Comprehensive and Methodical: Diagnostic and Management Approaches to Rapidly Progressive Dementia

  • Supriya Mahajan
  • Brian S. ApplebyEmail author
Dementia (J Pillai, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Dementia


Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone.

Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer’s disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD. Additionally, in the past decade, emerging retrospective data have led to suggested treatments in autoimmune encephalitis that, if instituted early, can protect patients against residual deficits and disease relapse.

Summary Here, we provide an integrative clinical and diagnostic treatment approach that is applicable to the various forms of RPD. We have highlighted the clinical features of selected types of RPD that have experienced advances in the last 10–15 years.


Rapidly progressive dementia Prion disease Autoimmune encephalitis Diagnosis Management Treatment 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that there is no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Department of NeurologyUniversity Hospitals Cleveland Medical CenterBeachwoodUSA
  2. 2.National Prion Disease Pathology Surveillance Center and the Departments of Neurology, Psychiatry, and PathologyCase Western Reserve University School of MedicineClevelandUSA
  3. 3.BeachwoodUSA

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