Boutaleb N, Ouhabi H, Bourazza A, Mosseddaq R
Service de neurologie, Hôpital militaire d'instruction Mohammed V de Rabat, Maroc.
Rev Neurol (Paris). 2000 Sep;156(8-9):790-3.
Xeroderma pigmentosum is a genodermatosis with neurological manifestations in approximately 18p. 100 of cases. Polymorphous and variably associated signs are observed, progressing with the clinical course. The etiology of the neurological breach remains unknown. We report two siblings who had xeroderma pigmentosum with intellectual deficiency, a pyramidal, cerebellar and cordonal syndrome, ophthalmoplegia, and axonal peripheral neuropathy. We discuss the epidemiological, clinical and electrophysiological aspects of the neurological breach in xeroderma pigmentosum.
着色性干皮病是一种遗传性皮肤病，约18%的病例有神经学表现。观察到多形性且存在不同关联的体征，随临床病程进展。神经学损害的病因尚不清楚。我们报告了两名患有着色性干皮病的兄弟姐妹，他们伴有智力缺陷、锥体束、小脑和脊髓综合征、眼肌麻痹以及轴索性周围神经病。我们讨论了着色性干皮病神经学损害的流行病学、临床和电生理方面。
