1.

Myasthenia gravis
(1) 主述：ptosis, double vision and limbs weakness aggravated in the late afternoon but better after rest in recent 2 months
(2) Aminoglycoside antibiotics (-mycin) will most probably exacerbate the symptoms
(3) 診斷依據：Clinical history、pyridostigmine(tensilon) test、Repetitive nerve stimulation(RST)、Chest CT(thymoma)
(4) 分類：
1 Group I：ocular muscle only
2 Group IIA：mild generalized weakness
3 Group IIB：moderate generalized weakness 且有輕微呼吸衰竭
4 Group III：acute fulminating-可能是急性發作
5 Group IV：severe upon mild or moderate at onset
2. Myasthenic Syndrome，Lamvert-Eaton Syndrome，LEMS
(1) Weakness：proximal > Distal；legs > arms，多從 legs 開始，少在眼睛
(2) Clinical symptoms：Ataxia、areflexia、autonomic dysfunction、Proximal muscle weakness
(3) Impairement of the presynaptic function：同 Botulism、Hemicholinium、Calcium blocking agent
3. Brain abscess
(1) 細菌(staphylococcus) is the most common pathogen
(2) 免疫缺乏的病人：弓漿蟲、Cryptococcus、TB、Aerobic G(-) bacteria→Virus encephalitis 不會造成 Brain abscess
(3) 可能的症狀：頭痛、發燒、噁心嘔吐、局部神經學症狀、視乳突水腫、脖子僵硬、意識改變、痙攣
(4) 從哪裡跑過來？
1 Otitis media、Mastoiditis、Dental infection、Frontoethmoidal and sphenoidal sinusitis、Head wound or neurosurgery
2 遠方：Bacterial endocarditis、Congenital heart disease、Lung abscess, empyema, bronchiectases
(5) 治療：能引流就引流(drain)，輔助以抗生素
4. 頭痛
(1) 原發性頭痛與次發性頭痛最大的差別：次發性頭痛是有明確病因(Underline)，病人常常描述是第一次出現這種痛法
(2) Migraine：
1 年輕女性多，重複發作，月經不規則與頭痛相關，喝酒和運動(physical activities)會惡化 Migraine
(3) Tension type headache
1 喝酒和運動可能減輕疼痛，壓力會誘發疼痛
(4) Cluster headache
1 男生多，眼眶周圍、痛哭流涕(lacrimation, rhinorrhea)、夜晚痛醒、運動也會惡化
(5) SAH
1 Sudden onset、explosive and thumderclap-like headache、located at the occipital area (Pcom aneurysm rupture)
5. Pain
(1) Entrapment syndrome
1 正中神經壓迫(carpal tunnel syndrome)
2 尺神經壓迫(tardive ulnar palsy)、橈神經壓迫
3 腳踝有 tarsal tunnel syndrome：Posterior tibial neuralgia
(2) Central mechanism：midbrain 的 PAG matter 和脊髓的 Dorsal horn
(3) 痛覺傳遞物質：週邊 Bradykinin、Prostagladin；中樞 Substance P(central sensitization、central pain、慢性疼痛有關)
(4) Neuropathic pain：Tingling、Burning、Numbness、蟻爬感
1 PNS：Diabetic polyneuropathy、Herpes zoster(Dorsal root ganglia)
2 CNS：Thalamic pain
6. Movement disorder
 (1) Chorea：
1 flow of movement from one body part to another (more affecting distal part)
