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MR of childhood metachromatic leukodystrophy.
AJNR Am J Neuroradiol. 1997 Apr; 18(4):733-8.AA

Abstract

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.

Authors+Show Affiliations

Department of Radiology, Seoul National University, College of Medicine, Korea.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

9127040

Citation

Kim, T S., et al. "MR of Childhood Metachromatic Leukodystrophy." AJNR. American Journal of Neuroradiology, vol. 18, no. 4, 1997, pp. 733-8.
Kim TS, Kim IO, Kim WS, et al. MR of childhood metachromatic leukodystrophy. AJNR Am J Neuroradiol. 1997;18(4):733-8.
Kim, T. S., Kim, I. O., Kim, W. S., Choi, Y. S., Lee, J. Y., Kim, O. W., Yeon, K. M., Kim, K. J., & Hwang, Y. S. (1997). MR of childhood metachromatic leukodystrophy. AJNR. American Journal of Neuroradiology, 18(4), 733-8.
Kim TS, et al. MR of Childhood Metachromatic Leukodystrophy. AJNR Am J Neuroradiol. 1997;18(4):733-8. PubMed PMID: 9127040.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - MR of childhood metachromatic leukodystrophy. AU - Kim,T S, AU - Kim,I O, AU - Kim,W S, AU - Choi,Y S, AU - Lee,J Y, AU - Kim,O W, AU - Yeon,K M, AU - Kim,K J, AU - Hwang,Y S, PY - 1997/4/1/pubmed PY - 1997/4/1/medline PY - 1997/4/1/entrez SP - 733 EP - 8 JF - AJNR. American journal of neuroradiology JO - AJNR Am J Neuroradiol VL - 18 IS - 4 N2 - 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. SN - 0195-6108 UR - https://www.unboundmedicine.com/medline/citation/9127040/MR_of_childhood_metachromatic_leukodystrophy_ L2 - http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=9127040 DB - PRIME DP - Unbound Medicine ER -