非典型帕金森病相关疾病的临床诊断思路

Clinical Diagnosis of the Diseases with Atypical Parkinsonian Disorders

目的探讨非典型帕金森病(APD)相关疾病的临床诊断思路。方法通过回顾性分析笔者医院2006～2011年收治的48例APD相关疾病患者的临床资料包括病史、体格检查、临床表现、实验室检查、影像学检查、认知功能评估、左旋多巴(L-dopa)试验结果,复习文献,总结分析临床资料特征,并依据APD相关疾病的诊断标准作出临床诊断。结果 48例患者中,肌张力增高42例、震颤40例、运动迟缓39例、姿势反射异常32例、步态异常29例、直立性低血压13例、腱反射亢进11例、认知功能障碍11例、垂直性凝视麻痹和球麻痹各10例、共济失调和病理征阳性各8例、角膜K-F环7例、视幻觉5例、一侧肢体忽略并失用3例;肝功能异常9例、血小板减少8例、血清铜蓝蛋白降低7例、凝血功能异常5例;头颅CT:基底核区低密度影19例,脑室扩大17例,脑萎缩14例;头颅MRI:基底节区异常信号30例,脑室扩大20例,弥漫性脑萎缩18例,脑干和小脑萎缩15例,海马萎缩7例,双侧苍白球T2高信号4例。壳核"裂隙征"8例,脑桥"十字征"7例,中脑"蜂鸟征"6例;L-dopa试验反应不良46例;蒙特利尔认知评估量表(MoCA)总分<26分23例。出院诊断:多系统萎缩(MSA)18例、进行性核上性麻痹(PSP)10例、Wilson病(WD)7例、路易体痴呆(DLB)6例、伴帕金森综合征(PS)的阿尔茨海默病(AD)5例和皮质基底核变性(CBD)2例。结论 APD相关疾病间,虽然临床表现有许多相似之处,但临床特征、诊断标准及治疗转归各不相同,正确区分十分必要。

Objective To investigate the ideas of the clinical diagnosis of the diseases with atypical parkinsonian disorders（APD）. Methods The clinical data of 48 patients with APD admitted to our hospital during 2006 to 2011 were analyzed retrospectively, including medical history, physical examinations, clinical manifestations, laboratory tests , imagings , cognitive assessment tests and levodopa （ L - dopa ） tests. The final clinical diagnosis was made according to the diagnostic criterias of APD related diseases. Results For clinical manifestations, among the 48 patients, there were 42 cases with hypermytoma, 40 cases with tremor, 39 cases with bradykinesia, 32 cases with abnormal postural reflex, 29 cases with abnormal gait, 13 cases with orthostatic hypotension, 12 cases with tendon hyperreflexia , 11 cases with cognitive impairment, 10 cases with vertical gaze palsy, 10 cases with bulbar palsy, 8 cases with ataxia, 8 cases with positive pathologic reflexes, 7 cases with K - F corneal ring, 5 cases with visual hallucination and 3 cases with unilateral limb ignorance duo to apraxia. Laboratory tests showed abnormal liver function in 9 cases , thrombocytopenia in 8 cases ,reduced serum cernloplasmin levels in 7 cases and abnormal coagulation monitoring in 5 cases. The CT scan revealed low density in basal ganglia in 19 cases, ventricular dilata- tion in 17 cases and cerebral atrophy in 14 cases. The MR imaging showed abnormal signals in the basal ganglia in 30 cases, ventricle expand in 20 cases, diffusive cerebral atrophy in 18 cases, atrophy in pons and cerebellum in 15 cases, hippocampal atrophy in 7 cases, high intensity in pallidum on T2 in 4 cases, ＂putamen fissure sign＂ in 8 cases, ＂cross sign＂ in pons in 7 cases and ＂hummingbird sign＂ in midbrain in 6 cases. Fourty - six cases showed poor response to L - dopa treatment. Montreal Cognitive Assessment Scale （MoCA） total scores 〈 26 in 23 cases. There were 18 cases diagnosed clinically as multiple system atrophy（MSA） , 10 cases diagnosed as progres- sive supranuclear palsy （PSP） , 7 cases diagnosed as Wilson disease（WD） , 6 cases diagnosed as dementia with Lewy bodies （DLB） , 5 cases diagnosed as Alzheimer＇ s disease （AD） with Parkinsonism syndrome （PS） and 2 cases diagnosed as corticobasal degeneration （ CBD）. Conclusion Although APD related diseases have some similarity in clinical manifestations, but their chnical characteristics, di- agnostic criterias and prognosis are different, so it＇ s very necessary to distinguish them.