2 Cannot be predicted：irregularly timed, non-repetitive, randomly distributed, and abrupt in nature.
(2) Dystonia：
1 Family history (+)
2 sustained muscle contractions with abnormal twisting and tremor of body parts
3 sensory and motor tricks：眼肌痙攣症(blepharospasm)、嘴巴亂動(Orolinguomandibular dyskinesia)、斜頸症
(Cervical dystonia)、寫字痙攣症(Writer’s cramp)、聲音講不出來(spasmodis dysphonia)。
4 DYT1: leg onset→exon 5 GAG deletion
5 DYT5(Segawa’s disease): dopa-responsive dystonia
6 DYT11: myoclonus-dystonia
7 DYT16: young-onset dystonia-parkinsonism
(3) Parkinsonism
1 SCNA mutation：PARK4 family，gain of function、Lewy body in the substantia nigra、More rapid
progression 、Higher prevalence of dementia, psychiatric and autonomic disturbance
2 LRRK2 mutation：PARK8 family，similar to typical late-onset PD、onset at 58 y/o, asymmetrical onset、L-DOPA
responsive、no dementia or autonomic dysfunction
3 Parkin mutation：PARK2 family，Autosomal recessive、relative benign course
7. Peripheral neuropathy and myopathy
(1) Denervated muscle 會出現 Muscle grouping、angulation、target formation
(2) DMD：最常見也是最嚴重的 muscular dystrophy；缺乏 dystrophin；遺傳模式為 XLR；Highest CK level(上萬)
(3) Emery-Dreifuss muscular dystrophy：Emerin gene defect、rigid spine(抬頭挺胸)、elbow contracture、Heart disease(Conduction
block)
(4) Myotonic dystrophy：AD(CTG repeat)、anitcipation、delayed open hand、Percussion myotonia (thenar m. and tongue)
(5) Facioscapulohumeral muscular dystrophy：Facial palsy、Scapular wining、Popeye’s arm(proximal part atrophy)→凸肚子站姿
(6) Ocularpharyngeal muscular dystrophy：占星者之姿，有明顯的抬頭紋(ptosis and dysphagia)
(7) 導致智商受損的 Muscular dystrophy：DMD、Myotonic dystrophy、Congenital muscular dystrophy(Fukuyama or non-
fukuyama)→智商仍 OK 的只剩下 Becker Muscular Dystrophy
(8) Central core myopathy：
1 RYR1 gene mutation(Chr 19q13.1)→Ca2+ over-release→Malignant hyperthermia
2 Low oxidative enzyme activities
(9) Prognosis in nerve regeneration：Neruapraxia > Axonotmesis > Neurotmesis (worst)
(10) Immune-mediated neuropathy：Guillain-Barre syndrome
8. Spinal cord disease：
(1) Brown-Sequard syndrome：同側運動及本體感覺(震動)喪失、對側痛溫覺喪失
(2) Syringomyelia：Sacral sparing、Sensory dissociation、Paraplegia、Quadriplegia
(3) Dermatome：Nipple(T4)、Umbilicus(T10)、Inguinal canal(T12)、Knee(L4)
9. Encephalopathy：
(1) Wernicke’s encephalopathy：最重要為 Ophthalmoplegia、Thiamine treatment、Mortality rate is 17%
(2) Uremic encephalopathy：
1 Clinical features：Twitch-convulsive syndrome
2 Conscious disturbance
3 EEG shows triphasic waves
10. Seizure and Epilepsy：
(1) Epileptic phenomenon：
1 Motor symptoms：肌肉無力
2 Sensory symptoms：觸痛覺或特殊感覺(味聽視覺)異常
3 Autonomic symptoms：goose skin、palpitation、sweating、flushing
4 Psychic symptoms：Panic and running(恐懼)、Laughing and talking(愉悅)
(2) 會誘發 seizure 的藥物：Antidepressants、antipsychotics、analgesics(cocaine)、Local anesthetic、General
anesthetic 、antibiotics(penicillin、ampicillin、isoniazid)、antineoplastics、bronchodilators、sympathomimetics
(3) 會誘發 seizure 的抗癲癇藥物：Carbamazepine、Phenytoin、Lamotrigine
(4) 不會誘發 seizure 的抗癲癇藥物：Ethosuximide、Valproate
