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LETTERS blocked the active inhibition of the motor through early adulthood with weakness,
cortex with inhibitory low-frequency rTMS. ataxia, dysarthria, spasticity, dystonia, tre-
Psychogenic aphonia: spectacular This spectacular effect could also be mor or psychosis. While stutter is reported
explained by an important laryngeal mus- accompanying other symptoms of LOTS,1 it
recovery after motor cortex cular activation produced by the motor is not reported as the sole initial manifesta-
transcranial magnetic stimulation cortex stimulation and not by the prefrontal tion. We report three patients who pre-
cortex stimulation. sented in childhood with developmental
Psychogenic aphonia is a disabling conver- Motor cortex rTMS could be a therapeutic stutter, years before developing other neu-
sion disorder with no standard psychother- option for psychogenic aphonia, and perhaps rological manifestations.
apeutic1 or speech-therapeutic treatment.2 for other motor conversion disorders. To our Patient 1: This 6-year-old girl, born to
We present here a case report describing a knowledge, this is the first description of non-consanguineous parents of Ashkenazi
promising new treatment for this disorder psychogenic aphonia rTMS treatment. The Jewish and non-Jewish European back-
based on repetitive transcranial magnetic potential benefit of such treatment should ground, was the product of an uncompli-
stimulation (rTMS). be evaluated in a randomised controlled trial cated pregnancy and delivery. She spoke her
A left-handed 18-year-old woman devel- versus placebo. first words at 10 months, sat independently
oped a sudden total loss of normal speech and crawled at 7 months, and walked
production which was preceded by hoarse- N Chastan,1 D Parain,1 E Vérin,2 J Weber,1 M A Faure,3 independently at 12.5 months. She devel-
J-P Marie4
ness. Coughing and syllabic ‘‘trillo’’ phona- oped a marked stutter at 3 years, fine motor
1
tion were normal, indicating normal Department of Neurophysiology, Rouen University Hospital, delays at 4 years, and subsequent deteriora-
University of Rouen, France; 2 Department of Physiology,
articulatory ability. An otolaryngological Rouen University Hospital & UPRES-EA 3830-GRHV,
tion of gross and fine motor skills, social
examination noted a normal larynx with no University of Rouen, France; 3 Phonatrician, Paris, France; regression, cognitive decline and reduced
sign of lesion or vocal cord palsy, and 4
Department of Otolaryngology, Rouen University Hospital, & speech output.
videostroboscopic examination showed no UPRES-EA 3830-GRHV, University of Rouen, France Neurological exam at age 6 showed poor
vocal-cord adduction except during coughing. Correspondence to: Professor J-P Marie, CHU de Rouen, attention, difficulty following one-step com-
Neurological examination and brain MRI Service d’ORL, Dévé 1er étage, 1 rue de Germont, 76031 mands, sparse and dysarthric speech, tongue
were normal. The patient reported moderate Rouen cedex, France; jean-paul.marie@chu-rouen.fr weakness, limb rigidity and toe walking.
familial conflict and academic problems. Competing interests: None. Diagnostic testing revealed leucocyte HEXA
Psychological evaluation revealed chronic See Editorial Commentary, p 4 activity of 4% (normal: 63–75%) and serum
anxiety. Thirty sessions of speech therapy HEXA activity of 2% (normal: 56–80%),
Received 20 May 2008
produce no improvement. The conversion Revised 18 September 2008 consistent with Tay–Sachs disease.
disorder persisted for 20 months. As a final Accepted 22 September 2008 Mutation analysis of the HEXA gene revealed
treatment, two rTMS sessions of 150 s compound heterozygosity for the
J Neurol Neurosurg Psychiatry 2009;80:94.
duration (50 stimuli delivered at 0.33 Hz, doi:10.1136/jnnp.2008.154302 +TATC1278–1281 and c.1496G.A p.R499H
and maximal intensity of 2.5 Tesla) were mutations. A paternal uncle had a develop-
attempted with a circular coil (P/N 9784-00). mental stutter.
