Lederman R J, Henry C E
Ann Neurol. 1978 Sep;4(3):199-204. doi: 10.1002/ana.410040302.
The clinical features of 42 patients with the only recently recognized and generally fatal neurological syndrome of progressive dialysis encephalopathy are reviewed and the electroencephalographic and neuropathological findings are summarized. Despite apparently successful hemodialysis, these patients develop a wide spectrum of neurological abnormalities. Of these, sudden onset of hesitant, nonfluent speech is the most characteristic and usually the earliest sign. Both dysphasic and dysarthritic elements are found, though the former predominate. Myoclonus, dementia, seizures, and gait difficulty are also seen in the majority of these patients. EEGs are more abnormal than would be expected for the clinical severity, with some type of high-voltage spike-wave pattern intermixed with abundant slow activity. The combination of clinical and EEG features in the appropriate setting is virtually diagnostic. Transient episodes with variable periods of complete or partial remission have been recognized. Neuropathological changes are surprisingly mild and nonspecific. The cause is uncertain; current speculation focuses on aluminum as the offending neurotoxin. Treatment remains unsatisfactory.
回顾了42例患有最近才被认识到的、通常致命的进行性透析性脑病神经综合征患者的临床特征，并总结了脑电图和神经病理学检查结果。尽管血液透析表面上取得了成功，但这些患者仍出现了广泛的神经异常。其中，突然出现言语迟疑、不流畅是最具特征性且通常是最早出现的症状。虽然以言语障碍为主，但也发现了构音障碍和言语不清的情况。大多数患者还出现肌阵挛、痴呆、癫痫发作和步态困难。脑电图异常程度比根据临床严重程度预期的更为严重，存在某种类型的高电压棘波模式，并伴有大量慢波活动。在适当的情况下，临床特征和脑电图特征相结合几乎具有诊断价值。已经认识到存在有完全或部分缓解的不同时期的短暂发作。神经病理学变化出人意料地轻微且不具特异性。病因尚不确定；目前的推测集中在铝是致病神经毒素上。治疗效果仍然不尽人意。