(5) Epileptic focus：Neuronal hyperactivity→Neuronal deformity(Broken dendrite)→Decreased neuron number→Gliosis
(6) Aura→partial seizure→focal cortical lesIon and focal onset→Carbamazepine and phenytoin
(7)
11. Brain tumor
(1) 不會造成 cluster headache(屬於原發性頭痛，沒有明顯的原因下才能診斷)
12. Dementia 失智症
(1) Alzheimer’s disease
1 病理特徵：senile plaques(Most specific) and neurofibrillary tangles scattered throughout the cortex
2 臨床特徵：Amnesia、aphasia、apraxia、agnosia、functional impairement，很少出現幻覺或妄想(DLB 特色)
3 65 歲以上人口中導致痴呆的最常見原因， prevalence rate 約 1.5%，每增加四歲約增加兩倍，80 歲時可以達
到 30%。
4 Late onset AD (sporadic)：apoE4 in chromosome 19
5 最終診斷靠的是屍體解剖(autopsy)才能確立
6 危險因子：年齡、基因、唐氏症(known) > Head trauma、Female、Vascular disease(possible)
7 可能的保護因子：estrogen、NSAID、education
8 台灣常見藥物：Tacrine、Donepenzil、Rivastigmine、Galantamine (沒有 Mematine)
(2) CJD：myoclonus、amnesia、characteristic EEG(periodic discharges in temporal area)、Histology(status spongiosus)
(3) DLB：frequent falls、syncope、visual hallucination、early dementia、Parkinsonism + (rigidity, masked face and slow motion)
(4) FTD：平均 62 歲發病(<AD)、人格改變→出現暴力傾向、出現 Primitive reflex (frontal lobe)、病理切片會看見 Pick’s body
13. What time needs CSF study：
(1) Meningoencephalitis
(2) SAH：Xanthochromia
(3) Meningocarcinomatosis
(4) Demyelinating disease：AIDP 有 albumino-cytological dissociateon
14. Meningitis：
(1) Bacteria：Neutrophil >1000、High protein、Low sugar(CSF/Plasma<0.4)
(2) Virus：Normal sugar
1 HSV：侵犯 temporal lobe 造成失憶(amnesia)、癲癇(periodic sharp wave complexes)
2 JE(日本腦炎)：盛行於夏天(5-10 月)、Myelopathy + Chorea / Ballism (Cortex+thalamus+basal ganglia)、死亡率
可到 1/3
(3) TB：Longer course(few weeks to months)
1 Complication：Obstructive hydrocephalus、Lumbar-sacral arachnoiditis、Occlusive vasculitis(easily stroke)、Thick
exudates that is especially abundant at the brain base、Tuberculoma
 (4) Fungus：Immunocompromised patient(自體免疫、化療病人、AIDS)
1 鴿糞(Cryptococcal)→肺部→血液→CNS
2 臨床表現：severe IICP、Obstructive Hydrocephalus
3 Complication：severe headache, nausea and vomiting、personality change、visual or acoustic impairement、body
weight loss with poor appetite→不會出現腦內出血
(5) Eosinophilic meningitis：
1 致病原：Coccidioidal meningitis、Angiostrongylus cantonensis
2 臨床表徵：Excruciating headache(緊箍咒：frontal→bitemporal→occipital)、Ophthalmoplegia、Blurred
vision 、Facial palsy
15. Multiple sclerosis：
(1) Lhermitte’s sign：An electrical sensation radiating down the spine or into the legs on briskly flexing the neck forward
(2) Uhthoff’s phenomenon：熱和運動會加劇症狀
(3) Internuclear ophthalmoplegia：MLF 受損
1 同時向左側看時→右眼不能往左看 + 左眼有 coarse nystagmus
2 同時往右側看時→左眼不能往右看 + 右眼有 coarse nystagmus
(4) Lab：oligoclonal band in the CSF (IgG index > 1.7)
(5) 15-50yr、女生大於男生兩倍、北歐國家多(北緯 40 度以上，溫帶)
(6) 典型症狀：Intermittent paresthsia and numbness、Monocular blurred vision、ataxic gait
16. 睡眠醫學
(1) VLPO induces sleep；Suprachiamatic nucleus is the master clock (負責清醒)
(2) REM：Ach in LDT (laterodorsal tegmental nucleus) & PPT ( pedunculopontine)
(3) Awake：Monoamine in the brain stem and caudal hypothalamus
(4) 失眠期間定義為大於一個月
