原发性干燥综合征合并帕金森综合征五例临床分析

Clinical analysis of five patients with primary Sjögren's syndrome and Parkinson's syndrome

目的探讨原发性干燥综合征(primary Sjögren's syndrome,pSS)合并帕金森综合征患者的临床特点及可能的发病机制。方法回顾性分析2016年6月至2019年7月首都医科大学宣武医院诊治的5例pSS合并帕金森综合征患者的临床表现、实验室检查、影像学特点及药物治疗过程资料。结果4例女性,1例男性,平均发病年龄65岁;两例为单侧受累,表现为震颤及强直,3例为双侧受累,表现为对称的强直及步态不稳等症状,1例帕金森综合征症状出现于pSS症状之前,3例出现于pSS之后,1例几乎同时出现;3例头颅MRI检查显示未见明显白质脱髓鞘病变,2例存在脑白质病变,其中1例可见小脑、脑干萎缩,1例行FDG-PET/CT及AV-133-PET/CT检查显示代谢改变与帕金森病相同,1例存在体位性低血压,2例膀胱残余尿量异常,2例最终诊断可能合并多系统萎缩。结论pSS合并帕金森综合征多见于女性,临床表现多样,可单侧受累也可双侧,部分患者颅脑MRI检查可发现脑白质病变,部分患者对美多芭反应较敏感,部分患者PET/CT检查结果与原发性帕金森病相同,推测pSS可能通过免疫机制增加帕金森综合征的发病风险。

Objective To investigate the clinical characteristics and possible pathogenesis of primary Sjögren's syndrome(pSS)accompanying with Parkinsonism syndrome.Methods The clinical manifestations,laboratory examinations,imaging characteristics and drug treatments of 5 patients with pSS accompanying with Parkinsonism syndrome treated in Xuanwu Hospital,Capital Medical University from June 2016 to July 2019 were analyzed retrospectively.Results Four female and one male patients with an average age of 65 years were included.There were two cases with unilateral involvement,characterized by tremors and rigidity;three cases with bilateral involvement,characterized by symmetrical symptoms such as rigidity and walking instability.One cases had Parkinsonism syndrome onset before the appearance of pSS,Three had Parkinsonism syndrome later,and one cases had the two disorders almost at the same time.Three cases had normal head MRI,two cases had white matter lesions,one of which showed cerebellar and brainstem atrophy.One patient underwent FDG-PET/CT and AV-133-PET/CT,and the metabolic changes were consistent with Parkinson's disease.One case had orthostatic hypotension,and two cases had abnormal post-void residual volume,the final diagnosis of these two cases might be Sjogren'syndrome accompanying with multiple system atrophy.Conclusions pSS accompanying with Parkinsonism syndrome is more common in women.The clinical manifestation varies;it can be unilateral or bilateral;cerebral MRI can be normal or with cerebral white matter lesions.Some patients are sensitive to levodopa/benserazide,and the PET/CT is the same as Parkinson's disease.It is speculated that pSS may increase the risk of Parkinsonism syndrome through immune mechanisms.