[MRI and computer-assisted tomography in Kufs disease. Apropos of a familial form]. / IRM et scanographie de la maladie de Kufs. A propos d'une forme familiale.
J Neuroradiol
; 23(1): 33-7, 1996 Jun.
Article
em Fr
| MEDLINE
| ID: mdl-8767916
ABSTRACT
Kufs disease is the adult form of ceroid neurolipofuscinosis, and an uncommon cause of degenerative nervous system disease affecting young adults. We present here 4 cases of family form revealed by a demential syndrome. In all 4 patients MRI showed diffuse cortical atrophy predominant in the parietal regions. In 3 of these 4 patients MRI also exhibited a low signal in T2-weighted sequences on the putamens. There was no abnormality of the white matter. Diagnosis was made by cerebral biopsy in one case and by rectal biopsy in all other cases. Although the MRI images are not specific, they must be used when the diagnosis of Kufs disease is suspected in young patients with demential syndrome.
Buscar no Google
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Imageamento por Ressonância Magnética
/
Tomografia Computadorizada por Raios X
/
Lipofuscinoses Ceroides Neuronais
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
Fr
Revista:
J Neuroradiol
Ano de publicação:
1996
Tipo de documento:
Article