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

doi:10.1007/s00247-003-1058-7

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 journal › Article › peer-review

39 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 language	English
Pages (from-to)	163-166
Number of pages	4
Journal	Pediatric Radiology
Volume	34
Issue number	2
DOIs	https://doi.org/10.1007/s00247-003-1058-7
Publication status	Published - 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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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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AU - Lee, Ki Yeol

AU - Seol, Hae Young

AU - Cha, Sang Hoon

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AB - 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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KW - Diffusion-weighted MRI

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

Abstract

Keywords

ASJC Scopus subject areas

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