The Evaluation of Rapidly Progressive Dementia

Bedford VA Hospital, Geriatric Research and Education Clinical Center (GRECC), Bedford.
The Neurologist (Impact Factor: 1.08). 03/2011; 17(2):67-74. DOI: 10.1097/NRL.0b013e31820ba5e3
Source: PubMed

ABSTRACT Rapidly progressive dementia (RPD) is a unique set of disorders resulting in cognitive, behavioral, and motor decline within 2 years. A variety of etiologies may contribute to RPD including neurodegenerative, inflammatory, infectious, and toxic-metabolic conditions. Jakob-Creutzfeldt disease (CJD) is frequently the most concerning diagnosis on the differential. The challenge for the neurologist is distinguishing prion disease from reversible processes that result in dementia.
This review discusses the clinical aspects and the diagnostic workup of RPD. Particular focus is given to both CJD and the potentially treatable inflammatory conditions that may cause a similar presentation. Furthermore, a standardized stepwise approach is outlined for patients presenting with RPD.
Neurologists should adopt a standardized approach to the rapidly presenting disease processes that may mimic CJD in their clinical and radiologic features.

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