Honnorat J, Flocard F, Ribot C, Saint-Pierre G, Pineau D, Peysson P, Kopp N
Service de Neuropathologie, Hôpital Neurologique, Lyon.
Rev Neurol (Paris). 1993;149(12):781-7.
A 31-year old woman died after 10 years of progressive dysautonomia and cerebellar and pyramidal symptoms. CT scan showed pontine, bulbar and cerebellar atrophy. Post-mortem examination revealed Rosenthal's fibers widespread throughout the CNS, but especially in the subependymal and perivascular regions. White matter cavitations involving peri-ventricular regions, hilum of dentate nuclei and pons were observed, leading to a diagnosis of adult form of Alexander's disease. At the age of 5, the patient had been operated upon for a chiasmatic tumor. Microscopic examination revealed a pilocytic astrocytoma without Rosenthal's fibers. No complementary radiotherapy had been done. Her mother has been operated upon in 1972, for a high-grade glioma and is still alive 20 years later. This suggests diffuse cerebral gliomatosis. This family history may suggest a relation between these different diseases. They might be the result of a transmissible astrocytic abnormality with varying expression.
一名31岁女性在经历10年进行性自主神经功能障碍以及小脑和锥体症状后死亡。CT扫描显示脑桥、延髓和小脑萎缩。尸检发现罗森塔尔纤维广泛分布于整个中枢神经系统，尤其是室管膜下和血管周围区域。观察到脑室周围区域、齿状核门和脑桥的白质空洞形成，从而诊断为成人型亚历山大病。患者5岁时曾因视交叉肿瘤接受手术。显微镜检查显示为无罗森塔尔纤维的毛细胞型星形细胞瘤。未进行辅助放疗。她的母亲于1972年因高级别胶质瘤接受手术，20年后仍然存活。这提示弥漫性脑胶质瘤病。该家族史可能表明这些不同疾病之间存在关联。它们可能是具有不同表现的可传播星形细胞异常的结果。