The first magnetic stimulation was applied to REFERENCES Patient 2: This 33-year-old right-handed
the left prefrontal cortex with no effect. One 1. Butcher P. Psychological processes in psychogenic
man, born to non-consanguineous parents of
voice disorder. Eur J Disord Commun 1995;30:467–74.
week later, a second rTMS session was 2. Maniecka-Aleksandrowicz B, Domeracka-Kolodziej non-Jewish European and Russian heritage,
applied to the right motor cortex with a A, Rozak-Komorowska A, et al. Management and was the product of an uncomplicated preg-
dramatic and immediate improvement, lead- therapy in functional aphonia: analysis of 500 cases. nancy, labour and delivery. He had one febrile
ing to a normal speech within a few days, and Otolaryngol Pol 2006;60:191–7. seizure at 9 months. Early developmental
still normal at 6 months’ follow-up. We 3. Burgmer M, Konrad C, Jansen A, et al. Abnormal milestones were normal. At 8 years, he
brain activation during movement observation in
attributed the clinical improvement to the developed an intermittent stutter. At age 14,
patients with conversion paralysis. Neuroimage
rTMS, and not to a placebo effect (lack of 2006;29:1336–43. he noted proximal weakness. Slowly progres-
effect with prefrontal cortex rTMS), or the 4. Ghaffar O, Staines WR, Feinstein A. Unexplained sive weakness and unsteadiness ensued. In his
natural course of the illness. neurologic symptoms: an fMRI study of sensory late teens, he had the first of several acute
Functional MRI studies have demon- conversion disorder. Neurology 2006;67:2036–8. psychotic episodes. In his late twenties, he
strated a reversible decreased activation of 5. Marshall JC, Halligan PW, Fink GR, et al. The developed progressive dysarthria and dyspha-
functional anatomy of a hysterical paralysis. Cognition
contralateral primary motor3 or somatosen- 1997;64:B1–8. gia. Diagnostic testing revealed leucocyte
sory cortex4 in conversion disorder patients, 6. Hurwitz TA, Prichard JW. Conversion disorder and HEXA activity of 5% (normal: 63–75%).
with motor and sensory symptoms respec- fMRI. Neurology 2006;67:1914–15. Mutation analysis revealed c.805G.A
tively. An abnormal activation of the orbi- 7. Schonfeldt-Lecuona C, Connemann BJ, Viviani R, et p.G269S and c.1510C.T p.R504C mutations.
tofrontal and anterior cingulate cortex has al. Transcranial magnetic stimulation in motor An older brother has LOTS.
conversion disorder: a short case series. J Clin
also been observed.5 The physiopathology of Neurophysiol 2006;23:472–5.
Neurological examination at age 33
conversion symptoms could be explained by showed an apathetic, inattentive male with
an active inhibition of these two prefrontal dysarthria, weakness of triceps, finger exten-
areas on primary motor areas.5 6 Late-onset Tay–Sachs disease sors, hip flexors, and left quadriceps, quad-
The modulation of cortical excitability by riceps fasciculations, pathologically brisk
rTMS could be a powerful new therapy for presenting as a childhood stutter patellar reflexes and extensor plantar
conversion disorders. In the past, patients Late-onset Tay–Sachs disease (LOTS) is a responses. There was limb dysmetria, ataxia,
with non-organic limb paralysis have been rare lysosomal storage disorder caused by dysdiadochokinesis, a postural tremor of
treated with 15 Hz rTMS sessions of motor deficient beta-hexosaminidase A (HEXA) both upper extremities and a wide-based,
cortex for 5–12 weeks,7 with variable bene- activity. Toxicity results from the accumu- spastic gait.
fits. Here, we hypothesise that they have lation of gangliosides in the central nervous Patient 3: This 39-year-old left-handed
activated the depressed motor cortex with system. In juvenile-onset forms, patients man born to non-consanguineous parents
excitatory high-frequency rTMS. On the present in childhood with progres- of Ashkenazi Jewish and non-Jewish
contrary, in our aphonic patient, recovery sive incoordination and/or developmental German background was the product of
was total with only one session of regression; in the ‘‘chronic’’ or ‘‘adult-onset’’ an uncomplicated pregnancy, labour and
low-frequency rTMS. We have possibly forms, patients present from childhood delivery. Early developmental milestones
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