进行性核上性麻痹临床特征及误诊分析  

Clinical Characteristics and Misdiagnosed Analysis of Progressive Supranuclear Palsy

作　　者：徐玉 沈静萍 谷建芳 陈立平 闫佳兰 高利涛 王丽 智建霞 徐秀红 王雪笠 XU Yu;SHEN Jing-ping;GU Jian-fang;CHEN Li-ping;YAN Jia-lan;GAO Li-tao;WANG Li;ZHI Jian-xia;XU Xiu-hong;WANG Xue-li(Department of Neurology,the 980 Hospital of PLA Joint Logistics Support Forces,Shijiazhuang 050082,China;Department of Nutrition,Beidaihe Rehabilitation and Recuperation Center of PLA Joint Logistics Support Forces,Qinhuangdao,Hebei 066100,China)

机构地区：[1]解放军联勤保障部队第九八〇医院神经内科,石家庄050082 [2]解放军联勤保障部队北戴河康复疗养中心营养科,河北秦皇岛066100

出　　处：《临床误诊误治》2021年第11期12-16,共5页Clinical Misdiagnosis & Mistherapy

基　　金：河北省卫生厅医学科学研究重点课题计划(20130325)。

摘　　要：目的探讨进行性核上性麻痹(PSP)的临床特征及误诊原因、防范措施。方法对2015年1月—2019年12月收治的曾误诊的PSP 15例的临床资料进行回顾性分析。结果本组误诊率为57.69%,以步态不稳和运动迟缓起病10例,以静止性震颤和垂直性核上性眼肌麻痹起病各2例,以认知功能障碍起病1例。15例均出现垂直性核上性眼肌麻痹,11例出现帕金森病症状。曾误诊为帕金森病11例,动眼神经麻痹2例,路易体痴呆和多系统萎缩各1例。误诊时间0.5~5.0年。本组均依据临床表现、头颅MRI检查结合相关诊断标准确诊PSP,予相应治疗,3例运动障碍稍有改善,但1个月后病情再次进展;12例症状无明显改善。结论PSP起病隐匿,临床表现复杂多样,早期缺乏相关诊断指标,极易误诊。临床医生应提高对其认识,详细询问病史和仔细查体,重视头颅影像学检查,并长期随访,以减少或避免该病误诊误治。Objective To investigate clinical characteristics,misdiagnosed causes and preventive measures of progressive supranuclear palsy(PSP).Methods Clinical data of 15 patients with PSP admitted between January 2015 and December 2019 who had been misdiagnosed was retrospectively analyzed.Results The misdiagnosis rate was 57.69%.The initial symptoms were gait instability and bradykinesia in 10 patients,static tremor and vertical ophthalmoplegia in 2 patients respectively,and cognitive dysfunction in 1 patient.All the 15 patients had vertical supranuclear ophthalmoplegia,and 11 patients had Parkinson's syndrome.Among the 15 patients in this study,Parkinson's disease(PD)was misdiagnosed in 11 patients,oculomotor paralysis in 2 patients,dementia with Lewy Bodies(DLB)in 1 patient,and multiple system atrophy(MSA)in 1 patient.The misdiagnosed duration ranged from 6 months to 5.0 years.All patients in this group were confirmed PSP based on clinical manifestations,cranial magnetic resonance imaging(MRI)and relevant diagnostic criteria.After corresponding treatments,dyskinesia conditions were improved slightly in 3 patients,but the conditions progressed again one month later.Symptoms of the other 12 patients were not obviously improved.Conclusion The onset of PSP is hidden,and its clinical manifestations are complicated and diverse.It is more likely to be misdiagnosed because of the lack of relevant diagnostic indicators during the early period.Clinicians should strengthen the understanding of PSP,consult the disease history and perform physical examination carefully,pay attentions to cranial imaging examination and perform long-term follow-up so as to reduce or avoid misdiagnosis and missed diagnosis in clinical practice.

关 键 词：核上麻痹 进行性 误诊 帕金森病 动眼神经疾病 痴呆 

分 类 号：R742.89[医药卫生—神经病学与精神病学]