ADULT-ONSET KRABBES-LEUKODYSTROPHY - A REPORT OF 2 CASES

Citation
M. Turazzini et al., ADULT-ONSET KRABBES-LEUKODYSTROPHY - A REPORT OF 2 CASES, Acta neurologica Scandinavica, 96(6), 1997, pp. 413-415
Citations number
23
Categorie Soggetti
Clinical Neurology
ISSN journal
00016314
Volume
96
Issue
6
Year of publication
1997
Pages
413 - 415
Database
ISI
SICI code
0001-6314(1997)96:6<413:AK-ARO>2.0.ZU;2-P
Abstract
Krabbe's disease with adult onset is rare; neurological symptoms begin in childhood or at a juvenile age. Two brothers with adult onset of t he disease are here reported; 1 sibling developed parapareto-ataxic ga it while the other was asymptomatic. Magnetic resonance imaging showed areas of demyelination in the white matter of the brain, while nerve conduction was completely normal. In both patients deficiency of galac tosylceramide beta-galactosidase was comparable to the infantile form.