散发性克雅氏病：秘鲁的病例系列。
Sporadic Creutzfeldt Jakob disease: Case series in Peru.
机构信息
Hospital Nacional Edgardo Rebagliati Martins, Departamento de Neurología, Lima, Peru.
Hospital Nacional Edgardo Rebagliati Martins, Departamento de Geriatría, Lima, Peru.
出版信息
Colomb Med (Cali). 2024 Mar 30;55(1):e2035821. doi: 10.25100/cm.v55i1.5821. eCollection 2024 Jan-Mar.
DESCRIPTION OF THE CASES
A series of 6 cases with a probable diagnosis of sporadic CJD, treated in a Peruvian national reference hospital, are presented.
CLINICAL FINDINGS
The relevant clinical signs were rapidly progressive dementia and myoclonus, followed by akinetic mutism and pyramidal signs.
TREATMENT AND RESULTS
Of the cases presented, 80% were men, with an average age of presentation of 65 years and duration from diagnosis to death of 6.5 months. Laboratory tests, images (Brain Resonance) and protein dosage 14.3.3 were performed to support the clinical suspicion. There is no effective treatment at the moment for said pathology.
CLINICAL RELEVANCE
Creutzfeldt-Jakob disease (CJD) is a progressive, fatal, neurodegenerative disease of low prevalence and incidence. Great clinical suspicion and the exclusion of other etiologies are required. Currently there is no treatment for this entity and there is a high probability of death before one year.
病例描述
呈现了一系列在秘鲁国家参考医院接受治疗的、可能诊断为散发性 Creutzfeldt-Jakob 病（CJD）的 6 例病例。
临床发现
相关的临床体征是快速进行性痴呆和肌阵挛，随后是无动性缄默和锥体束征。
治疗和结果
所呈现的病例中，80%为男性，平均发病年龄为 65 岁，从诊断到死亡的时间为 6.5 个月。进行了实验室检查、影像（脑磁共振）和蛋白定量 14.3.3，以支持临床怀疑。目前，对于这种病理学还没有有效的治疗方法。
临床相关性
克雅氏病（CJD）是一种罕见的、发病率和患病率都很低的进行性、致命性、神经退行性疾病。需要高度的临床怀疑和排除其他病因。目前，对于这种疾病还没有治疗方法，而且在一年内死亡的可能性很大。
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