作者：梁肖迪，周润津，曾婧纯，皮立宏，林兴栋，肖璐

单位：

1.510405广东省广州市，广州中医药大学第一附属医院医技科 2.510006广东省广州市，广州中医药大学针灸康复临床医学院 3.510405广东省广州市，广州中医药大学第一附属医院针灸科 4.510405广东省广州市，广州中医药大学第一附属医院脑病科 5.510405广东省广州市，广州中医药大学第三附属医院 6.341005江西省赣州市肿瘤医院中西医结合科 通信作者：林兴栋，E-mail：linxdlili@126.com

Units：

1.Medical Technology Department, the First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou510405, China 2.Clinical Medical College of Acupuncture & Moxibustion and Rehabilitation, Guangzhou University of Chinese Medicine,Guangzhou 510006, China 3.Department of Acupuncture, the First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405,China 4.Encephalopathy Department, the First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405,China 5.The Third Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405, China 6.Integrated TCM & Western Medicine Department, Ganzhou Cancer Hospital, Ganzhou 341005, China Corresponding author: LIN Xingdong, E-mail: linxdlili@126.com

关键词：

进行性核上性麻痹; 神经退行性病变; 临床特征;

Keywords：

Progressive supranuclear palsy; Neurodegenerative diseas; Clinical characteristics

CLC：

DOI：

10.12114/j.issn.1008-5971.2022.00.125

Funds：

广东省自然科学基金面上项目（2020A1515010392）；广东省中医药局科研项目基金（20162071）

摘要：

目的 分析进行性核上性麻痹（PSP）患者的临床特征及诊治情况。方法 计算机检索中国知网、维普网、万方数据知识服务平台、中国生物医学文献数据库、PubMed、Embase中关于PSP患者的个案报道及病例分析，检索时间为2010—2019年。由两位研究者独立阅读文献并提取信息，包括患者的临床特征、辅助检查、诊断和治疗、随访情况。结果 最终纳入58篇文献，包含167例患者。166例患者男女比例为2.13∶1，平均发病年龄为（64.5±9.0）岁；119例患者有发病至确诊时间，平均（3.0±1.7）年；126例患者有发病症状及临床表现，主要发病症状为肢体强直-少动55例（占43.6%）、姿势不稳46例（占36.5%），主要临床表现为假性球麻痹92例（占73.0%）、姿势不稳83例（占65.9%）、肢体强直-少动81例（占64.3%）、垂直性核上性眼肌麻痹65例（占51.6%）。124例患者行颅脑MRI检查，其中57例（占46.0%）显示脑实质萎缩（43例中脑萎缩）；85例患者有简易精神状态检查量表评分，平均为（19.3±5.7）分。167例患者中11例可疑，52例拟诊，104例确诊；42例（占25.1%）患者误诊。74例患者有多巴丝肼片治疗史，其中27例治疗无效、39例好转或稍有好转、2例症状加重。41例患者有随访结果，其中3例好转、7例病情稳定、10例病情加重、2例病情恶化、19例死亡。结论 近10年我国PSP患者仍以散发为主，男性多于女性，以老年人为主，主要发病症状为肢体强直-少动、姿势不稳，主要临床表现为假性球麻痹、姿势不稳、肢体强直-少动及垂直性核上性眼肌麻痹，颅脑MRI是诊断PSP的主要影像学方法，其治疗仍以症状靶向治疗为主。

Abstract：

【Abstract】　Objective To analyze the clinical characteristics, diagnosis and treatment of patients with progressivesupranuclear palsy (PSP) . Methods The case reports and case analysis of PSP patients in CNKI, VIP, Wanfang Data,China biomedical literature database, PubMed and Embase were searched by computer from 2010 to 2019. Two researchersindependently read the literature and extracted information, including clinical characteristics, auxiliary examination, diagnosisand treatment, and follow-up. Results Finally, 58 literature were included, including 167 patients. The male to female ratioof 166 patients was 2.13∶1, and the average age of onset was (64.5±9.0) years old; the average time from onset to diagnosis of119 patients was (3.0±1.7) years; 126 patients had symptoms and clinical manifestations. The main symptoms were rigidityless movent in 55 cases (43.6%) , postural instability in 46 cases (36.5%) ; the main clinical manifestations were pseudobulbarparalysis in 92 cases (73.0%) , postural instability in 83 cases (65.9%) , rigidity-less movent in 81 cases (64.3%) , and verticalsupranuclear gaze palsy in 65 cases (51.6%) . One hundred and twenty-four patients underwent brain MRI, of which 57 cases(46.0%) showed brain parenchymal atrophy (43 cases of midbrain atrophy) ; 85 patients had the score of Simple Intelligence StateExamination Scale, with an average of (19.3±5.7) . Among 167 patients, 11 were suspicious, 52 were suspected and 104 wereconfirmed; and 42 cases (25.1%) were misdiagnosed. Seventy-four patients had a history of treatment with dopashydrazine tablets,of which 27 cases were ineffective, 39 cases were improved or slightly improved, and 2 cases had aggravated symptoms. Fourtyone patients had follow-up results, of which 3 cases were improved, 7 cases were stable, 10 cases were aggravated, 2 cases wereworsened and 19 cases died. Conclusion In recent 10 years, PSP patients in China are still sporadic, more men than women,mainly the elderly. The main symptoms are rigidity-less movent and postural instability. The main clinical manifestations arepseudobulbar paralysis, postural instability, rigidity-less movent and vertical supranuclear gaze palsy. Brain MRI is the mainimaging means for the diagnosis of PSP, and its treatment is still symptom targeted therapy.

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