以精神障碍为首次发病症状的克-雅病1例  

A case of Creutzfeldt-Jakob disease with a mental disorder as the first manifestation

作　　者：田音 何笑笑[2] 黄晓瀚 及晓 Tian Yin;He Xiaoxiao;Huang Xiaohan;Ji Xiao(Department of Psychiatric Emergency,Beijing Anding Hospital,Beijing 100088,China;Peking University Sixth Hospital,Beijing 100191,China;Beijing Wisprit Technology Co.,Ltd.,Beijing 100193,China)

机构地区：[1]首都医科大学附属北京安定医院急诊科,北京100088 [2]北京大学第六医院,北京100191 [3]北京智精灵科技有限公司,北京100193

出　　处：《中华精神科杂志》2023年第2期122-125,共4页Chinese Journal of Psychiatry

摘　　要：克罗伊茨费尔特-雅各布病(Creutzfeldt-Jakob disease,CJD)简称克-雅病,是由朊蛋白感染所致的中枢神经系统变性疾病,其病程短、进展迅速,病死率高达100%。其首次发病症状复杂多样,临床极易误诊误治。本例患者为57岁男性,以精神障碍为首次发病症状就诊,存在进行性认知功能下降,由紧张综合征逐步进展为无反应觉醒状态。其神经系统检查可见颈强直、肌张力增高,腱反射亢进、肢体运动性共济失调、踝阵挛、右侧病理征阳性。辅助检查脑脊液中14-3-3蛋白阳性,脑电图呈现“周期性三相波”;头颅磁共振DWI示大脑皮质呈典型“花边征”。结合患者临床特征,根据WHO诊断标准,考虑诊断为很可能的克-雅病。在我院进行抗抑郁、MECT等治疗无好转,转院至神经内科后给予改善循环、营养神经、脑保护等相关对症支持治疗。患者临床症状进行性加重,针对本病无特异有效治疗。本文对患者临床资料进行回顾并进行相关文献复习,旨在帮助提高精神科临床医师对本病的甄别能力。Creutzfeldt-Jakob disease(CJD),referred to as Creutzfeldt-Jakob disease,is a degenerative disease of the central nervous system caused by prion protein infection.The course of CJD is short,the disease progress is rapid,and the fatality rate of CJD is 100%.The initial symptoms of the disease are complex and diverse,therefore,it could be easily misdiagnosed and mistreated in clinical practice.The patient was a 57-year-old male who presented with mental disorder as the first symptom.He had progressive cognitive decline,and gradually progressed from catatonia to unresponsive arousal.The nervous system examination showed neck stiffness,increased muscle tone,tendon hyperreflexia,limb motor ataxia,ankle clonus,and positive pathological signs on the right side'of the body'.Auxiliary examination showed positive 14-3-3 protein in cerebrospinal fluid,and EEG showed"periodic three-phase waves";head MRI DWI showed typical"lace sign"of cerebral cortex.Combined with the clinical characteristics of the patient,according to the WHO diagnostic criteria,the diagnosis is likely to be Creutzfeldt-Jakob disease.The patient has received antidepressant,modified electroconvulsive therapy(MECT)and other treatments in the department of psychiatry,and after being transferred to the neurology department,he symptomatic and supportive treatment related to improving circulation,nourishing nerves,and protecting the brain.However,the clinical symptoms of the patients were progressively worsened,and there was no specific and effective treatment for this disease.This article reviewed the clinical data of patients and relevant literature,hoping to improve the identification of the disease among psychiatric clinicians.

关 键 词：克-亚综合征 精神障碍 运动不能性缄默 14-3-3蛋白质类 脑电描记术 

分 类 号：R742.9[医药卫生—神经病学与精神病学]