Alexander disease(redirected from Demyelinogenic leukodystrophy)
Al·ex·an·der dis·ease(al-ek-zan'dĕr), [MIM*203450]
a rare, fatal degenerative disease of the central nervous system of infants, characterized by psychomotor retardation, seizures, and paralysis; megaloencephaly is associated with widespread leukodystrophic changes, especially in the frontal lobes.
Farlex Partner Medical Dictionary © Farlex 2012
Alexander diseaseA rare, progressive leukoencephalopathy (OMIM:203450) of the CNS, histologically characterised by widespread accumulation of Rosenthal fibres (cytoplasmic inclusions in astrocytes) and failure of central myelination. The most common form develops in infancy, and is accompanied by megalencephaly, developmental delay, spasticity, seizures and psychomotor retardation. Patients with juvenile or adult forms experience ataxia, bulbar signs and spasticity and slower progression.
Defects in GFAP, which encodes a major intermediate filament, cause Alexander disease.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
Al·ex·an·der dis·ease(al-ĕg-zan'dĕr di-zēz')
A rare, fatal central nervous system degenerative disease of infants, characterized by psychomotor retardation, seizures, and paralysis; megaloencephaly is associated with widespread leukodystrophic changes, especially in the frontal lobes.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
Alexander,William Stuart, 20th century New Zealand pathologist.
Alexander disease - a rare, fatal, central nervous system degenerative disease of infants.
Medical Eponyms © Farlex 2012