An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease

Rapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectio...

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Main Authors: Parmvir Parmar, Curtis L. Cooper, Daniel Kobewka
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Infectious Diseases
Online Access:http://dx.doi.org/10.1155/2018/2374179
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author Parmvir Parmar
Curtis L. Cooper
Daniel Kobewka
author_facet Parmvir Parmar
Curtis L. Cooper
Daniel Kobewka
author_sort Parmvir Parmar
collection DOAJ
description Rapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectious, inflammatory, autoimmune, neoplastic, metabolic, and neurodegenerative causes. We present the case of a previously highly functional 76-year-old individual who presented with a 6-month history of rapidly progressive dementia. His most prominent symptoms were cognitive impairment, aphasia, visual hallucinations, and ataxia. Following an extensive battery of tests in hospital, the differential diagnosis remained probable CJD versus autoimmune encephalitis. He clinically deteriorated and progressed to akinetic mutism and myoclonus. He passed away 8 weeks after his initial presentation to hospital, and an autopsy confirmed a diagnosis of sporadic CJD. We use this illustrative case as a framework to discuss the clinical and diagnostic considerations in the workup for rapidly progressive dementia. We also discuss CJD and autoimmune encephalitis, the two main diagnostic possibilities in our patient, in more detail.
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spelling doaj-art-4929a87dc80446b5a541797b4e47f31d2025-08-20T03:36:48ZengWileyCase Reports in Infectious Diseases2090-66252090-66332018-01-01201810.1155/2018/23741792374179An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob DiseaseParmvir Parmar0Curtis L. Cooper1Daniel Kobewka2Faculty of Medicine, University of Ottawa, Ottawa, ON, CanadaFaculty of Medicine, University of Ottawa, Ottawa, ON, CanadaFaculty of Medicine, University of Ottawa, Ottawa, ON, CanadaRapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectious, inflammatory, autoimmune, neoplastic, metabolic, and neurodegenerative causes. We present the case of a previously highly functional 76-year-old individual who presented with a 6-month history of rapidly progressive dementia. His most prominent symptoms were cognitive impairment, aphasia, visual hallucinations, and ataxia. Following an extensive battery of tests in hospital, the differential diagnosis remained probable CJD versus autoimmune encephalitis. He clinically deteriorated and progressed to akinetic mutism and myoclonus. He passed away 8 weeks after his initial presentation to hospital, and an autopsy confirmed a diagnosis of sporadic CJD. We use this illustrative case as a framework to discuss the clinical and diagnostic considerations in the workup for rapidly progressive dementia. We also discuss CJD and autoimmune encephalitis, the two main diagnostic possibilities in our patient, in more detail.http://dx.doi.org/10.1155/2018/2374179
spellingShingle Parmvir Parmar
Curtis L. Cooper
Daniel Kobewka
An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
Case Reports in Infectious Diseases
title An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
title_full An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
title_fullStr An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
title_full_unstemmed An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
title_short An Evaluation of Rapidly Progressive Dementia Culminating in a Diagnosis of Creutzfeldt–Jakob Disease
title_sort evaluation of rapidly progressive dementia culminating in a diagnosis of creutzfeldt jakob disease
url http://dx.doi.org/10.1155/2018/2374179
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