Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation

Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This ca...

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Main Authors: R. Williams, F. Cresswell, M. McClure, R. Lane
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2011/719583
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author R. Williams
F. Cresswell
M. McClure
R. Lane
author_facet R. Williams
F. Cresswell
M. McClure
R. Lane
author_sort R. Williams
collection DOAJ
description Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1) Electroencephalogram demonstrating periodic sharp wave complexes, (2) MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3) presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain histology confirmed a typical spongiform encephalopathy. Establishing an underlying aetiology is dementia is important not only for prognostic reasons but in order to detect potentially reversible causes. In cases of an atypical dementing illness our proposed investigations may assist in confirming or excluding underlying Creutzfeltd-Jakob disease.
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spelling doaj-art-b26c35d471eb44dcbb651aa255d844d42025-02-03T01:01:59ZengWileyCase Reports in Medicine1687-96271687-96352011-01-01201110.1155/2011/719583719583Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for InvestigationR. Williams0F. Cresswell1M. McClure2R. Lane3Department of Radiology, St George’s Hospital, Blackshaw Road, London SW17 0QT, UKLister Unit Infectious Diseases Department, Northwick Park Hospital, Harrow Road, London HA1 3UJ, UKDepartment of Renal Medicine, Hammersmith Hospital, Du Cane Road, London W12 0HS, UKDepartment of Neurology, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UKCognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1) Electroencephalogram demonstrating periodic sharp wave complexes, (2) MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3) presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain histology confirmed a typical spongiform encephalopathy. Establishing an underlying aetiology is dementia is important not only for prognostic reasons but in order to detect potentially reversible causes. In cases of an atypical dementing illness our proposed investigations may assist in confirming or excluding underlying Creutzfeltd-Jakob disease.http://dx.doi.org/10.1155/2011/719583
spellingShingle R. Williams
F. Cresswell
M. McClure
R. Lane
Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
Case Reports in Medicine
title Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
title_full Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
title_fullStr Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
title_full_unstemmed Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
title_short Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation
title_sort creutzfeldt jakob disease as a cause of cognitive decline and seizures in the elderly diagnostic pointers and strategy for investigation
url http://dx.doi.org/10.1155/2011/719583
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