神经白塞氏病的临床诊断  

The clinical diagnosis of neuro-Beh et's disease

作　　者：王晔[1] 王德生[1] WANG Ye;WANG Desheng(Department of Neurology,The First Affiliated Hospital of Harbin Medical University,Harbin 150001,China)

机构地区：[1]哈尔滨医科大学附属第一医院神经内科,黑龙江哈尔滨150001

出　　处：《中风与神经疾病杂志》2023年第7期652-654,共3页Journal of Apoplexy and Nervous Diseases

摘　　要：目的分析神经白塞氏病的临床表现及影像学特征。方法采用回顾性研究方法分析51例患者的临床资料,51例患者均符合白塞氏病国际研究小组1990年提出的诊断标准,并有不同程度的神经系统受损表现。结果常见头痛、感觉障碍、运动障碍、脑膜刺激征、共济失调;阵发性抽搐、精神障碍、偏盲、失语症等少见。磁共振检查急性期常见基底节区或脑干片状高信号,半球白质、脑室周围散在高信号,呈圆形、线形或新月状。脑脊液典型改变可见淋巴细胞、总蛋白量升高。诱发电位改变不明显。结论神经白塞氏病一般急性起病,呈缓解与复发或持续进展病程。神经白塞氏病临床可见脑干综合征、脑卒中样类型、精神异常型和弥漫型等类型。目前诊断以临床为主,在白塞氏病基础上出现神经系统症状和体征即可诊断。治疗主要用激素、细胞毒素类药物及血浆置换方法。Objective To investigate the clinical manifestation and radiological features of neuro-Beh et's disease(NBD).Methods A retrospective analysis was performed for the clinical data of 51 NBD patients,all of whom met the diagnostic criteria for NBD proposed by International Study Group for Beh et's Disease in 1990 and had the manifestation of varying degrees of nervous system damage.Results Common symptoms included headache,sensory disturbance,dyskinesia,meningeal irritation sign,and ataxia,and rare symptoms included paroxysmal convulsion,mental disorders,hemianopsia,and aphasia.Magnetic resonance showed patchy hyperintensity in the basal ganglia or the brainstem and sporadic hyperintensity with a round,linear or crescent shape in white matter or the periventricular region in the acute stage.Typical changes of cerebrospinal fluid included the increases in lymphocytes and total protein content.There was no significant change in evoked potential.Conclusion NBD often has an acute onset and a disease course of remission and recurrence or persistent progression.The types of NBD include brainstem syndrome,stroke-like type,mental disorder,and diffuse type in clinical practice.At present,the diagnosis of NBD mainly relies on the clinical observation of the symptoms and signs of the nervous system on the basis of Beh et's disease,and the main treatment methods include hormones,cytotoxic drugs,and plasma exchange.

关 键 词：白塞氏病 神经白塞氏病 临床特征 皮质类固醇 免疫抑制剂 

分 类 号：R597[医药卫生—内科学]