Creutzfeld-Jakob病(HvCJD)的Heidenhain变种是一种持续进行性和致命的神经退行性疾病,其特征是在其临床过程早期具有明显的视觉特征。然而,癫痫发作在HvCJD中非常罕见。描述了我们机构收治的HvCJD和癫痫发作患者的病史,然后对HvCJD和癫痫发作之间的关系进行了系统评价。对Medline数据库的系统搜索,PubMed,进行了PsycInfo,从成立到2019年11月,使用与\'Creutzfeldt-Jakob病\'和\'Heidenhain变体\'相关的关键字,为了确定HvCJD的癫痫发作频率,还有,癫痫发作符号学和电图特征。本系统评价的构建遵循系统评价和荟萃分析(PRISMA)指南的首选项目报告。所有研究,包括符合HvCJD诊断标准的患者的病例报告,其中有关临床表现的详细信息,成像,生化和脑电图检查结果均包括在内.共有46篇文章报道了73例患者。73例中只有4例(5.5%)发生癫痫发作。这些癫痫发作的符号学是局灶性运动性癫痫发作,有或没有继发性泛化和枕叶癫痫发作。影像学和电描记法的发现最常见于后脑皮质(尤其是枕骨和枕顶区)。这项系统评价表明,尽管大脑后部区域的成像频率和电图异常,但HvCJD的癫痫发作并不常见。本系统评价的一个主要局限性是出版物在临床数据报告不完整方面的可变性。特别是潜在的缉获报告不足,以及后续行动,这可能导致HvCJD患者报告的癫痫发作频率降低。
The Heidenhain variant of Creutzfeld-Jakob disease (HvCJD) is a relentlessly progressive and fatal neurodegenerative disorder characterised by prominent visual features early in its clinical course. However, seizures are uncommonly reported in HvCJD. The case history of a patient admitted to our institution with HvCJD and seizures is described followed by a systematic
review of the association between HvCJD and seizures. A systematic search of the databases Medline, PubMed, and PsycInfo was conducted, from inception to November 2019, using keywords relating to \'Creutzfeldt-Jakob disease\' and \'Heidenhain variant\', to ascertain the frequency of seizures in HvCJD, as well as, seizure semiology and electrographic features. The Preferred Items Reporting for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed in the construction of this systematic
review. All studies, including case reports of patients who met the diagnostic criteria for HvCJD where details pertaining to clinical presentation, imaging, biochemical and EEG findings were available were included. There were 46 articles reporting on a total of 73 patients. Seizures occurred in only four out of 73 cases (5.5%). The semiology of these seizures were focal motor seizures with or without secondary generalisation and occipital lobe seizures. Imaging and electrographic findings were most commonly abnormal in the posterior cerebral cortices (in particular the occipital and occipito-parietal regions). This systematic
review suggests that seizures are uncommon in HvCJD despite the frequency of imaging and electrographic abnormalities in the posterior cerebral regions. A key limitation of this systematic
review is the variability of publications in terms of incomplete reporting of clinical data, in particular potential under-reporting of seizures, as well as follow up, which may have contributed to the lower frequency of seizures reported in patients with HvCJD.