Document Detail


Creutzfeldt-Jakob disease. A survey of 14 patients.
MedLine Citation:
PMID:  9201337     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Creutzfeldt-Jakob disease (CJD) is a transmissible disease of the nervous system causatively related to the presence of an abnormal prion protein, with dementia, myoclonic jerks, and periodic EEG activity. Fourteen patients (7 females and 7 males) ranging from 26 to 76 years of age (median 59 years) were evaluated between 1974 and 1995 at the Neurologic Clinic of São Paulo University School of Medicine. The average duration of the disease was 12 months (3.5-34 months). Early clinical findings were: behaviour changes in 7 patients, dementia in 4, visual disturbances in 4, vertigo in 2, tremor in 9, and dystonia in one. Advanced symptoms were dementia and myoclonus in all patients. Pyramidal tract dysfunction was found in 6, cerebellar ataxia in 2, seizures in 3, nystagmus and vertigo in 4, and peripheral nervous system involvement in 2. Atypical clinical forms were found in 5 patients. Periodic EEG activity was found in 10 patients. Cerebrospinal fluid evaluation showed pleocytosis in 1 patient, higher protein content in 2, and higher gamma globulin level in 2. In 10 patients anatomopathological evidence in the central nervous system confirmed the clinical diagnosis by presenting with status spongiosus. All except one patient presented with the sporadic form of the disease.
Authors:
P E Marchiori; N Yasuda; H C Azevedo; M Orfão; D Callegaro; F I Yamamoto; M Scaff
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Arquivos de neuro-psiquiatria     Volume:  54     ISSN:  0004-282X     ISO Abbreviation:  Arq Neuropsiquiatr     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-10-27     Completed Date:  1997-10-27     Revised Date:  2009-11-11    
Medline Journal Info:
Nlm Unique ID:  0125444     Medline TA:  Arq Neuropsiquiatr     Country:  BRAZIL    
Other Details:
Languages:  eng     Pagination:  577-83     Citation Subset:  IM    
Affiliation:
Neurologic Clinic of the São Paulo University Medical School (FMUSP), São Paulo SP, Brasil.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Creutzfeldt-Jakob Syndrome / diagnosis*
Disease Progression
Female
Humans
Male
Middle Aged

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