摘要：

目的: 回顾1例新型冠状病毒感染后以Balint综合征为首发表现的Heidenhain变异型散发型克-雅病患者的临床资料，并复习相关文献，总结疾病的临床特征。方法与结果: 苏州大学附属第二医院于2023年8月19日收治1例81岁男性患者，临床表现为行走不稳、同时性失认、视觉性共济失调和眼动性失用，随后病情快速进展，出现痴呆、小脑受累和肌阵挛；脑脊液14-3-3γ蛋白阳性；头部MRI显示双侧顶枕颞叶皮质DWI高信号，呈“花边征”；脑电图呈现以额颞区为主的较多中高波幅慢波，最终诊断为以Balint综合征为首发表现的Heidenhain变异型散发型克-雅病。予以改善认知功能、控制肌阵挛等治疗，疗效欠佳。出院20 d后出现无动性缄默和呼吸困难，最终因呼吸衰竭死亡。结论: Balint综合征是散发型克-雅病的罕见表现，应注意早期识别。新型冠状病毒感染可能诱发或加速散发型克-雅病进展。

关键词: 克-亚综合征, 新型冠状病毒, 视觉障碍, 眼球运动障碍, 磁共振成像, 脑电描记术

Abstract:

Objective: To report the clinical diagnosis and treatment of a case of sporadic Creutzfeldt-Jakob disease (sCJD), Heidenhain variant, presenting initially with Balint syndrome following severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection, and to review relevant literature to summarize the clinical characteristics of this disease. Methods and Results: An 81-year-old male patient was admitted to The Second Affiliated Hospital of Soochow University on August 19, 2023, with clinical manifestations of gait instability, simultanagnosia, optic ataxia, and oculomotor apraxia (Balint syndrome). The condition progressively worsened, with the development of dementia, features of cerebellar involvement, and myoclonus. Cerebrospinal fluid tested positive for 14-3-3γ protein. DWI showed cortical hyperintensities in the bilateral parieto-occipito-temporal lobes, displaying the "cortical ribbon sign". EEG revealed frequent medium-to-high amplitude slow waves predominantly in the fronto-temporal region. The final diagnosis was sCJD, Heidenhain variant, presenting initially with Balint syndrome. Treatment aimed at improving cognition and controlling myoclonus yielded a poor response. Twenty days after discharge, the patient developed akinetic mutism and dyspnea, ultimately succumbing to respiratory failure. Conclusions: Balint syndrome is a rare initial clinical manifestation of sCJD, warranting early recognition. SARS-CoV-2 infection may potentially trigger or accelerate the progression of sCJD.

Key words: Creutzfeldt-Jakob syndrome, SARS-CoV-2, Vision disorders, Ocular motility disorders, Magnetic resonance imaging, Electroencephalography

中图分类号: 

• R741