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MR of childhood metachromatic leukodystrophy.

Kim, TS; Kim, IO; Kim, WS; Choi, YS; Lee, JY; Kim, OW; Yeon, KM; Kim, KJ; Hwang, YS
AJNR. American journal of neuroradiology, 18(4):733-738, 1997
Journal Title
AJNR. American journal of neuroradiology
PURPOSE: To investigate the MR findings of childhood metachromatic leukodystrophy (MLD).

METHODS: Nine MR imaging studies in seven children (five girls and two boys, 10 to 32 months old) with MLD were evaluated retrospectively for the extent and progression of white matter abnormalities and the presence of contrast enhancement.

RESULTS: All seven cases showed symmetric, confluent high signal intensity on T2-weighted images in the periventricular white matter and centrum semiovale. A posterior predominance of white matter abnormalities was noted in all cases. Although initially spared from demyelination in all cases, in one case, the subcortical U fibers were later involved in demyelination of follow-up MR studies. Other sites of involvement were the genu (n = 5) and splenium (n = 6) of the corpus callosum, the posterior limbs of the internal capsule (n = 5), the descending pyramidal tracts (n = 4), the claustrum (n = 4), and the cerebral white matter (n = 2); diffuse brain atrophy was seen in two cases. No enhancement of the lesion was seen on any of the five postcontrast examinations. A "tigroid" pattern, previously described in cases of Pelizaeus-Merzbacher disease, was noted in the centrum semiovale in six cases.

CONCLUSION: In late-infantile MLD, demyelination is more prominent in the occipital region. In addition to demyelination of the periventricular white matter, common manifestations include a "tigroid" pattern and involvement of the corpus callosum, the internal capsule, and the corticospinal tract.
MeSH terms
Brain/*pathologyCerebellum/pathologyCerebral Cortex/pathologyCerebral Ventricles/pathologyChild, PreschoolContrast MediaCorpus Callosum/pathologyDemyelinating Diseases/pathologyDiffuse Cerebral Sclerosis of Schilder/diagnosis/pathologyDisease ProgressionFemaleFollow-Up StudiesHumansImage EnhancementInfantLeukodystrophy, Metachromatic/diagnosis/*pathology*Magnetic Resonance ImagingMaleOccipital Lobe/pathologyPyramidal Tracts/pathologyRetrospective Studies
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Journal Papers > School of Medicine / Graduate School of Medicine > Radiology
AJOU Authors
김, 옥화
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