Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17
Arq. neuropsiquiatr
;
59(2A): 161-164, June 2001. ilus, tab
Article
in English
| LILACS
| ID: lil-288609
RESUMO
OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD), the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 Ý 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Chromosomes, Human, Pair 17
/
Creutzfeldt-Jakob Syndrome
/
Parkinsonian Disorders
Type of study:
Diagnostic study
Limits:
Adult
/
Female
/
Humans
/
Male
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
2001
Type:
Article
Affiliation country:
Brazil
/
Canada
Institution/Affiliation country:
McGill University/CA
/
State University of Londrina/BR
/
University of Säo Paulo/BR
Similar
MEDLINE
...
LILACS
LIS