Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical Center, Göttingen, Germany.
German Center for Neurodegenerative Diseases (DZNE), Göttingen, Germany.
Nat Rev Neurol. 2022 Jun;18(6):363-376. doi: 10.1038/s41582-022-00659-0. Epub 2022 May 4.
Rapidly progressive dementias (RPDs) are a group of heterogeneous disorders that include immune-mediated, infectious and metabolic encephalopathies, as well as prion diseases and atypically rapid presentations of more common neurodegenerative diseases. Some of these conditions are treatable, and some must be diagnosed promptly because of their potential infectivity. Prion disease is considered to be the prototypical RPD, but over the past two decades, epidemiological reports and the identification of various encephalitis-mediating antibodies have led to a growing recognition of other encephalopathies as potential causes of rapid cognitive decline. Knowledge of RPD aetiologies, syndromes and diagnostic work-up protocols will help clinicians to establish an early, accurate diagnosis, thereby reducing morbidity and mortality, especially in immune-mediated and other potentially reversible dementias. In this Review, we define the syndrome of RPD and shed light on its different aetiologies and on secondary factors that might contribute to rapid cognitive decline. We describe an extended diagnostic procedure in the context of important differential diagnoses, discuss the utility of biomarkers and summarize potential treatment options. In addition, we discuss treatment options such as high-dose steroid therapy in the context of therapy and diagnosis in clinically ambiguous cases.
快速进展性痴呆（RPD）是一组异质性疾病，包括免疫介导、感染和代谢性脑病，以及朊病毒病和更常见神经退行性疾病的非典型快速表现。其中一些疾病是可治疗的，而有些疾病由于其潜在传染性必须迅速诊断。朊病毒病被认为是典型的 RPD，但在过去的二十年中，流行病学报告和各种脑炎介导抗体的鉴定导致人们越来越认识到其他脑病可能是导致快速认知衰退的原因。了解 RPD 的病因、综合征和诊断检查方案将有助于临床医生尽早做出准确诊断，从而降低发病率和死亡率，尤其是在免疫介导和其他潜在可逆转的痴呆症中。在这篇综述中，我们定义了 RPD 综合征，并探讨了其不同的病因以及可能导致快速认知衰退的次要因素。我们在重要的鉴别诊断背景下描述了扩展的诊断程序，讨论了生物标志物的效用，并总结了潜在的治疗选择。此外，我们还讨论了在临床情况下具有模糊性的情况下治疗和诊断中类固醇激素治疗等治疗选择。
