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

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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.

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Authors and Affiliations

  1. Department of Diagnostic Radiology, Ansan Hospital, Korea University College of Medicine, #516 Kojan-Dong, 425-020, Ansan City, Kyungki-Do, Korea

    Jong Su Ha, Taik-Kun Kim, Ki Yeol Lee, Hae Young Seol & Sang Hoon Cha

  2. Department of Paediatrics, Korea University College of Medicine, Seoul, Korea

    Baik-Lin Eun & Hee Sun Lee

Authors
  1. Jong Su Ha
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  2. Taik-Kun Kim
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  3. Baik-Lin Eun
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  4. Hee Sun Lee
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  5. Ki Yeol Lee
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  6. Hae Young Seol
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  7. Sang Hoon Cha
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Correspondence to Taik-Kun Kim.

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Ha, J.S., Kim, TK., Eun, BL. et al. Maple syrup urine disease encephalopathy: a follow-up study in the acute stage using diffusion-weighted MRI. Pediatr Radiol 34, 163–166 (2004). https://doi.org/10.1007/s00247-003-1058-7

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  • DOI: https://doi.org/10.1007/s00247-003-1058-7

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