID RN RN SB SB MH MH MH MH MH MH MH MH MH MH MH MH PMC OID EDATMHDA-

Granuloma, Plasma Cell/diagnosis/*pathology Humans Magnetic Resonance Imaging Male Middle Aged Recurrence 2009/10/16 06:00 2011/03/31 06:00 2009/10/16 06:00 ppublish Beijing Da Xue Xue Bao. 2009 Oct 18;41(5):588-9. 19797780 NLM MEDLINE 20091002 20091022 20131213 1942-5546 (Electronic) 0025-6196 (Linking) 84 10 2009 Oct 42-year-old male methamphetamine user with dysarthria and facial droop. 912-5 10.1016/S0025-6196(11)60508-9 [doi] Obioha, Collins Chiedozie Obioha CC Department of Neurology, Mayo Clinic Hospital, Phoenix, AZ 85054, USA. Engel, Rodney A Engel RA Ingall, Timothy Ingall T eng Case Reports Journal Article United States Mayo Clin Proc Mayo Clinic proceedings 0405543 0 (Central Nervous System Stimulants) 44RAL3456C (Methamphetamine) AIM IM Adult Amphetamine-Related Disorders/*complications Aneurysm, Dissecting/*diagnosis/etiology/therapy Aortic Aneurysm/*diagnosis/etiology/therapy Brain Ischemia/diagnosis/etiology/therapy Central Nervous System Stimulants/adverse effects Dysarthria/*etiology Facial Paralysis/*etiology Humans Male Methamphetamine/adverse effects Stroke/*diagnosis/etiology/therapy PMC2755810 NLM: PMC2755810 2009/10/03 06:00 2009/10/23 06:00

CRDTAID AID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI t. PG LID AB -

2009/10/03 06:00 S0025-6196(11)60508-9 [pii] 10.1016/S0025-6196(11)60508-9 [doi] ppublish Mayo Clin Proc. 2009 Oct;84(10):912-5. doi: 10.1016/S0025-6196(11)60508-9. 19771294 NLM MEDLINE 20090922 20100107 20130827 1938-162X (Electronic) 1062-6050 (Linking) 44 5 2009 Sep-Oct Unilateral hypoglossal nerve injury in a collegiate wrestler: a case repor

534-7 10.4085/1062-6050-44.5.534 [doi] OBJECTIVE: To introduce the case of a collegiate wrestler who suffered a traumatic unilateral hypoglossal nerve injury. This case presents the oppo rtunity to discuss the diagnosis and treatment of a 20-year-old man with an injury to his right hypoglossal nerve. BACKGROUND: Injuries to the hypoglossal nerve (cr anial nerve XII) are rare. Most reported cases are the result of malignancy, wit h traumatic causes less common. In this case, a collegiate wrestler struck h is head on the wrestling mat during practice. No loss of consciousness occurred. T he wrestler initially demonstrated signs and symptoms of a mild concussion, w ith dizziness and a headache. These concussion symptoms cleared quickly, but t he athlete complained of difficulty swallowing (dysphagia) and demonstrated s lurred speech (dysarthria). Also, his tongue deviated toward the right. No other neurologic deficits were observed. DIFFERENTIAL DIAGNOSIS: Occipital-cervi cal junction fracture, syringomyelia, malignancy, iatrogenic causes, cranial n erve injury. TREATMENT: After initial injury recognition, the athletic trainer placed the patient in a cervical collar and transported him to the emergency depa rtment. The patient received prednisone, and the emergency medicine physician orde red cervical spine plain radiographs, brain computed tomography, and brain and internal auditory canal magnetic resonance imaging. The physician consulte d a neurologist, who managed the patient conservatively, with rest and no cont act activity. The neurologist allowed the patient to participate in wrestling 7 months after injury. UNIQUENESS: To our knowledge, no other reports of uni lateral

