就诊于神经内科的537例上睑下垂患者的病因分布及临床特征

Etiological distribution and clinical characteristics of 537 patients with ptosis in neurology department

目的探讨上睑下垂患者的病因分布及临床特征。设计回顾性病例系列。研究对象2023年1-12月就诊于北京同仁医院神经内科,表现为上睑下垂的患者537例。方法收集患者的临床资料,进行病因分类及统计分析。主要指标平均发病年龄、病程、性别、双侧受累以及是否伴随疼痛、复视、瞳孔受累等主要临床症状在不同病因亚组中的情况。结果537例患者中,男性251例,平均年龄(58.1±19.4)岁,双眼上睑下垂375例,单眼上睑下垂162例。病因分布:肌源性上睑下垂177例(33.0%),其中重症肌无力165例(30.7%);神经性上睑下垂90例(16.8%),主要为动眼神经麻痹86例(16.0%);假性上睑下垂233例(43.4%),其中睑痉挛197例(36.7%);病因不明37例(6.9%)。重症肌无力患者中,82.4%为双侧眼受累,95.8%存在晨轻暮重的波动症状,78.8%有双眼复视或眼球运动障碍,89.1%有不同程度额肌代偿,64.8%用力闭目后的埋睫征消失,57.8%有全身骨骼肌受累症状。睑痉挛患者无眼动障碍症状及主诉,且病程较长。38.9%偏侧面肌痉挛患者伴发作性眩晕和/或耳鸣。动眼神经麻痹患者通常伴有眼球运动障碍及疼痛,部分患者存在瞳孔受累,病程多较短。结论肌源性上睑下垂中重症肌无力最常见,假性上睑下垂以睑痉挛为主,动眼神经麻痹是神经源性上睑下垂的重要病因。不同病因的上睑下垂在临床特征上存在显著差异,需综合判断以优化个体化诊疗流程。

Objective To explore the distribution of etiology and clinical characteristics of patients with ptosis.Design Retrospective case series.Participants 537 patients presenting with ptosis from January to December 2023 in the Department of Neurology,Beijing Tongren Hospital.Methods Clinical data were collected for etiological classification and statistical analysis.Main Outcome Measures The average age of onset,disease duration,gender,bilateral involvement,and the presence of major clinical symptoms such as pain,diplopia,and pupillary involvement in different etiological subgroups.Results Among the 537 patients,251 were male with an average age of(58.1±19.4)years.Bilateral ptosis was observed in 375 cases,and unilateral ptosis in 162 cases.The etiology distribution was as follows:myogenic ptosis in 177 cases(33.0%),of which 165 cases(30.7%)were due to myasthenia gravis;neurogenic ptosis in 90 cases(16.8%),mainly due to oculomotor nerve paralysis in 86 cases(16.0%);pseudoptosis in 233 cases(43.4%),with eyelid spasm in 197 cases(36.7%);and undetermined causes in 37 cases(6.9%).Among patients with myasthenia gravis,82.4%had bilateral eye involvement,95.8%exhibited fluctuating symptoms of worsening in the evening,78.8%had diplopia or eye movement disorders,89.1%showed varying degrees of compensatory frontalis muscle activation,64.8%had disappearance of the burying eyelashes sign after forceful eyelid closure,and 57.8%had symptoms of systemic skeletal muscle involvement.Patients with eyelid spasm did not have eye movement disorders and typically had a longer disease course.38.9%of patients with unilateral facial spasm experienced paroxysmal vertigo and/or tinnitus.Patients with oculomotor nerve paralysis typically had eye movement disorders and pain,with some cases involving pupil impairment and generally a shorter disease duration.Conclusion Myasthenia gravis is the most common cause of myogenic ptosis,while eyelid spasm predominates in pseudoptosis,and oculomotor nerve paralysis is an important cause of neurogenic ptosis.Clinical features differ significantly among different etiologies of ptosis,necessitating comprehensive judgment to optimize individualized diagnostic and treatment processes.