[Posterior fossa syndrome terminating the course of histiocytosis X. Study of lesions of the central nervous system. Association with polyvinylpyrrolidone thesaurismosis].
The authors report the pathological and clinical findings in a case of histiocytosis X in a child. A rapidly progressive neurological syndrome of brain stem damage, occurred 5 years after the onset. Blockage of the 3rd ventricle led to surgical exploration of the posterior fossa. Lesions of the brain stem and cerebellum, in addition to the granuloma of the hypothalamus, are described. The neurological symptoms in histiocytosis X often suggest a space-occupying lesion but usually occur in the later prolonged forms in the adult. Association of thesaurismosis due to P.V.P., of therapeutic origin, produced numerous clinical skin and joint signs.
D001933:Brain Stem / Q000473:pathology; D002490:Central Nervous System / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology; D002648:Child; D003388:Cranial Fossa, Posterior; D003711:Demyelinating Diseases / Q000473:pathology; D003919:Diabetes Insipidus / Q000188:drug therapy / Q000209:etiology; D006099:Granuloma / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000473:pathology*; D006801:Humans; D006833:Hydrarthrosis / Q000473:pathology; D007031:Hypothalamus / Q000473:pathology; D008297:Male; D008659:Metabolic Diseases / Q000150:complications / Q000473:pathology*; D011205:Povidone / Q000009:adverse effects* / Q000627:therapeutic use; D012867:Skin / Q000473:pathology; D012886:Skull; D013577:Syndrome; D013583:Synovial Membrane / Q000473:pathology; D014973:Xanthomatosis / Q000209:etiology / Q000473:pathology
D011205:Povidone
Syndrome de la fosse postérieure terminant l'évolution d'une histiocytose X. Etude des lésions du système nerveux central. Association d'une thésaurismose à la polyvinyl-pyrrolidone