hypoglossal nerve injury from relatively low-energy trauma (including athl etics) exist. CONCLUSIONS: Hypoglossal nerve injury should be considered in indiv iduals with head injury who experience dysphagia and dysarthria. Athletes with he ad injuries require cranial nerve assessments. FAU - Loro, William A AU - Loro WA AD - US Army Medical Department Center and School, Fort Sam Houston, TX 78234-6 138, USA. william.loro@us.army.mil FAU - Owens, Brett AU - Owens B LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Athl Train JT - Journal of athletic training JID - 9301647 SB - IM MH - Athletic Injuries/*diagnosis/*rehabilitation MH - Diagnosis, Differential MH - Humans MH - *Hypoglossal Nerve Injuries MH - Male MH - Wrestling/*injuries MH - Young Adult PMC - PMC2742465 OID - NLM: PMC2742465 OTO - NOTNLM OT - dysarthria OT - dysphagia OT - tongue paralysis OT - twelfth cranial nerve EDAT- 2009/09/23 06:00 MHDA- 2010/01/08 06:00 CRDT- 2009/09/23 06:00 AID - 10.4085/1062-6050-44.5.534 [doi] PST - ppublish SO - J Athl Train. 2009 Sep-Oct;44(5):534-7. doi: 10.4085/1062-6050-44.5.534. PMIDOWN STATDA DCOMLR IS IS VI IP DP TI 19721837 NLM MEDLINE 20090901 20091208 20130827 2005-8330 (Electronic) 1229-6929 (Linking) 10 5 2009 Sep-Oct Hypereosinophilia with multiple thromboembolic cerebral infarcts and focal intracerebral hemorrhage. PG - 511-4 LID - 10.3348/kjr.2009.10.5.511 [doi] AB - We report a case of hypereosinophilia causing multiple areas of cerebral infarcts. A 52-year-old Korean man presented with dysarthria and weakness

in both arms. A brain MRI revealed multiple acute infarcts in the distal border zo ne with focal intracerebral hemorrhage, whereas a cerebral angiogram was not remar kable. The eosinophil count was 5,500/microL and was accompanied by elevated card iac enzyme levels. The pattern of cerebral infarcts and laboratory results sug gest a thromboembolic infarction associated with hypereosinophilia. FAU - Lee, Eun Ju AU - Lee EJ AD - Department of Radiology, Hanyang University College of Medicine, Seoul, Ko rea. FAU - Lee, Young-Jun AU - Lee YJ FAU - Lee, Seung Ro AU - Lee SR FAU - Park, Dong Woo AU - Park DW FAU - Kim, Hyun Young AU - Kim HY LA - eng PT - Case Reports PT - Journal Article DEP - 20090825 PL - Korea (South) TA - Korean J Radiol JT - Korean journal of radiology : official journal of the Korean Radiological Society JID - 100956096 SB - IM MH - Cerebral Hemorrhage/diagnosis/*etiology MH - Cerebral Infarction/diagnosis/*etiology MH - Diagnosis, Differential MH - Eosinophilia/*complications/drug therapy MH - Humans MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged PMC - PMC2731870 OID - NLM: PMC2731870 OTO - NOTNLM OT - Hypereosinophilic syndrome OT - Infarction, cerebral OT - Intracerebral hemorrhage OT - Thromboembolism EDAT- 2009/09/02 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/02 09:00 PHST- 2008/11/06 [received] PHST- 2009/02/03 [accepted] PHST- 2009/08/25 [epublish] AID - 10.3348/kjr.2009.10.5.511 [doi] PST - ppublish SO - Korean J Radiol. 2009 Sep-Oct;10(5):511-4. doi: 10.3348/kjr.2009.10.5.511. Epub 2009 Aug 25. PMID- 19654273

OWN STATDA DCOMLR IS IS VI IP DP TI PG AB temic nced

NLM MEDLINE 20090805 20100929 20131121 1945-1997 (Electronic) 0098-6151 (Linking) 109 7 2009 Jul Brain amyloidoma with cerebral hemorrhage. 372-5 Unlike systemic amyloidosis, the diagnosis of brain amyloidoma without sys manifestations is clinically challenging. Despite the availability of adva brain imaging technology, such conditions are difficult to ascertain witho

