Paterson Ross W, Takada Leonel T, Geschwind Michael D
University of California (RWP, LTT, MDG), San Francisco Memory and Aging Center, San Francisco; National Hospital for Neurology and Neurosurgery (RWP), Queen Square, London, UK; and Department of Neurology (LTT), University of Sao Paulo, Sao Paulo, Brazil.
Neurol Clin Pract. 2012 Sep;2(3):187-200. doi: 10.1212/CPJ.0b013e31826b2ae8.
Rapidly progressive dementias are conditions that typically cause dementia over weeks or months. They are a particular challenge to neurologists as the differential diagnosis often is different from the more typical, slowly progressive dementias. Early and accurate diagnosis is essential, as many of the etiologies are treatable. The information in this review is in part based on experience through our rapidly progressive dementia program at the University of California San Francisco, Memory and Aging Center. As treatment of a rapidly progressive dementia is entirely dependent on the diagnosis, we present a comprehensive, structured, but pragmatic approach to diagnosis, including key clinical, laboratory, and radiologic features. For the 2 most common causes of rapid dementia, treatment algorithms for the autoimmune encephalopathies and symptomatic management for the neurodegenerative causes are discussed.
快速进展性痴呆是通常在数周或数月内导致痴呆的病症。它们对神经科医生来说是一项特殊挑战,因为鉴别诊断往往不同于更典型的、缓慢进展性痴呆。早期准确诊断至关重要,因为许多病因是可治疗的。本综述中的信息部分基于我们在加利福尼亚大学旧金山分校记忆与衰老中心的快速进展性痴呆项目所积累的经验。由于快速进展性痴呆的治疗完全取决于诊断,我们提出一种全面、结构化但务实的诊断方法,包括关键的临床、实验室和放射学特征。对于快速痴呆的2种最常见病因,讨论了自身免疫性脑病的治疗算法以及神经退行性病因的对症处理。