(5) Obstructive sleep apnea (OSA)的治療黃金原則為 Nasal CPAP 正壓氧呼吸治療
(6) Restless Leg Syndrome：有不舒服的感覺所以一直動，(夜晚)休息的時候變嚴重，與缺鐵性貧血(IDA)相關
(7) Periodic limb movement disorder：睡著之後只有四肢(腳>手)亂動、小孩(>5times/hr)、大人(>15times/hr)
(8) REM sleep behavior disorder = REM w/o atonia (EMG≠0)：由伴侶給的 History 跟 Polysomnography 來診斷
(9) Narcolepsy：DDx with syncope
1 特色為 Cataplex：Brief episodes of sudden bilateral loss of muscle tone；其他如 Sleep paralysis(鬼壓床)、
Hypnopompic( 睡後) or Hypnagogic(睡前) hallucination
2 Sleep study：Maintence of wakefulness test、Multiple sleep latency test(白天關暗暗<5 分鐘睡著→確診；<8 分鐘
→懷疑)、Polysomnography
(10) Dyssomnia：睡太多或睡太少→Narcolepsy、PLMD、RLS
(11) Parasomnia：合併怪異行為
1 REM：sleep paralysis、Nightmares、RBD
2 Non-REM：Sleep talking/walking/terrors、Nocturnal leg cramps
17. Vertigo
(1) Peripheral vertigo：episodic, monodirectional nystagmus(fast phase 朝正常耳)，可能存在聽力喪失或耳鳴，休息會緩解
1 有哪些疾病？BPPV、Meniere’s disease、Vestibular neuritis
2 Meniere’s diesase：Endolymphatic hydrops→眩暈、單側耳鳴及低頻聽力喪失(triad)→病人抱怨耳朵漲漲的、NE
出現 Rotatory-horizontal nystagmus beating toward the affected ear (can be abolished by visual fixation)→不管幾歲都可
能得到，偏好中老年人。
(2) Central vertigo：persistent, multidirectional nystagmus, with other CN sign(56789,10 都在附近)，嚴重跌倒，藥物效果不好
18. Stroke evaluation
(1) Diagnosis：
1 Onset：acute or sudden
2 Focal neurological deficits
3 Syncope can result from global ischemia、cardiac arrest、sinus arrest→Do not belong to stroke syndrome
4 Isolated symptoms like dizziness、vertigo、tinnitus、facial palsy、incontinence、amnesia is not due to stroke
(2) Most diagnostic for cerebral embolism：Source of emboli
(3) ICA/ophthalmic a. stroke：Monoocular amaurosis fugax (黑矇症)
(4) Major risk factors：age > 65yr、HTN、DM、Heart disease(AF, valvular heart disease)、Previous TIA or stroke
(5) Minor risk factors：dyslipidemia、alcohol、smoking、physical inactivity
19. Conscious disturbance：
(1) Metabolic disorders：delirium→general weakness→asterixis (myoclonus or tremor)
20. ANS dysfunction
(1) Valsalva maneuver：
1 Phase 1: Increased intrathoracic pressure
2 Phase 2: reduce venous return (HR increase)：
a. Early: reduced cardiac output with BP drop (胸內
壓力高使回心血量下降)
b. Late: increased peripheral resistance preventing
further BP drop(交感神經興奮)
3 Phase 3: fall in intrathoracic pressure
4 Phase 4: increased venous return with high peripheral
resistance
(2) 症狀：Orthostatic hypotension、Supine hypertension、Postexercise
hypotension、Postpradial hypotension、Heat-exposure hypotension
(3) MSA：Central type
1 影響 IML 和 Onuf’s nucleus(Onuf’s nucleus 是控制 Ext. anal sphincter 和 Ext. urethal sphincter 的運動神經核，
位於 Spinal cord 的 Anterior horn。)
2 周邊自主神經纖維的 catecholamine 濃度是在正常範圍內
3 給予 NE 後對於血壓提升的效果不大
(4) Pure autonomic failure (PAF)：peripheral type
1 影響的是交感神經節後神經元
2 不會出現 CNS impairment 的症狀，如 Parkinsonism，Pyramidal sign。
3 躺著時候 NE 很低，突然站起來後 NE 也沒有提高，對於 NE 的血壓反應很顯著
4 PAF 的 Prognosis 較 MSA 為佳，Life span 與一般人差不多。
(5) DM autonomic neuropathy：
1 主犯週邊自主神經，如 Vagus n.、Splanchnic n.
2 最重要症狀是 hypoglycemia unawareness→交感失去提升血糖的能力→Hypoglycemic conscious impairement