title: [Striato-nigral degeneration. A propos of 2 anatomo-clinical cases].
abstract: Two sporadic cases of striato-nigral degeneration were characterized by the onset at the age of 61 of an akinetic-hypertonic syndrome and of a minimal or absent rest tremor, with a pyramidal syndrome, sphincter disorders, dysphonia, difficulty in swallowing and an unfavorable development in the space of two or three years not affected by L-Dopa. In one case, total insomnia was demonstrated by means of 3 polygraphic recordings during the night, two of them consecutive, and aprobenecide test showed a definite fall in H.V.A. and 5 H.I.A.A. in the lumbar cerebrospinal fluid. Anatomical verification showed, in one case, isolated putamino-nigral degeneration, and in the other associated with lesions of the olivo-cerebellopontine system. Much of the excess pigmentation found only in the putamen was melanotic in character and resulted perhaps from the striato-nigral degeneration, making it possible to classify this more accurately among the other multisystem degenerations, especially olivo-ponto-cerebellar atrophy and Shy-Drager syndrome
mesh_terms: D001927:Brain Diseases / Q000134:cerebrospinal fluid / Q000473:pathology / Q000503:physiopathology*; D003342:Corpus Striatum; D005260:Female; D006719:Homovanillic Acid / Q000134:cerebrospinal fluid; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000134:cerebrospinal fluid; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000473:pathology / Q000503:physiopathology*; D011699:Putamen / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology; D013577:Syndrome
chemical_list: D006897:Hydroxyindoleacetic Acid; D006719:Homovanillic Acid
vernacular_title: Dégénérescence striato-nigrique. A propos de deux observations anatomo-cliniques