【摘要】：目的探討進(jìn)行性核上性麻痹(progressive supranuclearpalsy,PSP)的臨床表現(xiàn)、影像學(xué)表現(xiàn)、神經(jīng)心理學(xué)量表評估結(jié)果、治療及預(yù)后,以提高臨床醫(yī)師對該病的認(rèn)識。方法收集近2年青島大學(xué)醫(yī)學(xué)院醫(yī)學(xué)院、濟(jì)寧市精神病防治院診斷的3例PSP患者相關(guān)病例資料。并在萬方醫(yī)學(xué)網(wǎng)及Pub Med上輸入關(guān)鍵詞“進(jìn)行性核上性麻痹”或“腦磁共振成像”,檢索PSP病例報道的相關(guān)文獻(xiàn)。統(tǒng)計PSP患者的臨床表現(xiàn)及腦磁共振特點(diǎn),并進(jìn)行分析。結(jié)果病例中男1例,女2例。2例以步態(tài)不穩(wěn)為首發(fā)癥狀,1例以帕金森癥狀為首發(fā)癥狀。3例患者皆出現(xiàn)核上性眼肌麻痹、假性延髓麻痹、帕金森癥狀,2例患者出現(xiàn)認(rèn)知和行為障礙。神經(jīng)心理學(xué)測驗中,2例患者出現(xiàn)中度認(rèn)知功能障礙。腦MR結(jié)果中,1例可見中腦被蓋部萎縮。復(fù)習(xí)23例PSP文獻(xiàn),其中男性18例,女性5例,起病年齡在47-71歲。合并統(tǒng)計男女比例約為2.7:1,其中55歲以后起病者約占96%。合并統(tǒng)計首發(fā)癥狀:步態(tài)不穩(wěn)12例(52%),帕金森癥狀6例(26%),認(rèn)知障礙2例(8%),眼部癥狀3例(11%)。病程中23例出現(xiàn)核上性眼肌麻痹,19例出現(xiàn)假性性延髓麻痹(82%),19例出現(xiàn)帕金森癥狀(82%),14例出現(xiàn)認(rèn)知和行為障礙(60%)。出現(xiàn)核上性眼肌麻痹患者中,13例出現(xiàn)核上性向上向下掃視緩慢(68%),6例出現(xiàn)向下掃視緩慢(31%),3例出現(xiàn)向上掃視緩慢(15%),4例出現(xiàn)凝視麻痹(21%)。假性球麻痹2年內(nèi)出現(xiàn)的比例為25%,2年后出現(xiàn)的比例為75%。出現(xiàn)假性延髓麻痹患者中,6例先于凝視麻痹出現(xiàn)(50%),3例晚于凝視麻痹出現(xiàn)(25%),3例與凝視麻痹同時出現(xiàn)(25%)。出現(xiàn)帕金森征候群的患者中,17例出現(xiàn)軸性肌張力障礙(89%),15例出現(xiàn)四肢肌張力障礙(78%)。出現(xiàn)認(rèn)知和行為障礙的患者中,9例出現(xiàn)記憶力減退(62%),7例出現(xiàn)計算力下降(50%),4例出現(xiàn)情感淡漠(28%)。腦MR結(jié)果中,12例出現(xiàn)中腦被蓋部萎縮(63%),8例出現(xiàn)“蜂鳥征”(42%)。結(jié)論進(jìn)行性核上性麻痹是中樞神經(jīng)系統(tǒng)變性疾病,患病率較低,多見于中老年,男性多于女性。進(jìn)行性核上性麻痹首發(fā)癥狀差異性較大,癥狀部分疊加,容易誤診。步態(tài)不穩(wěn)、平衡障礙是最常見的首發(fā)癥狀,有助于進(jìn)行性核上性麻痹的診斷。核上性眼肌麻痹是PSP的核心癥狀,多出現(xiàn)較晚,對早期診斷幫助不大。中腦萎縮、“蜂鳥征”為腦MR特征性表現(xiàn),典型腦MR多在疾病中后期出現(xiàn),對早期診斷幫助不大。
[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.
【學(xué)位授予單位】：青島大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R742.5

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