散发性克雅氏病的诊断挑战:典型临床表现阴性的病例研究

IF 1 Q3 MEDICINE, GENERAL & INTERNAL
Xiaoyu Zhu, Ran Li, Yu Zhu, Yunlong Tan
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引用次数: 0

摘要

克雅氏病(Creutzfeldt-Jakob disease, CJD)是一种罕见且致命的神经退行性疾病,由朊蛋白(PrPSc)异构体错误折叠引起。这种疾病的特点是进展迅速,缺乏有效的治疗方案,使其具有特别的破坏性。在其各种亚型中,散发性克雅氏病(sCJD)在人类中最常见。由于其非特异性症状和难以将其与其他神经退行性疾病区分开来,因此在死亡前诊断CJD具有挑战性。病例报告我们详细介绍了一名58岁女性疑似sCJD的初步表现,突出了非典型症状和延长的生存期。本病例的独特之处在于,尽管患者表现出相对典型的临床特征,但所有CJD的实验室和检查结果均为阴性。这导致患者在疾病的早期阶段在多个部门之间转移,包括精神科,使其难以得到正确的诊断和治疗。病人通过各种医疗咨询的旅程强调了诊断这种罕见疾病的复杂性。它说明了考虑临床表现和补充检查的整体方法的重要性。结论:过度依赖辅助检查,加上多个临床科室对sCJD的认识不足,以及对临床症状缺乏关注,最终导致误诊为精神障碍。这引发了一个复杂而漫长的诊断过程,大大加重了患者及其家人的负担。这种情况强调了在使用诊断工具时识别罕见疾病如sCJD的临床表现的重要性。此外,它强调了加强临床科室之间的跨学科沟通和合作的必要性,以促进及时和准确地识别sCJD。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Diagnostic Challenges in Sporadic Creutzfeldt-Jakob Disease: A Case Study of Typical Clinical Presentation with Negative Findings.

BACKGROUND Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder caused by the misfolded isoform of the prion protein (PrPSc). The disease is characterized by rapid progression and the absence of effective treatment options, making it particularly devastating. Among its various subtypes, sporadic Creutzfeldt-Jakob disease (sCJD) is the most common in humans. Diagnosing CJD before death is challenging due to its nonspecific symptoms and the difficulty in distinguishing it from other neurodegenerative conditions. CASE REPORT We detail the initial presentation of a 58-year-old woman with suspected sCJD, highlighting the atypical symptoms and prolonged survival. The uniqueness of this case lies in the fact that, despite the patient exhibiting relatively typical clinical features, all laboratory and examination results for CJD consistently returned negative. This led to the patient being transferred between multiple departments, including psychiatry, during the early stages of the disease, making it difficult to receive a correct diagnosis and treatment. The patient's journey through various medical consultations underscores the complexity of diagnosing such a rare condition. It illustrates the importance of a holistic approach that considers both clinical presentation and supplementary examinations. CONCLUSIONS Excessive reliance on supplementary examinations, coupled with insufficient awareness of sCJD across multiple clinical departments and a lack of attentiveness to clinical symptoms, culminated in a misdiagnosis as a psychiatric disorder. This initiated a convoluted and protracted diagnostic process, significantly exacerbating the burden on both the patient and her family. This situation underscores the critical importance of recognizing clinical presentations in rare diseases like sCJD while employing diagnostic tools. Furthermore, it highlights the necessity for enhanced interdisciplinary communication and collaboration among clinical departments to facilitate timely and accurate identification of sCJD.

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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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