Reusche E, Gerke P, Krüger S, Rohwer J, Lindner B, Rob P M
Institut für Pathologie, Bereich Neuropathologie, Medizinische Universitt Lübeck.
Dtsch Med Wochenschr. 1999 Feb 19;124(7):176-81. doi: 10.1055/s-2007-1024270.
A 73-year-old woman in renal failure for the past 22 years had been on haemodialysis for 16 years. Because of hyperphosphataemia and peptic ulcers she had been on aluminium-containing antacids with a total intake over time of about 8 kg "pure" aluminium. Over the past 11 years she had biphasic symptoms of death anxieties and depression. She also had amnesic aphasia and some extrapyramidal symptoms as well as generalized convulsive seizures and recurrent falls.
Cranial computed tomography merely revealed signs of a microangiopathy and an age-related decrease in brain volume. The EEG showed intermittent changes while the CSF and ECG were unremarkable. There was no benzodiazepine or ethanol in the blood.
After excluding stroke with secondary epilepsy, uraemic encephalopathy was assumed to be the cause of the severe organic psychiatric syndrome. In the last few days before her death the patient had disturbance of consciousness and of breathing. She died during grotesque tossing movements, thought to be due to a brain stem stroke. Autopsy revealed high-grade myocardial hypertrophy caused by the hypertension, contracted kidney of vascular cause, hyperplasia of the parathyroid and calcification of the renal parenchyma as a sign of secondary parathyroidism. The CNS showed severe dialysis-associated encephalopathy with characteristic argyrophilic, aluminium-induced lysosomal intracytoplasmic inclusions in the choroid plexus epithelium, cortical glia and numerous neuron populations. Laser microprobe mass analysis (LAMMA) confirmed manifold increase in subcellular aluminium content, especially in the neuronal cytoplasm, also demonstrated by atom absorption spectrometry. Additional distinct deposition of beta A4-amyloid, typical of Alzheimer's disease, was probably age-related rather than associated with the dialysis and the aluminium uptake.
Dialysis-associated encephalopathy must be taken into account as a possible cause of aetiologically uncertain neuropsychiatric symptoms in patients on chronic haemodialysis.
一名73岁女性，肾衰竭22年，已接受血液透析16年。因高磷血症和消化性溃疡，她长期服用含铝抗酸剂，累计摄入约8千克“纯”铝。在过去11年里，她出现了双相性死亡焦虑和抑郁症状。她还患有遗忘性失语、一些锥体外系症状、全身性惊厥发作及反复跌倒。
头颅计算机断层扫描仅显示微血管病变迹象及与年龄相关的脑容量减小。脑电图显示间歇性变化，脑脊液和心电图无异常。血液中未检测到苯二氮䓬类药物或乙醇。
排除继发性癫痫的中风后，推测尿毒症性脑病是严重器质性精神综合征的病因。在她去世前的最后几天，患者出现意识和呼吸障碍。她在离奇的翻滚动作中死亡，推测是脑干中风所致。尸检显示高血压导致的重度心肌肥厚、血管性肾萎缩、甲状旁腺增生以及肾实质钙化，为继发性甲状旁腺功能亢进的表现。中枢神经系统显示严重的透析相关性脑病，脉络丛上皮、皮质神经胶质细胞和众多神经元群体中有特征性的嗜银性、铝诱导的溶酶体细胞质内包涵体。激光微探针质谱分析（LAMMA）证实亚细胞铝含量大量增加，尤其是在神经元细胞质中，原子吸收光谱法也证实了这一点。此外，典型的阿尔茨海默病β淀粉样蛋白的明显沉积可能与年龄有关，而非与透析及铝摄入相关。
对于慢性血液透析患者，病因不明的神经精神症状，必须考虑透析相关性脑病这一可能病因。
