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Age and Ageing Advance Access originally published online on December 8, 2007
Age and Ageing 2008 37(2):231-232; doi:10.1093/ageing/afm191
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Copyright © The Author 2007. Published by Oxford University Press on behalf of the British Geriatrics Society.

Case Reports

Vision loss due to coincident ocular and central causes in a patient with Heidenhain variant Creutzfeldt-Jakob disease

Maria Foundas1, Mark D. Donaldson1, Ian L. McAllister2 and Leslie R. Bridges3

1 Bentley Health Service, Mills Street, Bentley, Perth, Western Australia
2 Lions Eye Institute, Perth, Western Australia
3 Royal Perth Hospital, Perth, Western Australia

Address correspondence to: Maria Foundas. Tel: 0419 931 682; Fax: (+61) 089 444 8893. Email: Maria.Foundas{at}health.wa.gov.au

Abstract

Creutzfeldt-Jakob disease (CJD) is a degenerative disease of the brain associated with a rapidly progressive spongiform encephalopathy. Visual symptoms and neuro-ophthalmological signs are not infrequent, and presentation to an ophthalmologist may result. A case is reported of an 89-years-old gentleman who presented with a short history of isolated deterioration in vision. He underwent ocular intervention but subsequently developed progressive dementia, asterixis, myoclonus, cerebellar and extrapyramidal signs, and cortical blindness. An electroencephalogram was consistent with CJD. The patient progressively deteriorated and died 9 weeks after symptom onset. Limited post-mortem examination confirmed CJD.

Keywords: cortical blindness, Creutzfeldt-Jakob disease, dementia, myoclonus, elderly

Received 1 May 2007; accepted in revised form 19 September 2007.


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