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Neurologist. 2011 Mar;17(2):67-74. doi: 10.1097/NRL.0b013e31820ba5e3.

The evaluation of rapidly progressive dementia.

Author information

  • 1Bedford VA Hospital, Geriatric Research and Education Clinical Center (GRECC), Bedford. Michael.H.Rosenbloom@HealthPartners.com

Abstract

BACKGROUND:

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.

REVIEW SUMMARY:

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.

CONCLUSIONS:

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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