克雅氏病伴异常视幻觉1例报告

S. Rho, Hyo Joon Kim, Min Park
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摘要

克雅氏病(Creutzfeldt-Jakob disease, CJD)最初于1921年被发现,是一种罕见的传染性朊病毒疾病。目前已知的克雅氏病有四种。散发性、家族性、医源性和变异型,其中散发性克雅氏病(sCJD)最为常见,sCJD具有独特的临床和诊断特征。它是一种进展迅速的致命传染病,通常在发病一年内导致死亡。这种疾病被认为是由一种被称为朊蛋白的细胞糖蛋白的异常异构体引起的,大约百万分之一的人会发现这种疾病。散发性克雅氏病与其他朊病毒疾病一样,被归类为传染性海绵状脑病,在大约85%的患者中以散发性疾病的形式发生,没有可识别的传播模式。目前的诊断标准包括明显的表型,脑电图(EEG)周期性的锐波和慢波复合体。散发性克雅氏病(sCJD)患者的临床特征可能是多种症状。由于临床特征不同,在初步评估时可能需要与脑血管疾病或精神症状区分。在这种情况下,我们讨论的临床病例57岁的女性患者多发性神经症状。患者在评估和保守治疗中表现出爱丽丝梦游仙境综合征的临床特征,并被诊断为sCJD。在这方面,我们认为未来sCJD患者需要评估和管理,同时认识到AIWS等非特异性神经症状的可能性。
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Creutzfeldt–Jakob Disease with Unusual Visual Hallucination : A Case Report
Initially described in 1921, Creutzfeldt-Jakob disease (CJD) is a rare, transmissible prion disease. There are four types of CJD known. Sporadic, familial, iatrogenic and variant, of which sporadic Creutzfeldt-Jakob disease (sCJD) is the most common, and sCJD has characteristic clinical and diagnostic features. It is a rapidly progressive, deadly infectious disease that usually results in death within a year of onset. The disease is thought to be caused by an abnormal isoform of a cellular glycoprotein known as prion protein, and is found in about 1 in 1 million people. Sporadic Creutzfeldt-Jakob disease, like other prion diseases, is classified as a transmissible spongiform encephalopathy and occurs as a sporadic disease without a recognizable transmission pattern in approximately 85% of patients. Current criteria for diagnosis include a distinct phenotype, periodic sharp and slow-wave complexes at electroencephalography (EEG), Clinical features seen in patients with sporadic Creutzfeldt-Jakob disease (sCJD) may be diverse symptoms. Due to diverse clinical features, it may be necessary to differentiate from cerebrovascular disease or psychotic symptoms during initial evaluation. In this case, we discuss the clinical case of a 57-year-old female patient with multiple neurological symptoms. The patient showed clinical features of alice in Wonderland syndrome during evaluation and conservative care, and was diagnosed with sCJD. In this regard, it is considered that evaluation and management are required along with recognition of the possibility of non-specific neurological symptoms such as AIWS in sCJD patients in the future.
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