Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases
The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia,...
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| Language: | English |
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Thieme Revinter Publicações
1998-09-01
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| Series: | Arquivos de Neuro-Psiquiatria |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1998000300003&tlng=en |
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| author | CARLOS M. DE CASTRO COSTA JEAN MARIE BRUCHER CHRISTIAN LATERRE |
| author_facet | CARLOS M. DE CASTRO COSTA JEAN MARIE BRUCHER CHRISTIAN LATERRE |
| author_sort | CARLOS M. DE CASTRO COSTA |
| collection | DOAJ |
| description | The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56%) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people. |
| format | Article |
| id | doaj-art-aa6ca5c86c4a4eb59f5c68942ce0601c |
| institution | DOAJ |
| issn | 1678-4227 |
| language | English |
| publishDate | 1998-09-01 |
| publisher | Thieme Revinter Publicações |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj-art-aa6ca5c86c4a4eb59f5c68942ce0601c2025-08-20T03:19:02ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-42271998-09-01563A35636510.1590/S0004-282X1998000300003Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite casesCARLOS M. DE CASTRO COSTA0JEAN MARIE BRUCHER1CHRISTIAN LATERRE2Universidade Federal do CearáCatholic University of LouvainCatholic University of LouvainThe authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56%) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1998000300003&tlng=ensporadic Creutzfeldt-Jakob diseasedementiaperiodic EEGneuropathologyprion protein (PrP) amyloid plaqueprion disease |
| spellingShingle | CARLOS M. DE CASTRO COSTA JEAN MARIE BRUCHER CHRISTIAN LATERRE Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases Arquivos de Neuro-Psiquiatria sporadic Creutzfeldt-Jakob disease dementia periodic EEG neuropathology prion protein (PrP) amyloid plaque prion disease |
| title | Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases |
| title_full | Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases |
| title_fullStr | Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases |
| title_full_unstemmed | Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases |
| title_short | Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases |
| title_sort | sporadic creutzfeldt jakob disease a clinico neuropathological analysis of nine definite cases |
| topic | sporadic Creutzfeldt-Jakob disease dementia periodic EEG neuropathology prion protein (PrP) amyloid plaque prion disease |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1998000300003&tlng=en |
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