Burn D J, Bates D
Department of Neurology, Royal Victoria Infirmary, Newcastle upon Tyne, UK.
J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):810-21. doi: 10.1136/jnnp.65.6.810.
Renal failure is relatively common, but except in association with spina bifida or paraplegia it is unlikely to occur as a result of disease of the CNS. Renal failure, however, commonly affects the nervous system. The effects of kidney failure on the nervous system are more pronounced when failure is acute. In addition to the important problems related to renal failure there are both acquired and genetically determined diseases which may affect the kidney and the brain. Those acquired diseases include the vasculitides, the paraproteinaemias, and various granulomatous conditions (considered in other chapters of Neurology and Medicine). In two of the most commonly encountered genetically determined diseases, Von Hippel-Lindau disease and polycystic kidney disease, location of pathogenic mutations will provide improved screening programmes and, possibly, allow therapeutic intervention. Uraemia may affect both the central and peripheral nervous systems. Whereas the clinical features of uraemia are well documented, the pathophysiology is less well understood and probably multifactorial. Uraemic encephalopathy, which classically fluctuates, is associated with problems in cognition and memory and may progress to delirium, convulsions, and coma. The encephalopathy may initially worsen with periods of dialysis and almost certainly relates to altered metabolic states in association with ionic changes and possibly impaired synaptic function. Renal failure may affect the peripheral nervous system, resulting in a neuropathy which shows a predilection for large diameter axons. This may be reversed by dialysis and transplantation. The myopathy seen in renal failure, often associated with bone pain and tenderness, is similar to that encountered in primary hyperparathyroidism and osteomalacia. Dialysis itself is associated with neurological syndromes including the dysequilibrium syndrome, subdural haematoma, and Wernicke's encephalopathy. Dialysis dementia, which was prevalent during the 1970s, has reduced in frequency with the use of aluminium free dialysate. With the introduction of transplantation and the concomitant use of powerful immunosuppressive drugs, the pattern of neurological problems encountered in renal replacement therapy has shifted. Five per cent of patients develop nerve injuries during renal transplantation, and up to 40% of patients experience neurological side effects from cyclosporine. Furthermore, CNS infections, often fungal in type, have been reported in up to 45% of transplant patients coming to postmortem. The nature of the involvement of neurologists with their nephrology colleagues is therefore evolving.
肾衰竭相对常见,但除了与脊柱裂或截瘫相关外,不太可能因中枢神经系统疾病而发生。然而,肾衰竭通常会影响神经系统。肾衰竭对神经系统的影响在急性肾衰竭时更为明显。除了与肾衰竭相关的重要问题外,还有一些后天性和基因决定的疾病可能会影响肾脏和大脑。那些后天性疾病包括血管炎、副蛋白血症和各种肉芽肿性疾病(在神经病学和医学的其他章节中讨论)。在两种最常见的基因决定疾病,即冯·希佩尔-林道病和多囊肾病中,致病突变的定位将提供改进的筛查方案,并可能允许进行治疗干预。尿毒症可能会影响中枢神经系统和周围神经系统。虽然尿毒症的临床特征已有充分记录,但其病理生理学了解较少,可能是多因素的。尿毒症性脑病通常呈波动性,与认知和记忆问题有关,可能会发展为谵妄、惊厥和昏迷。脑病最初可能会随着透析期而恶化,几乎可以肯定与离子变化相关的代谢状态改变以及可能受损的突触功能有关。肾衰竭可能会影响周围神经系统,导致一种对大直径轴突有偏好的神经病变。这种病变可通过透析和移植得到逆转。肾衰竭中出现的肌病,通常与骨痛和压痛有关,与原发性甲状旁腺功能亢进和骨软化症中遇到的肌病相似。透析本身与包括失衡综合征、硬膜下血肿和韦尼克脑病在内的神经综合征有关。在20世纪70年代很普遍的透析性痴呆,随着无铝透析液的使用,发病率有所降低。随着移植的引入以及同时使用强效免疫抑制药物,肾脏替代治疗中遇到的神经问题模式已经发生了变化。5%的患者在肾移植期间会发生神经损伤,高达40%的患者会出现环孢素的神经副作用。此外,在高达45%的接受尸检的移植患者中报告了中枢神经系统感染,通常为真菌类型。因此,神经科医生与肾病科同事的合作性质正在不断演变。