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Postencephalitic stereotyped involuntary movements responsive to L ‐Dopa
Author(s) -
Picard F.,
de SaintMartin A.,
Salmon E.,
Hirsch E.,
Marescaux C.
Publication year - 1996
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870110513
Subject(s) - dystonia , substantia nigra , putamen , dopaminergic , medicine , parkinsonism , magnetic resonance imaging , hyperkinesia , psychology , akinetic mutism , movement disorders , anesthesia , neuroscience , dopamine , disease , radiology
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 D 2 antagonist and was fully reproduced by a D 2 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.