Department of Nephrology and Transplantation, Fiona Stanley Hospital, 11-15 Robin Warren Drive, Murdoch, WA 6150, Australia.
Department of Neurology, Sir Charles Gairdner Hospital, Hospital Avenue Nedlands, Perth, WA 6009, Australia.
QJM. 2022 Jun 7;115(6):359-366. doi: 10.1093/qjmed/hcab140.
Acute extrapyramidal movement disorders in dialysis patients are rare, inconsistently defined and have uncertain aetiology and prognosis.
Define diagnostic criteria, prognosis and risk factors.
Retrospective case series review of 20 patients (14 female, mean age 62 years) receiving dialysis for a median of 15 (interquartile range 4-35) months who presented with acute parkinsonism (AP = 11) or chorea/athetosis (CA = 9).
All patients had type 2 diabetes (HbA1c 6.8 ± 1.0) and had received metformin. Lactic acidosis was present in 2 patients at presentation and serum lactate was elevated in 7/15 patients tested. No patient had abnormal copper or thyroid metabolism and 5/8 patients tested returned marginal abnormalities in heavy metal screening. Magnetic resonance imaging (MRI) revealed characteristic bilateral symmetric T2 hyperintensity of the basal ganglia (BG), predominantly putamen and globus pallidus (the lentiform nucleus) and more extensive involvement of the external and internal capsules in patients with AP presentation. Post-mortem demonstrated cytotoxic necrosis of the BG. Therapy included thiamine, intensive dialysis and cessation of metformin. Two patients died acutely, nine recovered and nine had residual symptoms. Median survival did not differ by presentation: AP 24 [95% confidence interval (CI) 21-27] and CA 33 (95% CI 32-35) months, P = 0.21.
There are two distinct clinical extrapyramidal movement disorders associated with specific diagnostic MRI imaging that support the diagnosis of the extrapyramidal syndromes of chronic kidney disease and dialysis. The associations with diabetes, metformin and metabolic acidosis suggest a common pathogenic mechanism but require additional study. Early recognition and treatment may improve outcomes.
透析患者急性锥体外系运动障碍罕见，定义不一致，病因和预后不确定。
定义诊断标准、预后和危险因素。
回顾性分析 20 例（14 例女性，平均年龄 62 岁）接受透析治疗的患者，透析中位时间为 15 个月（四分位距 4-35 个月），出现急性帕金森病（AP=11 例）或舞蹈症/手足徐动症（CA=9 例）。
所有患者均患有 2 型糖尿病（HbA1c 6.8±1.0），并接受二甲双胍治疗。2 例患者在就诊时存在乳酸酸中毒，7/15 例检测的血清乳酸升高。无患者存在异常铜或甲状腺代谢，5/8 例检测的重金属筛查结果显示边缘异常。磁共振成像（MRI）显示特征性双侧基底节（BG）T2 高信号，主要为壳核和苍白球（豆状核），AP 表现患者的外囊和内囊更广泛受累。尸检显示 BG 细胞毒性坏死。治疗包括硫胺素、强化透析和停用二甲双胍。2 例患者急性死亡，9 例患者恢复，9 例患者仍有残留症状。中位生存时间与表现无关：AP 24 个月（95%置信区间 21-27 个月）和 CA 33 个月（95%置信区间 32-35 个月），P=0.21。
有两种不同的临床锥体外系运动障碍与特定的 MRI 成像诊断相关，支持慢性肾脏病和透析的锥体外系综合征的诊断。与糖尿病、二甲双胍和代谢性酸中毒的关联提示存在共同的发病机制，但需要进一步研究。早期识别和治疗可能改善结局。
