Alexander's Disease: Unique Presentation
Alexander's Disease: Unique Presentation
复制标题
亚历山大氏病：独特的表现
作者：
M. Gingold;J. Bodensteiner;S. Schochet;M. Jaynes
亚急性坏死性脑脊髓病（Leigh综合征）是指一种以乳酸酸中毒为特征的雾状疾病实体，临床表现多样，病理表现一致。几种代谢异常与Leigh综合征有关，但许多病例未发现代谢异常。我们报告一个临床、代谢和神经放射学表现为Leigh综合征的病例。此外，我们的患者表现出其他不寻常的临床表现，包括眼运动异常。然而，神经病理学上，亚历山大病的诊断得到了证实。查阅文献未发现其他有本例患者所表现的代谢异常和临床表现的亚历山大病病例（J Child Neurol 1999; 14:25 -336）。
Subacute necrotizing encephalomyelopathy (Leigh syndrome) refers to a nebulous disease entity characterized by lactic acidosis, a wide variety a clinical manifestations, and a consistent conglomeration of pathlogic findings. Several abnormalities in metabolism have been delineated in association with Leigh syndrome, but many cases have no identified metabolic abnormality. We report a case that clinically, metabolically, and neuroradiologically appeared to be Leigh syndrome. In addition, our patient exhibited other unusual clinical findings, including ocular motility abnormalities. Neuropathologically, however, the diagnosis of Alexander's disease was confirmed. A review of the literature failed to find other cases of Alexander's disease reported with the metabolic abnormalities and clinical manifestations with which our patient presented (J Child Neurol 1999;14:325-336).
影响因子：
4.2
作者：
McNaught, KSP;Olanow, CW
通讯作者：
Olanow, CW