[Olivo-ponto-cerebellar and striato-nigral atrophy. 3 anatomo-clinical cases. Review of the literature].
AOPC is frequently associated with degeneration in other systems, the striato-nigral in particular. Three anatomical-clinical cases of AOPC complicated by striato-nigral degeneration of varying gravity are described. Case 1 had a complex history: rigidity with axial predominance, plus signs in neurovegetative system, ocular palsy, myoclonia and fasciculation and a massive atrophy of the olivo-ponto-cerebellar and striato-nigral systems. Case 2 presented as a static and kinetic cerebellar syndrome; the anatomical confirmation of the AOPC involvement showing a patchy nigro-striatal degeneration but without clinical signs. Case 3 evolved as a Parkinson-type syndrome differentiated from true Parkinson's disease by signs affecting the pyramidal system and the relative importance of static signs, the lesions then involving namely the striato-nigral system. 69 allied cases in the literature are referred to and commented upon demonstrating the great clinical and anatomical variation in presentation of AOPC. The concept of "multiple system atrophy" allows most cases to be grouped under a general heading. The degenerative diseases of the CNS, in so far as their etiology remains obscure, make up a "waiting list".
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Kohler
D000368:Aged; D001284:Atrophy; D002531:Cerebellum / Q000473:pathology*; D003342:Corpus Striatum / Q000473:pathology*; D003711:Demyelinating Diseases / Q000473:pathology; D005260:Female; D005911:Gliosis / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration; D009468:Neuromuscular Diseases / Q000473:pathology; D009847:Olivary Nucleus / Q000473:pathology*; D010300:Parkinson Disease / Q000473:pathology; D011149:Pons / Q000473:pathology*; D013378:Substantia Nigra / Q000473:pathology*
Atrophie olivo-ponto-cérébelleuse et striato-nigrique. Trois cas anatomo-cliniques. Revue de la littérature.