夏学锋 易咏红 廖卫平 陆雪芬 彭隆祥 夏学锋、易咏红、 廖卫平、 陆雪芬 510182 广州医学院神经科学研究所；彭隆祥 湖南医科大学电镜室 中华神经科杂志 1999 0 32 3


    关键词：中枢神经系统疾病；；病理学；脑；活组织检查 期刊 zhsjkzz 0 论著 fur -->


    

【摘要】 目的 进一步认识Alexander病的临床和病理特征，探讨诊断方法。方法 对2例具有智能发育迟缓、惊厥等脑损害症状病例的临床和影像学，其中1例脑活检病理检查进行了研究。结果 1例婴儿期起病者头颅大，CT扫描示前额明显的广泛性脑白质密度减低特征，MRI示脑室旁白质T₂ 高信号；另1例学龄期起病，CT扫描显示脑萎缩，脑活检光镜下发现脱髓鞘和胶质细胞增生，电镜下观察到大量Rosenthal纤维(RFs)及不规则的颗粒状沉积物。结论 临床和影像学检查结合，可帮助对该病的拟诊，对临床疑诊的病例，应做脑活检电镜观察以明确诊断。

Clinical and pathological findings in Alexander disease

XIA Xuefeng^* , YI Yonghong, LIAO Weiping, et al. * Research Institute of Neurosciences, Guangzhou Medical College, Guangzhou 510182

【Abstract】 Objective To further understand theclinicopathological characterics of Alexander disease. Method Twopatients with mental retardation and convulsive seizures were subjected to the clincalstudy, light and electron microscopic study of brain biopsy. Results Onecase with infantile onset had macrocephaly. The CT scan and MRI showedprominentlow-density white matter throughout the cerebral hemispheres. MRI showed prominent alsoincreased T₂ signal in the periventricular whitematter. Another patient had anonset at school-age. The CT scan demonstrated brain atrophy. Histological examination onbrain biopsy revealed diffuse demyelination and fibrous gliosis. Rosenthal fibers werefound as bundles of glial and dense granular large masses on electron microscopy. Conclusion Alexanderdisease was diagnosed for the patients on clinical,neuroradiological and neuropathologicalbasis.

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