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目的 探讨散发性Creutzfeldt Jakob病(sCJD)的临床、病理及影像学特点。方法 回顾性分析 12例sCJD患者的临床表现、脑电图(EEG)、影像学特点及病理资料。结果 (1)本组男7例,女5例,平均发 病年龄49岁;3例以视觉缺失急性起病,9例以智能下降,精神、行为异常或共济失调亚急性起病;12例均有 痴呆、肌阵挛和锥体外系体征。(2)9例脑电图(EEG)表现典型的、1例表现不典型的三相波,(3)12例头颅 MRI检查,5例出现双侧基底节区T2加权像WI对称性高信号;8例同时行弥散加权(DWI)扫描,均表现为额 叶或/和枕叶DWI高信号,并有5例伴双侧基底节区对称性DWI高信号。(4)1例尸检及6例脑活检均具备 CJD病理特点。结论 sCJD在具备典型临床表现基础上,动态EEG及头颅MRIDWI扫描可为CJD的早期临 床诊断提供依据。
Objective To investigate the clinical, pathological and imaging features of sporadic Creutzfeldt Jakob disease (sCJD). Methods Retrospective analysis of 12 cases of sCJD patients with clinical manifestations, electroencephalogram (EEG), imaging features and pathological data. Results (1) The group of 7 males and 5 females, the average age of onset was 49 years old; 3 cases of acute onset of visual impairment, 9 cases of mental decline, mental, behavioral disorders or ataxia subacute onset; 12 cases All have dementia, myoclonus and extrapyramidal signs. (2) 9 cases of EEG showed typical, 1 case showed atypical three-phase wave, (3) 12 cases of brain MRI examination, 5 cases of bilateral basal ganglia T2 weighted WI symmetry high signal ; 8 patients underwent simultaneous DWI scans, both showed high DWI signal of frontal lobe and / or occipital lobe, and 5 patients had bilateral DWI hyperintense signal of basal ganglia. (4) 1 case of autopsy and 6 cases of brain biopsy have CJD pathological features. Conclusion Based on the typical clinical manifestations of sCJD, dynamic EEG and MRDWI scan can provide the basis for the early clinical diagnosis of CJD.