HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT
Creutzfeldt-Jakob disease (CJD) is a rare spongiform encephalopathy characterized by a rapid neurodegenerative progress, caused by a misfolded variant of the cellular prion protein (PrP) known as PrPSc. The clinical presentation of sCJD includes a wide range of neurological signs of cortical, subcor...
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Brazilian Society of Geriatrics and Gerontology
2020-05-01
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doaj-542bc89688f042e99dfc10c51fef64102020-11-25T02:49:18ZengBrazilian Society of Geriatrics and GerontologyGeriatrics, Gerontology and Aging2447-21232020-05-01141717510.5327/Z2447-212320191900063HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORTLaura Furtado Pessoa de Mendonça0https://orcid.org/0000-0002-4253-9296Pedro Maia Nobre Rocha Saffi1https://orcid.org/0000-0002-7401-5710Luciana Lilian Louzada Martini2https://orcid.org/0000-0002-4160-4826Luciano Farage3https://orcid.org/0000-0002-2293-3771Einstein Francisco Camargos4https://orcid.org/0000-0003-1991-5529Faculty of Medicine, Universidade de Brasília - Brasília (DF), Brazil.Faculty of Medicine, Universidade de Brasília - Brasília (DF), Brazil.Multidisciplinary Center for the Elderly, University Hospital of Brasília, Universidade de Brasília - Brasília (DF), Brazil. Postgraduate in Medical Sciences, Faculty of Medicine, Universidade de Brasília - Brasília (DF), Brazil.Postgraduate in Medical Sciences, Faculty of Medicine, Universidade de Brasília - Brasília (DF), Brazil.Multidisciplinary Center for the Elderly, University Hospital of Brasília, Universidade de Brasília - Brasília (DF), Brazil. Postgraduate in Medical Sciences, Faculty of Medicine, Universidade de Brasília - Brasília (DF), Brazil.Creutzfeldt-Jakob disease (CJD) is a rare spongiform encephalopathy characterized by a rapid neurodegenerative progress, caused by a misfolded variant of the cellular prion protein (PrP) known as PrPSc. The clinical presentation of sCJD includes a wide range of neurological signs of cortical, subcortical, or cerebellar origin, either isolated or in various combinations. Due to this protean clinical presentation form, sCJD must be distinguished from other dementias. In this case report, we discuss the Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD), a rare variant characterized by early visual symptoms and typical findings in imaging scans. Our patient presented rapidly progressive dementia and a history of visual hallucinations. As for other prion diseases, only symptomatic treatment is available for HvCJD. Thirty years of clinical investigation of patients with prion disease have resulted in little progress in either defining or evaluating potential treatments.https://ggaging.com/details/561/en-US/heidenhain-variant-of-creutzfeldt-jakob-disease-in-brazil--a-case-reportdementiageriatricsprion diseasesprion proteins |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Laura Furtado Pessoa de Mendonça Pedro Maia Nobre Rocha Saffi Luciana Lilian Louzada Martini Luciano Farage Einstein Francisco Camargos |
spellingShingle |
Laura Furtado Pessoa de Mendonça Pedro Maia Nobre Rocha Saffi Luciana Lilian Louzada Martini Luciano Farage Einstein Francisco Camargos HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT Geriatrics, Gerontology and Aging dementia geriatrics prion diseases prion proteins |
author_facet |
Laura Furtado Pessoa de Mendonça Pedro Maia Nobre Rocha Saffi Luciana Lilian Louzada Martini Luciano Farage Einstein Francisco Camargos |
author_sort |
Laura Furtado Pessoa de Mendonça |
title |
HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT |
title_short |
HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT |
title_full |
HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT |
title_fullStr |
HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT |
title_full_unstemmed |
HEIDENHAIN VARIANT OF CREUTZFELDT-JAKOB DISEASE IN BRAZIL: A CASE REPORT |
title_sort |
heidenhain variant of creutzfeldt-jakob disease in brazil: a case report |
publisher |
Brazilian Society of Geriatrics and Gerontology |
series |
Geriatrics, Gerontology and Aging |
issn |
2447-2123 |
publishDate |
2020-05-01 |
description |
Creutzfeldt-Jakob disease (CJD) is a rare spongiform encephalopathy characterized by a rapid neurodegenerative progress, caused by a misfolded variant of the cellular prion protein (PrP) known as PrPSc. The clinical presentation of sCJD includes a wide range of neurological signs of cortical, subcortical, or cerebellar origin, either isolated or in various combinations. Due to this protean clinical presentation form, sCJD must be distinguished from other dementias. In this case report, we discuss the Heidenhain variant of Creutzfeldt-Jakob disease (HvCJD), a rare variant characterized by early visual symptoms and typical findings in imaging scans. Our patient presented rapidly progressive dementia and a history of visual hallucinations. As for other prion diseases, only symptomatic treatment is available for HvCJD. Thirty years of clinical investigation of patients with prion disease have resulted in little progress in either defining or evaluating potential treatments. |
topic |
dementia geriatrics prion diseases prion proteins |
url |
https://ggaging.com/details/561/en-US/heidenhain-variant-of-creutzfeldt-jakob-disease-in-brazil--a-case-report |
work_keys_str_mv |
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