ut brain biopsy or autopsy. We report the case of a 64-year-old woman who pre sented with frontal lobe syndrome with abnormal linear enhancement on brain magne tic resonance imaging. Results from a stereotactic biopsy revealed lambda-posi tive protein deposition in the brain parenchyma. During the course of illness, the patient had an acute cerebral hemorrhage, which manifested with hemiparesi s, dysarthria, and pathologic crying. Review of the literature revealed 15 ca ses of primary brain amyloidoma. Patients had similar protein deposits but in dif ferent regions of the brain and therefore presented with various neurologic sympt oms. FAU - Labro, Henrick AU - Labro H AD - Department of Medicine and Neurological Institute at St John West Shore Ho spital, University Hospitals Case Medical Center in Cleveland, Ohio 44145, USA. henrick.labro@csauh.com FAU - Al-Kadhimi, Zaid AU - Al-Kadhimi Z FAU - Djmil, Mounir AU - Djmil M FAU - Oghlakian, Roger AU - Oghlakian R FAU - Alshekhlee, Amer AU - Alshekhlee A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Am Osteopath Assoc JT - The Journal of the American Osteopathic Association JID - 7503065 RN - 0 (Anticonvulsants) RN - 0 (Antineoplastic Agents, Alkylating) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 4Z8R6ORS6L (Thalidomide)

RN RN RN SB MH MH MH MH MH MH MH MH MH MH MH MH MH MH MH EDATMHDACRDTAID PST SO PMIDOWN STATDA DCOMLR IS IS VI IP DP TI on of PG LID FAU AU FAU AU FAU AU LA PT PT TT teter DEP PL TA JT JID RN -

614OI1Z5WI (Valproic Acid) 7S5I7G3JQL (Dexamethasone) Q41OR9510P (Melphalan) IM Amyloidosis/*diagnosis/pathology Anticonvulsants/therapeutic use Antineoplastic Agents, Alkylating/therapeutic use Brain Neoplasms/complications/*diagnosis/drug therapy/pathology Cerebral Hemorrhage/*diagnosis/drug therapy/etiology Dexamethasone/therapeutic use Female Glucocorticoids/therapeutic use Humans Immunosuppressive Agents/therapeutic use Magnetic Resonance Imaging Melphalan/therapeutic use Middle Aged Thalidomide/therapeutic use Valproic Acid/therapeutic use 2009/08/06 09:00 2010/09/30 06:00 2009/08/06 09:00 109/7/372 [pii] ppublish J Am Osteopath Assoc. 2009 Jul;109(7):372-5. 19403227 NLM MEDLINE 20090805 20091104 20131121 1579-2129 (Electronic) 0300-2896 (Linking) 45 9 2009 Sep [Acute transient ataxia caused by local lidocaine injection during inserti a pleural catheter]. 472-3 10.1016/j.arbres.2009.01.008 [doi] Porcel, Jose Manuel Porcel JM Brieva, Luis Brieva L Antoni Schoenenberger, Joan Antoni Schoenenberger J spa Case Reports Letter Ataxia aguda transitoria por lidocaina local durante la insercion de un ca pleural. 20090428 Spain Arch Bronconeumol Archivos de bronconeumologia 0354720 0 (Anesthetics, Local)

RN - 98PI200987 (Lidocaine) RN - B6E06QE59J (Mepivacaine) SB - IM MH - Acute Disease MH - Anesthetics, Local/administration & dosage/*adverse effects/pharmacokineti cs MH - Catheterization MH - Cerebellar Ataxia/*chemically induced MH - Community-Acquired Infections/complications MH - Diabetes Mellitus, Type 1/complications MH - Diplopia/*chemically induced MH - *Drainage/instrumentation MH - Dysarthria/*chemically induced MH - Humans MH - Injections, Subcutaneous MH - Lidocaine/administration & dosage/*adverse effects/pharmacokinetics MH - Male MH - Mepivacaine/administration & dosage MH - Middle Aged MH - Pleural Effusion/*surgery MH - Pneumonia/complications MH - Thorax EDAT- 2009/05/01 09:00 MHDA- 2009/11/05 06:00 CRDT- 2009/05/01 09:00 PHST- 2009/01/10 [received] PHST- 2009/01/19 [revised] PHST- 2009/01/25 [accepted] PHST- 2009/04/28 [aheadofprint] AID - S0300-2896(09)00157-4 [pii] AID - 10.1016/j.arbres.2009.01.008 [doi] PST - ppublish SO - Arch Bronconeumol. 2009 Sep;45(9):472-3. doi: 10.1016/j.arbres.2009.01.008 . Epub 2009 Apr 28. PMID- 19363575 OWN - NLM STAT- MEDLINE DA - 20090413 DCOM- 20090415 IS - 1651-2081 (Electronic) IS - 1650-1977 (Linking) VI - 41 IP - 5 DP - 2009 Apr TI - Communicating using the eyes without remembering it: cognitive rehabilitat ion in a severely brain-injured patient with amnesia, tetraplegia and anarthria. PG - 393-6 LID - 10.2340/16501977-0344 [doi] AB - We describe here a case of cognitive rehabilitation in a young patient wit h closed head injury, who had dense anterograde amnesia and such disabling neurological defects (tetraplegia and anarthria) that the condition evoked some features of an incomplete locked-in syndrome. After a prolonged period of no communicative possibility, the patient underwent a specific training, base d on

