leukodystrophy

(redirected from Leucodystrophy)
Also found in: Dictionary, Encyclopedia.

leukodystrophy

 [loo″ko-dis´tro-fe]
disturbance of the white substance of the brain. See also adrenoleukodystrophy and leukoencephalopathy.
metachromatic leukodystrophy a hereditary leukoencephalopathy, marked by accumulation of a sphingolipid (sulfatide) in tissues, with diffuse loss of myelin in the central nervous system and progressive dementia and paralysis; classified according to age of onset as infantile, juvenile, and adult.

leu·ko·dys·tro·phy

(lū'kō-dis'trŏ-fē),
Term for a group of white matter diseases, some familial, characterized by progressive cerebral deterioration usually in early life, and pathologically by primary absence or degeneration of the myelin of the central and peripheral nervous systems with glial reaction; probably related to a defect in lipid metabolism; most leukodystrophies are autosomal recessive, several are X-linked recessive, and a few are autosomal dominant.
See also: Canavan disease.
[leuko- + G. dys, bad, + trophē, nourishment]

leukodystrophy

(lo͞o′kō-dĭs′trə-fē)
n.
Any of various genetic, usually inherited diseases characterized by a defect in the development or maintenance of myelin.

leukodystrophy

Neurology A heterogeneous group of disorders of cerebral myelin or its metabolism–eg, Krabbe's disease, metachromatic leukodystrophy, 'sphingolipidoses', that share certain pathological features Clinical 'White matter disease', ie predominantly motor, dominated by progressive paralysis and ataxia rather than dementia. See Globoid cell leukodystrophy.

leu·ko·dys·tro·phy

(lū'kō-dis'trŏ-fē)
A group of white matter diseases, some familial, characterized by progressive cerebral deterioration in early life and primary absence or degeneration of the myelin of the central and peripheral nervous systems; probably related to a defect in lipid metabolism; the adult type of Pelizaeus-Merzbacher disease is inherited as an autosomal dominant trait.
Synonym(s): leucodystrophy.
[leuko- + G. dys, bad, + trophē, nourishment]
References in periodicals archive ?
of Percentage of Substrate Patients Distribution Infectious Aetiology 13 30.23% Gliosis 6 13.95% Vascular Disease 5 11.63% Tumours 4 9.30% Congenital Malformations 4 9.30% Phakomatoses 3 6.98% Encephalitis 3 6.98% Mesial Temporal Sclerosis 2 4.65% Leucodystrophy 2 4.65% Vascular Malformations 1 2.33% Total 43 Table 5: Distribution of Cases According to Sex Sex No.
Adult-onset autosomal dominant leucodystrophy (ADLD; OMIM = 169500) was first reported in an American-Irish kindred in 1984.
Remote symptomatic 16 cases (34.8%) -Nonprogressive -cerebral palsy 7 -epilepsy 1 -cerebral venousmalformations 1 -bilateral occipital gliosis 1 -periventricular leucomalacia 1 -previous intracranial bleed 2 -Avm of left occipital lobe 1 Progressive -metachromatic leucodystrophy 1 -Rasmussen encephalitis 1 3.
Lysosomal storage Gaucher's disease, disorders Niemann-Pick disease Disorders of lipid Fatty acid Oxidation metabolism Defects, Sphingolipidoses Mitochondrial Kearns-Sayre syndrome disorders Peroxisomal Zellweger syndrome, disorders Adreno leucodystrophy Trace metal Menke's Kinky Hair disorders syndrome, Wilson's disease Chart 1: Provisional diagnosis No diagnosis 1 Hypothyroidism 2 Wilsons disease 1 Pyrexia for evaluation 1 Inborn errors of metabolism 14 Hepatomegaly for evaluation 7 Hematological Malignancy 1 Hemolytic anemia 6 Note: Table made from bar graph.