Sabouraud O, Chatel M, Menault F, Dien Peron J, Cartier F, Garre M, Gary J, Pecker S
Rev Neurol (Paris). 1978 Oct;134(10):575-600.
Clinical and Neuropathological data on sixteen cases of progressive myoclonic encephalopathy are reported. This neurological syndrome appears after an average duration of thirty two months of haemodialysis and leads to death in four and a half months, and is characterized by myoclonus, speech disorder, epileptic seizures, and mental-status changes. At first, clinical signs and symptoms are related to haemodialysis, later they become permanent. An early diagnosis is based on EEG which is the only useful laboratory test, demonstrating bisynchronous slow-wave bursts. The caracteristic histopathologic findings are neuronal depopulation, lipofuscin accumulation, and appearance of Neurofibrillary degeneration, especially in Motor cortex, red nucleus and dentato-olivary systems. It seems to be justified to attribute P.M.D.E. to aluminium chronic poisonning; the source of the aluminium intoxication is not aluminium containing phosphate-binding gels but intravenously administreted tape-water. The intracellular binding of aluminium is shown from a histochemical study employing fluorescent stain Morin.
报告了16例进行性肌阵挛性脑病的临床和神经病理学数据。这种神经综合征在平均32个月的血液透析后出现，并在4个半月内导致死亡，其特征为肌阵挛、言语障碍、癫痫发作和精神状态改变。起初，临床体征和症状与血液透析有关，后来变得持续存在。早期诊断基于脑电图，这是唯一有用的实验室检查，显示双同步慢波爆发。特征性的组织病理学发现是神经元减少、脂褐素积累和神经原纤维变性的出现，尤其是在运动皮质、红核和齿状核-橄榄系统。将进行性肌阵挛性脑病归因于铝慢性中毒似乎是合理的；铝中毒的来源不是含铝的磷酸盐结合凝胶，而是静脉注射的自来水。通过使用荧光染料桑色素的组织化学研究显示了铝的细胞内结合。