principles of errorless learning, with the aim of using a computerized eye-tracker system. Although, due to memory disturbances, the patient alwa ys denied ever having used the eye-tracker system, learned to use the compute rized device and improved interaction with the environment. This favourable outc ome may serve as a stimulus for devising new training approaches in patients with complex patterns of cognitive impairments, even when associated with severe motor impairments. FAU - Trojano, Luigi AU - Trojano L AD - Department of Psychology, Second University of Naples, Via Vivaldi 43, IT81100 Caserta, Italy. luigi.trojano@unina2.it FAU - Moretta, Pasquale AU - Moretta P FAU - Estraneo, Anna AU - Estraneo A LA - eng PT - Case Reports PT - Journal Article PL - Sweden TA - J Rehabil Med JT - Journal of rehabilitation medicine : official journal of the UEMS European Board of Physical and Rehabilitation Medicine JID - 101088169 SB - IM MH - Accidents, Traffic MH - Adult MH - Amnesia/etiology/psychology/*rehabilitation MH - Brain Injuries/complications/psychology/*rehabilitation MH - Cognition Disorders/etiology/psychology/*rehabilitation MH - Dysarthria/etiology/psychology/*rehabilitation MH - *Eye Movements MH - Head Injuries, Closed/complications/psychology/*rehabilitation MH - Humans MH - Learning MH - Male MH - *Nonverbal Communication/psychology MH - Quadriplegia/psychology/*rehabilitation MH - Treatment Outcome MH - User-Computer Interface EDAT- 2009/04/14 09:00 MHDA- 2009/04/16 09:00 CRDT- 2009/04/14 09:00 AID - 10.2340/16501977-0344 [doi] PST - ppublish SO - J Rehabil Med. 2009 Apr;41(5):393-6. doi: 10.2340/16501977-0344. PMIDOWN STATDA DCOMLR IS IS 19326416 NLM MEDLINE 20090514 20090602 20131121 1545-5017 (Electronic) 1545-5009 (Linking)

VI - 53 IP - 1 DP - 2009 Jul TI - False-photosensitivity and transient hemiparesis following high-dose intra venous and intrathecal methotrexate for treatment of acute lymphoblastic leukemia . PG - 103-5 LID - 10.1002/pbc.21896 [doi] AB - We describe a patient who was treated with high-dose intravenous and intra thecal methotrexate for acute lymphoblastic leukemia, and who manifested a false photosensitivity reaction with no prior evidence of sun exposure. This pat ient later experienced delayed transient hemiparesis following methotrexate administration, although without long-term sequelae. The etiology of these events is obscure, but suggestive of a vasculitic or immune-mediated reaction to methotrexate. CI - Copyright 2009 Wiley-Liss, Inc. FAU - Shah, Nilay AU - Shah N AD - Division of Pediatric Oncology, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland, USA. FAU - Zambidis, Elias T AU - Zambidis ET LA - eng GR - K08 HL077595/HL/NHLBI NIH HHS/United States GR - K08 HL077595-01/HL/NHLBI NIH HHS/United States GR - K08 HL077595-02/HL/NHLBI NIH HHS/United States GR - K08 HL077595-03/HL/NHLBI NIH HHS/United States GR - K08 HL077595-04/HL/NHLBI NIH HHS/United States GR - K08 HL077595-05/HL/NHLBI NIH HHS/United States PT - Case Reports PT - Journal Article PL - United States TA - Pediatr Blood Cancer JT - Pediatric blood & cancer JID - 101186624 RN - 0 (Nucleic Acid Synthesis Inhibitors) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adolescent MH - Dysarthria/chemically induced MH - False Positive Reactions MH - Humans MH - Injections, Intravenous MH - Injections, Spinal MH - Male MH - Methotrexate/*administration & dosage/*adverse effects MH - Nucleic Acid Synthesis Inhibitors/administration & dosage/adverse effects MH - Paresis/*chemically induced MH - Photosensitivity Disorders/*chemically induced MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*drug therapy MH - Recurrence PMC - PMC3073488 MID - NIHMS129680 OID - NLM: NIHMS129680 OID - NLM: PMC3073488

