Piura Yoav D, Corriveau-Lecavalier Nick, Abu Dabrh Abd Moain, Geschwind Michael D, Brigham Tara J, Day Gregory S
Department of Neurology, Mayo Clinic in Florida, 4500 San Pablo Road S, Jacksonville, FL, 32224, USA.
Department of Psychology and Psychiatry, Mayo Clinic, Rochester, MN, USA.
J Neurol. 2024 Dec 16;272(1):67. doi: 10.1007/s00415-024-12845-9.
The term rapid progressive dementia (RPD) may be applied to patients who develop dementia within 1 year or complete incapacitation within 2 years of the first symptom of impairment. However, in select cases, cognitive impairment may emerge abruptly, with symptoms evolving across hours or days. We sought to determine the frequency, etiologies, and factors that associated with ultra-RPD.
Ultra-RPD was defined as persistent dementia (global Clinical Dementia Rating ≥ 1), developing within 7 days of initial symptoms. Patients with ultra-RPD were identified via case review of patients enrolled in a prospective study of RPD at two tertiary care centers (February 2016-September 2023) followed by a systematic review of multiple English-language databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (completed January 2024).
Three of 188 patients with RPD enrolled in our prospective series met the proposed definition for ultra-RPD (frequency = 1.6%). Systematic review yielded 57 additional cases from 47 publications (60 total cases). Ultra-RPD was attributed to vascular (40%), toxic/metabolic (22%), autoimmune/inflammatory (20%), and iatrogenic/structural (12%) causes. Lesions within the Papez circuit were detected in 52/59 (88%) of patients on neuroimaging. Twelve patients (20%) had potentially treatable causes of ultra-RPD.
Patients with ultra-RPD were rarely encountered in our prospective series, representing < 2% of cases of RPD, and rarely reported in the extant literature. The evaluation of patients with ultra-RPD should prioritize testing for vascular, toxic/metabolic, and autoimmune/inflammatory conditions that affect neuroanatomical structures or networks critical for memory formation and retrieval.
快速进展性痴呆（RPD）这一术语可用于描述在出现损害的首发症状后1年内发生痴呆或在2年内完全丧失行为能力的患者。然而，在某些特定情况下，认知障碍可能会突然出现，症状在数小时或数天内演变。我们试图确定与超快速进展性痴呆（ultra - RPD）相关的频率、病因及因素。
超快速进展性痴呆定义为在初始症状出现后7天内发生持续性痴呆（总体临床痴呆评定量表≥1）。通过对两个三级医疗中心（2016年2月至2023年9月）参与RPD前瞻性研究的患者进行病例回顾，随后按照系统评价和Meta分析的首选报告项目（于2024年1月完成）对多个英文数据库进行系统评价，从而确定超快速进展性痴呆患者。
在我们前瞻性系列研究纳入的188例RPD患者中，有3例符合超快速进展性痴呆的拟议定义（频率 = 1.6%）。系统评价从47篇出版物中又获得了57例病例（共60例）。超快速进展性痴呆归因于血管性（40%）、毒性/代谢性（22%）、自身免疫/炎症性（20%）以及医源性/结构性（12%）病因。神经影像学检查发现52/59（88%）的患者在帕佩兹环路内有病变。12例患者（20%）有超快速进展性痴呆的潜在可治疗病因。
在我们的前瞻性系列研究中，超快速进展性痴呆患者很少见，占RPD病例的不到2%，且在现有文献中也很少报道。对超快速进展性痴呆患者的评估应优先检测影响对记忆形成和检索至关重要的神经解剖结构或网络的血管性、毒性/代谢性以及自身免疫/炎症性疾病。
