The aim of this section is to prepare the neuro- everyday neurological practice and to give him logist-in-training for the FMH examination, to or her updates on recent controversies in clinical confront her or him with specific problems of Contributions and correspondence to PD Dr. med. Hans H. Jung, Depar tment of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, CH-8091 Zurich, [email protected] The 60-year-old right-handed man, originating that he could live alone in his apartment in the from Serbia, was sent for a neurological con - future. At admission he was fully oriented, he sultation because of a gait disorder. His son and had an impaired short-term memory and was in his ex-wife reported changes of his personality, a depressive mood. Neurological examination difficulties to articulate and a peculiar gait since revealed no oculomotor abnormalities, no sen- several years. Because of a depression he was in sory-motor deficits and a normal coordination.
ambulatory psychiatric care. He had lost 30 kilo- Tendon reflexes were brisk, plantar responses grammes of body weight in the last 3 years were flexor. Gait was broad based without clear and reported to have troubles to fall asleep. gait ataxia. There was a generalised hyperkine- At the time of the first consultation, he had a sia of limbs and to a lesser extent of the face medication with diazepam 10 mg per day. Due and the trunk with sudden brisk movements to his health problems, his relatives doubted What are possible causes of a choreatic movement disorder? Which are the components of a chorea syndrome? What are symptomatic treatments of chorea? S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E Due to the history of the patient, a paraneo- of the lateral ventricles which is ob vious in plastic chorea syndrome was suggested. Exten- the coronal sections but not necessarily in sive work-up including chest- and abdomen- the axial sections (arrows, fig. 1A and 1B). computed tomo graphy as well as determination FDG-positron emission tomography showed a of anti-neuronal paraneoplastic antibodies was severely impaired striatal FDG uptake. Because negative. Blood smears did not reveal ery thro - of the negative evaluation of a symptomatic cyte acanthocytosis. Immunological evalua- chorea syndrome, a neurodegenerative chorea tion including anti-double-stranded DNA anti - syndrome was suspected. After appropriate bodies and anti-phospholipid antibodies was ge netic counselling a molecular genetic ana - negative. Cerebral magnetic re sonance imaging lysis of the huntingtin gene was performed, revealed a slight atrophy of the head of the demonstrating 43 CAG repeats in the hunting - caudate nucleus with consecutive enlargement Table 1. Neurodegenerative chorea syndromes.
S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E Treatment with tiapride (Tiapridal®) 3 × 100 mg disorder was less pronounced, and he could still and mirtazepine (Remeron®) 1 × 30 mg was live in his own apartment with minor support initiated. After 3 months sleep quality had con- of relatives and the local social service.
siderably improved, the choreatic movement fi A choreatic movement disorder may have improve the quality of life of patients Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol. 2006;5:589–602.
Jung HH. Differentialdiagnose hereditärer Chorea-Syndrome.
Walker RH, Jung HH, Dobson-Stone C, Rampoldi L, Sano A, Schweiz Arch Neurol Psychiatr. 2002;153:185–8.
Tison F, et al. Neurologic phenotypes associated with acanthocytosis. Neurology. 2007;68:92–8.
S C H W E I Z E R A R C H I V F Ü R N E U R O L O G I E U N D P S Y C H I A T R I E obsessive-compulsive disorders as well as manicand psychotic symptoms.
Choreatic movements may be the consequence ofan inflammatory alteration of the basal ganglia in the context of a rheumatic fever, a systemic lupus Cognitive dysfunction in chorea syndromes often erythematodes or a phospholipid antibody syn- spares long-term memory but impairs executive drome. Rarely, choreatic movement disorders functions, such as organising, planning, checking have been described as a paraneoplastic syndrome.
or adapting alternatives, and delays the acquisition A choreatic movement disorder may arise as a of new motor skills. Anosognosia is often evident.
side effect of medications such as neuroleptics.
Structural brain lesions of the striatum or the subthalamic nucleus such as an ischaemia hae mor- r hage or a brain tumour may cause haemichorea or hemiballism. Tic disorders or psychogenic move- Choreatic movement disorders may be amelior - ment disorders may have chorea-like features.
ated by dopamine antagonists or dopamine delet - Finally, a choreatic movement disorder may be a ing drugs. First-line medications are dopamine symptom of a neurodegenerative chorea syndrome antagonistic neuroleptics such as tiapride (Tia pridal®), 3 × 100 mg to 3 × 400 mg per day, or sulpi -ride (Dogmatil®), 2 × 50 mg to 2 × 400 mg per day.
Other neuroleptic drugs with antichoreatic effect are clozapine (Leponex®), 3 × 6.25 to 3 × 25 mg, or haloperidole (Haldol®), 3 × 0.5 to 3 × 2 mg.
Because of the high risk of tardive dyskinesias Chorea is the ancient Greek word for “dancing”. or other extrapyramidal side effects, however, A choreatic movement disorder is defined as rapid, haloperidole is reserved for severe, otherwise irregular, spontaneous movements which arise untreatable choreatic movement disorders. An accidently and abruptly flow from one part of the alternative to the neuroleptic drugs is the dopa - body to the other. Choreatic movement may be mine depleting drug tetrabenazine (Nitoman®), incorporated in voluntary movements such as in 25 to 100 mg per day. A relevant possible adverse effect is the development of a depression. Tetra -benazine is not approved in Switzerland, but there is an approval for the treatment of choreatic Psychiatric manifestations are frequent in chorea movement disorders in Germany. It may be pre- syndromes and may precede motor or cognitive scribed as an “orphan drug” for the treatment of manifestations. Psychiatric manifestations may in- a choreatic movement disorder after approval of clude changes of personality, affective disorders, assumption of costs by the health insurance.



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