伴有Holmes—Adie综合征的重症肌无力一例

Myasthenia gravis with Holmes-Adie syndrome:a case report

Holmes-Adie综合征(HAS)是以强直瞳孔、腱反射消失为主要特点的临床综合征,多为特发性,也可见于颅内病变、颅脑外伤、感染、副肿瘤综合征等,但少见与重症肌无力(MG)合并存在的报道。我们报道1例MG合并HAS的患者,其临床表现为波动性四肢无力伴呼吸困难,伴右上睑下垂,双侧瞳孔不等大,左侧瞳孔3mm,右侧瞳孔2mm,左侧瞳孔直接、间接对光反射迟钝,右侧瞳孔对光反射灵敏。用毛果芸香碱稀释滴眼液(O.0625%)滴双眼,10min后左眼瞳孔缩小至2mm,右侧瞳孔仍为2mm。胸部CT检查发现胸腺瘤。经胸腺切除、糖皮质激素、丙种球蛋白及他克莫司治疗后,患者右上睑及四肢无力好转,但左侧瞳孔直径及对光反射无变化。结合患者的症状、体征及辅助检查提示MG合并HAS。分析了MG合并HAS的临床特点,以提高临床对该病的认识,避免误诊及漏诊。

Holmes-Adie syndrome (HAS)is a clinical syndrome mainly characterized by tonic pupil and disappearance of tendon reflex.It is mostly idiopathic and can also be seen in cerebral diseases,such as trauma,infection and tumors.However,it is rarely reported to be accompanied with myasthenia gravis (MG). We report a case of MG and HAS,whose clinical manifestations were fluctuation of limb weakness,breathing difficulties,right ptosis.Her pupils were unequal:the left pupil was 3mm,the right pupil was 2mm,direct and indirect light reflex was slow in left pupil,and right pupil was sensitive to light reflex.The left eye pupil shrank to 2mm after dripped pilocarpine diluent for 10 minutes,while the right pupil was still 2mm.Chest CT examination revealed thymoma.After treatment with thymectomy,glucocorticoid,immunoglobulins and tacrolimus,her symptoms of MG were improved,but the left pupil diameter and light reflex were not changed.Combined with the patient's symptoms,physical signs and examinations,this patient was diagnosed as MG accompanied with HAS.