Kuncl R W, Duncan G, Watson D, Alderson K, Rogawski M A, Peper M
N Engl J Med. 1987 Jun 18;316(25):1562-8. doi: 10.1056/NEJM198706183162502.
Although colchicine has been used for centuries, its neuromuscular toxicity in humans is largely unrecognized. In this report we describe a characteristic syndrome of myopathy and neuropathy and present 12 new cases of the condition. Colchicine myopathy may occur in patients with gout who take customary doses of the drug but who have elevated plasma drug levels because of altered renal function. It usually presents with proximal weakness and always presents with elevation of serum creatine kinase; both features remit within three to four weeks after the drug is discontinued. The accompanying axonal polyneuropathy is mild and resolves slowly. Electromyography of proximal muscles shows a myopathy that is marked by abnormal spontaneous activity. Because of these features, colchicine myoneuropathy is usually misdiagnosed initially, either as probable polymyositis or as uremic neuropathy. The myopathy is vacuolar, marked by accumulation of lysosomes and autophagic vacuoles unrelated to necrosis or to the mild denervation in distal muscles. The morphologic changes in muscle suggest that the pathogenesis involves disruption of a microtubule-dependent cytoskeletal network that interacts with lysosomes. Correct diagnosis may save patients with this disorder from inappropriate therapy.
尽管秋水仙碱已被使用了几个世纪,但其在人类中的神经肌肉毒性在很大程度上未被认识到。在本报告中,我们描述了一种特征性的肌病和神经病变综合征,并介绍了12例该病症的新病例。秋水仙碱肌病可能发生在痛风患者中,这些患者服用常规剂量的药物,但由于肾功能改变导致血浆药物水平升高。它通常表现为近端肌无力,且总是伴有血清肌酸激酶升高;停药后三到四周内这两个特征都会缓解。伴随的轴索性多发性神经病较轻且缓解缓慢。近端肌肉的肌电图显示为一种以异常自发电活动为特征的肌病。由于这些特征,秋水仙碱肌神经病变最初通常会被误诊,要么被误诊为可能的多发性肌炎,要么被误诊为尿毒症性神经病。这种肌病是泡状的,其特征是溶酶体和自噬泡的积累,与坏死或远端肌肉的轻度失神经支配无关。肌肉的形态学变化表明,其发病机制涉及与溶酶体相互作用的微管依赖性细胞骨架网络的破坏。正确的诊断可以使患有这种疾病的患者避免不适当的治疗。