Magnetic resonance imaging in cerebrotendinous xanthomatosis: a prospective clinical and neuroradiological study.
J Neurol Sci
; 122(1): 102-8, 1994 Mar.
Article
em En
| MEDLINE
| ID: mdl-8195796
ABSTRACT
We describe MRI findings in 13 persons with typical clinical, EEG, CT and biochemical features of cerebrotendinous xanthomatosis (CTX). MRI showed brain (13/13) and cerebellar (12/13) atrophy and diffuse white matter hypodensity (4/13) presumably reflecting sterol infiltration with demyelination. Focal lesions were rare (2/13). Mass effect, edema or enhancement were not observed. Treatment with chenodeoxycholic acid (CDCA) 750 mg/day orally improved neurological and biochemical abnormalities. MRI appears to be of little value in following improvement after treatment has begun. Otherwise, the MRI studies are very sensitive and useful in diagnosing early incomplete forms of CTX.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Xantomatose
Tipo de estudo:
Observational_studies
Limite:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
J Neurol Sci
Ano de publicação:
1994
Tipo de documento:
Article