Maple syrup urine disease encephalopathy: A follow-up study in the acute stage using diffusion-weighted MRI

Jong Su Ha, Taik Kun Kim, Baik Lin Eun, Hee Sun Lee, Ki Yeol Lee, Hae Young Seol, Sang Hoon Cha

    Research output: Contribution to journalArticlepeer-review

    34 Citations (Scopus)

    Abstract

    Neonatal maple syrup urine disease (MSUD) is associated with diffuse oedema and characteristic MSUD oedema. We present a newborn infant with two coexisting different types of oedema. The myelinated white matter showed a marked decrease in the water apparent diffusion coefficient (ADC) compatible with cytotoxic oedema. The unmyelinated white matter showed an increase in ADC, consistent with vasogenic-interstitial oedema. On follow-up studies, the cytotoxic oedema showed improvement, but the vasogenic-interstitial oedema progressed into brain atrophy.

    Original languageEnglish
    Pages (from-to)163-166
    Number of pages4
    JournalPediatric Radiology
    Volume34
    Issue number2
    DOIs
    Publication statusPublished - 2004 Feb

    Keywords

    • Brain
    • Diffusion-weighted MRI
    • Maple syrup urine disease
    • Metabolic
    • Neonate

    ASJC Scopus subject areas

    • Pediatrics, Perinatology, and Child Health
    • Radiology Nuclear Medicine and imaging

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