Objective To investigate the diagnosis of the disorders with Parkinson-like symptoms (PLS). Methods The clinical data of 78 patients with PLS admitted in our hospital during 2008-01 to 2010-12 were analyzed retrospectively, including medical history, physical examinations, clinical manifestations, blood biochemistry, parathyroxin, ceruloplasmin, levodopa (L-dopa ) test, brain CT scan and MR imaging. The final clinical diagnosis was made according to the diagnostic criteria of PLS related disorders. Results For clinical manifestations, among the 78 patients, there were 74 cases with hypermyotonia, 67 cases with tremor, 65 cases with bradykinesia, 56 cases with abnormal postural reflex, 52 cases with abnormal gait, 5 cases with orthostatic hypotension, 4 cases with vertical gaze palsy, 2 cases with positive K-F corneal ring, 2 cases with visual hallucination, and 1 case with unilateral limb ignorance due to apraxia. The blood biochemistry tests showed abnormal liver function in 5 cases, reduced serum ceruloplasmin level in 2 cases and reduced serum parathyroxin level in 1 case. The CT scan revealed low density in basal ganglia in 59 cases, cerebral atrophy in 35 cases, encephalomalacia in 10 cases, and calcifications existing in the basal ganglia and cerebellar hemisphere in 2 cases. The MR imaging showed abnormal signals in the basal ganglia, the brain stem and the brain whiterfnatter in 65 cases, atrophy in pons and cerebellum in 5 cases, "putamen fissure sign" in 4 cases, "cross sign" in pons in 3 cases, cerebral "hummingbirds sign" in 1 case, high intensity in pallidum on T2 in 2 cases. 31 cases showed poor response to L-dopa treatment. There were 36 cases diagnosed clinically as PD, 28 cases diagnosed as parkinsonian syndrome (PS), 4 cases diagnosed as multiple system atrophy (MSA), 4 cases diagnosed as progressive supranuclear palsy (PSP), 2 cases diagnosed as Lewy body (DLB), 2 cases diagnosed as Wilson disease (WD), 1 case diagnosed as basal ganglia calcification (BGC) and 1 case diagnosed as corticobasal degeneration (CBD).Among those 28 cases diagnosed as PS, 16 cases were induced by cerebral vascular diseases, 5 cases by poisoning (3 cases by carbon monoxide and the other 2 cases by pesticide ), 3 cases by brain injury, 2 cases by encephalitis, and 2 cases by drugs. Conclusions Reliable medical history collection, characteristic signs finding, special radiographic changes, serum ceruloplasmin and parathyroxin abnormalities, and poor response to L-dopa treatment contribute greatly to the diagnosis and differential diagnosis of PLS-related disorders.%目的 探讨具有帕金森病样症状(PLS)相关疾病的临床诊断.方法 回顾性分析作者医院2008-01-2010-12住院的78例具有PLS患者的临床资料,包括病史、体格检查、临床表现、血液学检查[包括血生化(肝功能、肾功能、心肌酶谱、电解质、血糖、血脂全套)、血甲状旁腺素、血铜蓝蛋白]、左旋多巴(L-dopa)试验、头CT 和头MRI检查等,并依据PLS相关疾病诊断标准进行临床诊断.结果 本组78例患者中,有肌张力增高74例、震颤67例、运动迟缓65例、姿势反射异常56例、步态异常52例、直立性低血压5例、垂直性凝视麻痹4例、角膜K-F环阳性和视幻觉各2例、一侧肢体忽略并失用1例；有肝功能异常5例、血清铜蓝蛋白降低2例和血清甲状旁腺素降低1例；头CT检出基底节区低密度影59例,脑萎缩35例,脑叶软化灶10例,基底节区和小脑半球钙化灶2例；头MRI检出基底节区、脑干、大脑白质异常信号65例,脑桥和小脑萎缩5例,“壳核裂隙征”4例,脑桥“十字征”3例,双侧苍白球T2高信号2例,脑干“蜂鸟征”1例.L-dopa试验反应不良31例.临床诊断为帕金森病(PD)36例,帕金森综合征(PS)28例[其中血管性16例、中毒后5例(一氧化碳中毒后3例,农药中毒后2例)、脑外伤后3例、感染后和药物性各2例],多系统萎缩(MSA)和进行性核上性麻痹(PSP)各4例,路易体痴呆(DLB)和Wilson病(WD)各2例,基底节钙化症(BGC)和皮质基底节变性(CBD)各1例.结论 详细可靠的病史、特异性体征、特征性影像学改变、铜蓝蛋白和甲状旁腺素降低及L-dopa试验反应不良,对于PLS患者相关疾病的诊断和鉴别诊断具有重要意义.
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