进行性核上性麻痹的临床特点研究

发布时间：2018-11-22 10:04

【摘要】：目的探讨进行性核上性麻痹(progressive supranuclearpalsy,PSP)的临床表现、影像学表现、神经心理学量表评估结果、治疗及预后,以提高临床医师对该病的认识。方法收集近2年青岛大学医学院医学院、济宁市精神病防治院诊断的3例PSP患者相关病例资料。并在万方医学网及Pub Med上输入关键词“进行性核上性麻痹”或“脑磁共振成像”,检索PSP病例报道的相关文献。统计PSP患者的临床表现及脑磁共振特点,并进行分析。结果病例中男1例,女2例。2例以步态不稳为首发症状,1例以帕金森症状为首发症状。3例患者皆出现核上性眼肌麻痹、假性延髓麻痹、帕金森症状,2例患者出现认知和行为障碍。神经心理学测验中,2例患者出现中度认知功能障碍。脑MR结果中,1例可见中脑被盖部萎缩。复习23例PSP文献,其中男性18例,女性5例,起病年龄在47-71岁。合并统计男女比例约为2.7:1,其中55岁以后起病者约占96%。合并统计首发症状:步态不稳12例(52%),帕金森症状6例(26%),认知障碍2例(8%),眼部症状3例(11%)。病程中23例出现核上性眼肌麻痹,19例出现假性性延髓麻痹(82%),19例出现帕金森症状(82%),14例出现认知和行为障碍(60%)。出现核上性眼肌麻痹患者中,13例出现核上性向上向下扫视缓慢(68%),6例出现向下扫视缓慢(31%),3例出现向上扫视缓慢(15%),4例出现凝视麻痹(21%)。假性球麻痹2年内出现的比例为25%,2年后出现的比例为75%。出现假性延髓麻痹患者中,6例先于凝视麻痹出现(50%),3例晚于凝视麻痹出现(25%),3例与凝视麻痹同时出现(25%)。出现帕金森征候群的患者中,17例出现轴性肌张力障碍(89%),15例出现四肢肌张力障碍(78%)。出现认知和行为障碍的患者中,9例出现记忆力减退(62%),7例出现计算力下降(50%),4例出现情感淡漠(28%)。脑MR结果中,12例出现中脑被盖部萎缩(63%),8例出现“蜂鸟征”(42%)。结论进行性核上性麻痹是中枢神经系统变性疾病,患病率较低,多见于中老年,男性多于女性。进行性核上性麻痹首发症状差异性较大,症状部分叠加,容易误诊。步态不稳、平衡障碍是最常见的首发症状,有助于进行性核上性麻痹的诊断。核上性眼肌麻痹是PSP的核心症状,多出现较晚,对早期诊断帮助不大。中脑萎缩、“蜂鸟征”为脑MR特征性表现,典型脑MR多在疾病中后期出现,对早期诊断帮助不大。
[Abstract]:Objective to investigate the clinical manifestations, imaging manifestations, neuropsychological evaluation results, treatment and prognosis of progressive supranuclear palsy (progressive supranuclearpalsy,PSP) in order to improve the understanding of the disease among clinicians. Methods the data of 3 cases of PSP diagnosed by the Medical College of Qingdao University and Jining Psychiatric Hospital in recent 2 years were collected. The key words "progressive supranuclear paralysis" or "cerebral magnetic resonance imaging" were inputted on Wanfang Medical Network and Pub Med to search the relevant literatures reported on PSP cases. The clinical manifestations and brain MRI features of PSP patients were analyzed. Results there were 1 male and 2 female, 2 cases with gait instability, 1 case with Parkinson's disease, 3 cases with supranuclear ophthalmoplegia, pseudobulbar palsy, Parkinson's disease. Cognitive and behavioral disorders were found in 2 patients. In neuropsychological tests, moderate cognitive impairment was found in 2 patients. According to the results of MR, atrophy of the tegmental part was found in 1 case. 23 cases of PSP were reviewed, including 18 males and 5 females. The onset age was 47-71 years. According to the combined statistics, the ratio of male to female is about 2.7: 1, of which 96 are from the age of 55. The first symptoms were gait instability in 12 cases (52%), Parkinson's symptom in 6 cases (26%), cognitive impairment in 2 cases (8%) and ocular symptoms in 3 cases (11%). In the course of the disease, 23 cases had supranuclear ophthalmoplegia, 19 cases had pseudobulbar palsy (82%), 19 cases had Parkinson's symptoms (82%), and 14 cases had cognitive and behavioral disorders (60%). Among the patients with supra-nuclear ophthalmoplegia, 13 (68%) had a slow upward downward scan, 6 (31%) had a slow downward scan, 3 (15%) had a slow upward scan, and 4 (21%) had staring paralysis. The proportion of pseudobulbar paralysis in 2 years was 25 and that after 2 years was 75. Among the patients with pseudobulbar palsy, 6 cases appeared earlier than staring paralysis (50%), 3 cases appeared later than staring paralysis (25%), 3 cases appeared at the same time as staring paralysis (25%). Among the patients with Parkinson's syndrome group, 17 had axial dystonia (89%) and 15 had limb dystonia (78%). Among the patients with cognitive and behavioral disorders, 9 had memory impairment (62%), 7 had decreased computational power (50%) and 4 had emotional apathy (28%). In brain MR, 12 cases (63%) had mesencephalic tegmental atrophy and 8 cases (42%) had hummingbird sign. Conclusion progressive supranuclear paralysis is a degenerative disease of the central nervous system. The initial symptoms of progressive supranuclear paralysis are different, and the symptoms are partially superimposed, which are easily misdiagnosed. Gait instability and balance disorder are the most common initial symptoms, and contribute to the diagnosis of progressive supranuclear paralysis. Supranuclear ophthalmoplegia is the core symptom of PSP. Midbrain atrophy, "hummingbird sign" is the characteristic manifestation of brain MR, typical brain MR appears in the middle and late stage of disease, so it is of little help to early diagnosis.
【学位授予单位】：青岛大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R742.5

【参考文献】