title: Adult type of leukodystrophy. Krabbe's disease?
abstract: A 24-year-old man developed progressive dementia in seven years. The patient suffered a severe bronchopneumonia and eventually died few days later. Brain coronal sections showed a soft gray-brownish discoloration of white matter of centrum ovale but the subcortical arcuate fibers and the interne capsule were preserved. Microscopically, the white matter showed marked loss of myelin and oligodendrocytes, abundant hypertrophic astrocytes and numerous "globoid cells". The latter showed strong positivity in immunostains for a mouse monoclonal antigalactocerebroside antibody. The presence of these cells in the brain white matter might be the morphological basis to classify the present case as one of Krabbe's Leukodystrophy.
authors.affiliation: Department of Pathology, Hostpiral Clinico y Provincial, Barcelona, Spain.
authors.forename: F F
authors.initials: FF
authors.lastname: Cruz-Sanchez
authors.forename: J A
authors.initials: JA
authors.lastname: Martos
authors.forename: A
authors.initials: A
authors.lastname: Rives
authors.forename: T
authors.initials: T
authors.lastname: Ribalta
authors.forename: I
authors.initials: I
authors.lastname: Ferrer
authors.forename: A
authors.initials: A
authors.lastname: Cardesa
mesh_terms: D000328:Adult; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D005699:Galactosylceramides / Q000032:analysis; D006801:Humans; D007150:Immunohistochemistry; D007965:Leukodystrophy, Globoid Cell / Q000378:metabolism* / Q000473:pathology; D008297:Male
chemical_list: D005699:Galactosylceramides