Devivo D C, Malas D, Nelson J S, Land V J
Neurology. 1977 Jul;27(7):609-13. doi: 10.1212/wnl.27.7.609.
A 14-year-old boy survived for 7 years after the initial diagnosis and treatment of acute lymphocytic leukemia. Neurologic deterioration occured repeatedly throughout his complicated clinical course but it was most severe and only partially reversible following orally administered pyrimethamine. The neuropathologic lesions were distinctive and included a diffuse reactive astrocytosis, axonal degeneration, status spongiosis, circumscribed foci of demyelination and coagulative necrosis, and mural thickening with luminal narrowing of microcirculatory vessels. This collection of findings represents the leukoencephalopathy of childhood leukemia that we and others believe results in large part from the combined effects of cranial irradiation and chemotherapy. The role of folic acid antagonists, namely methotrexate and pyrimethamine, are particularly noteworthy in this regard.
一名14岁男孩在急性淋巴细胞白血病初次诊断和治疗后存活了7年。在其复杂的临床病程中,神经功能恶化反复出现,但在口服乙胺嘧啶后最为严重且仅部分可逆。神经病理学病变具有特征性,包括弥漫性反应性星形细胞增生、轴索变性、海绵状状态、局限性脱髓鞘和凝固性坏死灶,以及微循环血管壁增厚伴管腔狭窄。我们和其他人认为,这一系列发现代表了儿童白血病性白质脑病,其很大程度上是由颅脑照射和化疗的联合作用导致的。在这方面,叶酸拮抗剂,即甲氨蝶呤和乙胺嘧啶的作用尤其值得关注。