Movement Disorders (revue)

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Postencephalitic stereotyped involuntary movements responsive to L‐Dopa

Identifieur interne : 005594 ( Main/Exploration ); précédent : 005593; suivant : 005595

Postencephalitic stereotyped involuntary movements responsive to L‐Dopa

Auteurs : Picard [France] ; A. De Saint-Martin [France] ; E. Salmon [Belgique] ; E. Hirsch [France] ; C. Marescaux [France]

Source :

RBID : ISTEX:2BBFA4DB1DDB650C8F3636B263B3BF9146E63FEB

English descriptors

Abstract

In 1954, at the age of 5 years, our patient had an encephalitic syndrome associated with a prolonged lethargic state. After this episode, he developed a severe parkinsonian syndrome that, after a few years, was associated with axial dystonia and stereotyped abnormal movements of the upper limbs. This complex and progressive extrapyramidal syndrome had many similarities to the encephalitis lethargica as described by von Economo. Results of cerebral computed tomography and magnetic resonance imaging were normal. Fluorodopa positron emission tomography showed a significant bilateral reduction of tracer accumulation in both putamen, similar to that observed in patients with idiopathic Parkinson's disease. However, in this patient, treatment with L‐Dopa suppressed all akinetic, dystonic and dyskinetic symptoms. The effectiveness of L‐Dopa was abolished by administration of a D2 antagonist and was fully reproduced by a D2 agonist. In conclusion, this patient presented a complex postencephalitic, extrapyramidal syndrome, with akinetic symptoms and involuntary movements. These symptoms appeared to be related to a limited lesion of the dopaminergic neurons of the zona compacta of the substantia nigra.

Url:
DOI: 10.1002/mds.870110513


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="de">In 1954, at the age of 5 years, our patient had an encephalitic syndrome associated with a prolonged lethargic state. After this episode, he developed a severe parkinsonian syndrome that, after a few years, was associated with axial dystonia and stereotyped abnormal movements of the upper limbs. This complex and progressive extrapyramidal syndrome had many similarities to the encephalitis lethargica as described by von Economo. Results of cerebral computed tomography and magnetic resonance imaging were normal. Fluorodopa positron emission tomography showed a significant bilateral reduction of tracer accumulation in both putamen, similar to that observed in patients with idiopathic Parkinson's disease. However, in this patient, treatment with L‐Dopa suppressed all akinetic, dystonic and dyskinetic symptoms. The effectiveness of L‐Dopa was abolished by administration of a D2 antagonist and was fully reproduced by a D2 agonist. In conclusion, this patient presented a complex postencephalitic, extrapyramidal syndrome, with akinetic symptoms and involuntary movements. These symptoms appeared to be related to a limited lesion of the dopaminergic neurons of the zona compacta of the substantia nigra.</div>
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