表现为星形细胞瘤的亚历山大病
Alexander's disease presenting as astrocytoma.
作者信息
French T A, Bower B D, Cameron A H
出版信息
J Neurol Neurosurg Psychiatry. 1976 Aug;39(8):803-9. doi: 10.1136/jnnp.39.8.803.
Abstract
A case of progressive fibrinoid degeneration of astrocytes is described. Symptoms began with convulsions at the age of 6 weeks and cerebral tumour was suspected because of enlargement of the head, increased intracranial tension, and marked proliferation of astrocytes in the brain biopsy which was interpreted as an astrocytoma. Fibres and granules staining deeply with PTAH are a constant feature of the few cases so far described. The aetiology is unknown but an inherited metabolic defect of astrocytes may be responsible.
摘要
本文描述了一例星形胶质细胞进行性纤维蛋白样变性的病例。症状始于6周龄时的惊厥，由于头部增大、颅内压升高以及脑活检中星形胶质细胞明显增生（被解释为星形细胞瘤），怀疑患有脑肿瘤。迄今为止所描述的少数病例中，用磷钨酸苏木精（PTAH）染色呈深染的纤维和颗粒是一个恒定特征。病因不明，但可能是星形胶质细胞的遗传性代谢缺陷所致。
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Dysmyelinogenic leukodystrophy; report of a case of a new, presumably familial type of leukodystrophy with megalobarencephaly.脱髓鞘性脑白质营养不良症；一例新的、可能为家族性的伴有巨脑回的脑白质营养不良症病例报告。
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[Case of leukodystrophy with diffuse Rosenthal fiber formation (Alexander's disease)].[伴有弥漫性罗森塔尔纤维形成的脑白质营养不良病例（亚历山大病）]
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