103例国人进行性核上性麻痹患者的临床特征

Clinical characteristic of 103 Chinese patients with progressive supranuclear palsy

目的 为全面了解国人进行性核上性麻痹(psp)患者的临床特征.方法 在中国知网和维普数据网上检索1980年～2009年有关psp的报道并分析其临床特征.结果 共56个研究103例患者被搜集.所有病例均为散发.其中,34.3%的患者其发病症状为肢体强直和运动减少,95.1%的患者为垂直性核上性眼肌麻痹.辅助检查方面,70.5%的患者提示脑电图结果异常;21.4%的患者呈现脑脊液轻度异常;37例患者行ct检查,5.4%提示中脑萎缩;55例患者行mri检查,16.4%显示中脑萎缩.11例患者的简易智能状态检查表(mmse)评分均<23分.共有9例患者进行了病理学的检查.所有患者均未进行基因检查.40例患者在发病初期被误诊,分别有58%和18%的患者被误诊为帕金森病(pd)和帕金森综合征(pds).共有44例患者进行左旋多巴、金刚烷胺和苯海索治疗,仅有7例患者在运动方面有改善,没有患者在眼球运动上有改善.结论 psp为散发性疾病,通常是中老年发病.性别在发病年龄上无差异.女性从发病到就诊的时间短于男性.肢体的强直-少动及早期的姿势不稳是发病时最常见的两个症状.伴随着疾病的发展,垂直性核上性眼肌麻痹及肢体的强直-少动有更高的出现率.mri是用于psp诊断的一项常见的、敏感的及无创性检查.psp患者在发病早期通常被误诊为pd或pds,使用抗帕金森治疗通常效果较差.

Objective To thoroughly explore the clinical characteristic of progressive supranuclear palsy(PSP) in Chinese patients.Methods The reports about PSP were researched on China National Knowledge Internet(CNKI) and VIP webs from 1980 to 2009 and its clinical features were analyzed.Results A total of 56 studies involving 103 patients were collected and all the patients were sporadic.Among them,the onset of symptoms were akinetic-rigid and less exercise features accounted for 34.3% and vertical supranuclear gaze palsy was reported in 95.1% cases.As for the auxiliary examination,70.5% of patients with abnormal EEG and 21.4% of patients showed mild abnormalities in cerebrospinal fluid.Thirty seven patients underwent CT examination and 5.4% of them showed brain atrophy.Firty five patients underwent MRI and 16.4% of them showed brain atrophy.The Mini Mental State Examination(MMSE) scores in 11 patients were less than 23 points.Total 9 patients had the pathological examination.No patient had genetic examination.Forty patients were misdiagnosed in the early stages,there were 58% and 18% of them misdiagnosed as Parkinson's disease(PD) and Parkinson's syndrome(PDS) respectively.Total of 44 patients were treated with levodopa,amantadine and trihexyphenidyl,and only 7 patients were improved in movement.There was no patient improved in eye movement.Conclusion PSP is a sporadic disease and usually attacked on middle-aged people.There was no gender difference in age.The course from onset to diagnosis of females was shorter than male's.Akinetic rigid-less exercise and the early onset of postural instability are the two most common symptoms.With the development of the disease,vertical supranuclear ophthalmoplegia and akinetic rigid-less exercise have a higher occurrence rate.MRI is a common,sensitive and noninvasive diagnosis for PSP.Patients with early onset of PSP is often misdiagnosed as PD or PDS,using of anti-Parkinson's treatment is usually less effective.