Geschwind Michael D
Continuum (Minneap Minn). 2016 Apr;22(2 Dementia):510-37. doi: 10.1212/CON.0000000000000319.
This article presents a practical and informative approach to the evaluation of a patient with a rapidly progressive dementia (RPD).
Prion diseases are the prototypical causes of RPD, but reversible causes of RPD might mimic prion disease and should always be considered in a differential diagnosis. Aside from prion diseases, the most common causes of RPD are atypical presentations of other neurodegenerative disorders, curable disorders including autoimmune encephalopathies, as well as some infections, and neoplasms. Numerous recent case reports suggest dural arterial venous fistulas sometimes cause RPDs.
RPDs, in which patients typically develop dementia over weeks to months, require an alternative differential than the slowly progressive dementias that occur over a few years. Because of their rapid decline, patients with RPDs necessitate urgent evaluation and often require an extensive workup, typically with multiple tests being sent or performed concurrently. Jakob-Creutzfeldt disease, perhaps the prototypical RPD, is often the first diagnosis many neurologists consider when treating a patient with rapid cognitive decline. Many conditions other than prion disease, however, including numerous reversible or curable conditions, can present as an RPD. This chapter discusses some of the major etiologies for RPDs and offers an algorithm for diagnosis.
本文介绍了一种评估快速进展性痴呆（RPD）患者的实用且信息丰富的方法。
朊病毒病是RPD的典型病因，但RPD的可逆病因可能会模仿朊病毒病，在鉴别诊断时应始终予以考虑。除朊病毒病外，RPD最常见的病因是其他神经退行性疾病的非典型表现、包括自身免疫性脑病在内的可治愈疾病，以及一些感染和肿瘤。近期众多病例报告表明，硬脑膜动静脉瘘有时会导致RPD。
RPD患者通常在数周或数月内发展为痴呆，其鉴别诊断与数年发生的缓慢进展性痴呆不同。由于病情迅速恶化，RPD患者需要紧急评估，通常需要进行全面检查，通常会同时进行多项检查。克雅氏病可能是典型的RPD，在治疗认知快速下降的患者时，往往是许多神经科医生首先考虑的诊断。然而，除朊病毒病外，许多其他疾病，包括众多可逆或可治愈的疾病，都可能表现为RPD。本章讨论了RPD的一些主要病因，并提供了诊断算法。
