Faivre J, Pecker J, Ferrand B
Sem Hop. 1975;51(37-38):2229-37.
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.
作者报告了一名儿童组织细胞增多症X的病理和临床发现。发病5年后出现了一种快速进展的脑干损伤神经综合征。第三脑室阻塞导致后颅窝手术探查。除下丘脑肉芽肿外，还描述了脑干和小脑的病变。组织细胞增多症X的神经症状常提示占位性病变，但通常发生在成人后期的长期形式中。由治疗性聚乙烯吡咯烷酮（P.V.P.）引起的贮积症产生了许多临床皮肤和关节体征。