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2009/03/28 09:00 2009/06/03 09:00 2009/03/28 09:00 10.1002/pbc.21896 [doi] ppublish Pediatr Blood Cancer. 2009 Jul;53(1):103-5. doi: 10.1002/pbc.21896.

PMID- 19156018 OWN - NLM STAT- MEDLINE DA - 20090703 DCOM- 20100323 IS - 1973-9095 (Electronic) IS - 1973-9087 (Linking) VI - 45 IP - 2 DP - 2009 Jun TI - A Cochrane review of treatment for dysarthria following acquired brain inj ury in children and adolescents. PG - 197-204 AB - BACKGROUND: The expression ''acquired brain injury'' (ABI) incorporates a range of etiologies including cerebrovascular accident, brain tumour and traumat ic brain injury. ABI is a common cause of disability in the pediatric populat ion, and dysarthria is a common and often persistent sequelae associated with A BI in children. OBJECTIVES: The aim of this study was to assess the efficacy of intervention delivered by Speech and Language Pathologists/Therapists targ eting dysarthric speech in children resulting from acquired brain injury. METHOD S: Several electronic databases were searched up to January 2007. The review considered randomised controlled trials (RCTs) and quasi-randomised studie s of children aged 3 to 16 years with acquired dysarthria grouped by aetiology (e.g., brain tumour, traumatic brain injury, cerebrovascular accident). Both auth ors independently assessed the titles and abstracts for relevance (100% interrater reliability) and the full text version of all potentially relevant article s was obtained. No studies met inclusion criteria. RESULTS: Of 2091 titles and abstracts identified, full text versions of only three were obtained. The remaining 2 088 were excluded, largely on the basis of not including dysar thria, being diagnostic or descriptive papers, and for concerning adults rather t han children. All obtained articles were excluded due to including populations without ABI, adults with dysarthria, or inappropriate design. Thus, no stu dies met inclusion criteria. CONCLUSIONS: The review demonstrates a critical la ck of studies, let alone RCTs, addressing treatment efficacy for dysarthria in c hildren with ABI. Possible reasons to explain this lack of data include 1) a lack of

understanding of the characteristics or natural history of dysarthria asso ciated with this population; 2) the lack of a diagnostic classification system fo r children precluding the development of well targeted intervention programs ; and 3) the heterogeneity of both the etiologies and resultant possible dysarth ria types of pediatric ABI. Efforts should first be directed at modest well-controlled studies to identify likely efficacious treatments that may then be trialled in multicentre collaborations using quasi-randomised or RCT methodology. FAU - Morgan, A T AU - Morgan AT AD - Healthy Development [Theme], Language and Literacy, Murdoch Children Resea rch Institute, Melbourne, Australia. angela.morgan@mcri.edu.au FAU - Vogel, A P AU - Vogel AP LA - eng PT - Journal Article PT - Review DEP - 20090121 PL - Italy TA - Eur J Phys Rehabil Med JT - European journal of physical and rehabilitation medicine JID - 101465662 SB - IM MH - Adolescent MH - Brain Injuries/*complications/*rehabilitation MH - Child MH - Child, Preschool MH - Dysarthria/*etiology/*rehabilitation MH - Evidence-Based Medicine MH - Humans MH - Randomized Controlled Trials as Topic MH - Speech Therapy/*methods RF - 39 EDAT- 2009/01/22 09:00 MHDA- 2010/03/24 06:00 CRDT- 2009/01/22 09:00 PHST- 2009/01/21 [aheadofprint] AID - R33092061 [pii] PST - ppublish SO - Eur J Phys Rehabil Med. 2009 Jun;45(2):197-204. Epub 2009 Jan 21.