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目的：探讨肝豆状核变性（WD）患者脑干萎缩的发生率及其临床特征。方法：回顾我科收治的WD患者的临床表现、实验室检查及神经影像学等资料，分析伴有脑干萎缩的WD患者的临床特征。结果：50例患者的头颅MRI或CT均提示颅内出现异常信号，主要累及双侧豆状核、尾状核、丘脑、小脑和脑干。12例伴有广泛大脑萎缩，6例伴有小脑萎缩，6例伴有脑干萎缩（均以桥脑为著），2例影像学符合桥脑小脑橄榄萎缩。伴有脑干萎缩的患者球麻痹(25%)、锥体束征(50%)、眼震(25%)的发生率有升高的趋势，而病程、铜蓝蛋白、血清铜与不伴有脑干萎缩的患者无明显差异（P＞0.05）。结论：脑干萎缩在WD患者中并不罕见，此类患者较多伴有脑干损害的症状和体征，部分患者易被误诊。“,”Objective To investigate the incidence and clinical features of patients with Wilson’s disease (WD) plus brain stem atrophy. Methods Reviewing clinical manifestations, laboratory tests and neuroimaging data of WD patients admitted in our department and analyzing the clinical features in patient with WD plus brain stem atrophy. Results Cranial MRI or CT showed intracranial abnormal signal in 50 cases with WD, mainly involving the bilateral lenticular nucleus, caudate nucleus, thalamus, cerebel um and brain stem.12 cases with generalized brain atrophy, 6 cases associated with cerebel ar atrophy, 6 cases with brain stem atrophy (pons was more evident), and 2 case meet the imaging criterion of cerebel opontine olive atrophy. The incidence of bulbar palsy (25%), pyramidal tract signs (50%), and nystagmus (25%) in patients with brain stem atrophy has a elevated tendency. However, no significant differences in the course of the disease, ceruloplasmin, serum copper are observed WD patients in with brain stem atrophy. Conclusion Brain stem atrophy is not uncommon in WD patients. These patients are more associated with signs and symptoms of brain stem damage. Some patients maybe frequently misdiagnosed.