Russo L S, Aron A, Anderson P J
Neurology. 1976 Jul;26(7):607-14. doi: 10.1212/wnl.26.7.607.
A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination. This may be the first report of a case with significant neuronal changes and inflammation within the brain stem in this disease. The clinical picture of this entity is varied and age-dependent. Alexander's disease may be a motor system specific entity secondary to astrocytic dysfunction.
一名患有亚历山大病幼年起病症状的男孩,临床表现为假性延髓麻痹、共济失调和痉挛性截瘫。中枢神经系统的病理检查显示弥漫性存在罗森塔尔纤维和斑片状脱髓鞘。这可能是该疾病中首例脑干内存在显著神经元变化和炎症的病例报告。该疾病的临床表现多样且与年龄相关。亚历山大病可能是继发于星形细胞功能障碍的运动系统特异性疾病。