以失用症为突出表现的克雅病一例

Apraxia of Creutzfeldt-Jakob disease: a case report

分析1例2020年8月在中国医学科学院北京协和医院神经科住院的克雅病患者的临床表现和检查结果,并对其进行全面的神经心理学评估和失用相关评估。该患者为65岁男性,隐匿起病,症状逐渐进展,临床表现为失用症、快速进展的痴呆和锥体外系症状,头颅磁共振成像提示双侧大脑半球"花边征",脑脊液14-3-3蛋白阳性,符合很可能克雅病的诊断标准,其失用症为典型的观念运动性失用。经复习文献,发现失用也可以是克雅病的主要神经心理损害表现,临床诊治中需要重视失用症的规范和正确评价、及时识别。

The clinical manifestations and examination results of a case of Creutzfeldt-Jakob disease(CJD)admitted to the Department of Neurology of Peking Union Medical College Hospital,Chinese Academy of Medical Sciences in August 2020 were analyzed,and a comprehensive neuropsychological assessment and assessment of apraxia were conducted.The neuropsychological characteristics of apraxia in CJD patient and the progress in the research and evaluation of apraxia were reviewed.The patient was a 65-year-old male with insidious onset and progressive symptoms,whose clinical manifestations were apraxia,rapidly progressing dementia,and extrapyramidal symptoms.The magnetic resonance imaging showed hyper-intense signal in diffusion weighted imaging in bilateral cerebral hemispheres,and 14-3-3 protein in cerebrospinal fluid was positive,which were consistent with the probable CJD diagnostic criteria.The patient exhibited prominent signs and symptoms of ideomotor apraxia.It has been reported in the literature that apraxia can also be the main neuropsychological manifestation of CJD.It is necessary to pay attention to the standard evaluation and timely identification of apraxia in clinical diagnosis.