AI Article Synopsis

  • Rapidly progressive dementia (RPD) involves a group of disorders that lead to cognitive and motor decline within two years, with various causes including neurodegenerative and infectious diseases, notably Jakob-Creutzfeldt disease (CJD).
  • This review highlights the clinical features and necessary diagnostic steps for RPD, emphasizing the importance of identifying treatable conditions that can mimic CJD.
  • A standardized approach is recommended for neurologists to effectively manage patients with rapidly presenting symptoms that resemble CJD.

Article 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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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3356774PMC
http://dx.doi.org/10.1097/NRL.0b013e31820ba5e3DOI Listing

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