Thalamic and basal ganglia hyperdensities--a CT marker for globoid cell leukodystrophy?
Neuropediatrics
; 23(1): 30-1, 1992 Feb.
Article
in En
| MEDLINE
| ID: mdl-1565214
ABSTRACT
Globoid cell leukodystrophy (Krabbe's disease) is a rare autosomal recessive lipidosis, with signs restricted to the nervous system, and is caused by deficiency of the lysosomal hydrolase galactocerebroside beta-galactosidase (galactocerebrosidase). In recent years there have been reports of neurological variants, where age of onset and manifestations differed from the classical form. In other cases, specific CT findings, mainly hyperdensities, have been seen. We report a case of late-infantile Krabbe's disease where thalamic and basal ganglia hyperdensities have been seen on CT scan. We also stress that clinicians should be aware of the possibility of Krabbe's disease in infants who display progressive encephalopathy and these CT findings.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Thalamus
/
Basal Ganglia
/
Tomography, X-Ray Computed
/
Leukodystrophy, Globoid Cell
Limits:
Female
/
Humans
/
Infant
Language:
En
Journal:
Neuropediatrics
Year:
1992
Document type:
Article
Affiliation country:
Brazil