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

33 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

Access to Document

10.1007/s00247-003-1058-7

Fingerprint Dive into the research topics of 'Maple syrup urine disease encephalopathy: A follow-up study in the acute stage using diffusion-weighted MRI'. Together they form a unique fingerprint.

Cite this

@article{92aa1ce182d34f2aa7e452db6301abe8,

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

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.",

keywords = "Brain, Diffusion-weighted MRI, Maple syrup urine disease, Metabolic, Neonate",

author = "Ha, {Jong Su} and Kim, {Taik Kun} and Eun, {Baik Lin} and Lee, {Hee Sun} and Lee, {Ki Yeol} and Seol, {Hae Young} and Cha, {Sang Hoon}",

year = "2004",

month = feb,

doi = "10.1007/s00247-003-1058-7",

language = "English",

volume = "34",

pages = "163--166",

journal = "Pediatric Radiology",

issn = "0301-0449",

publisher = "Springer Verlag",

number = "2",

}

TY - JOUR

T1 - Maple syrup urine disease encephalopathy

T2 - A follow-up study in the acute stage using diffusion-weighted MRI

AU - Ha, Jong Su

AU - Kim, Taik Kun

AU - Eun, Baik Lin

AU - Lee, Hee Sun

AU - Lee, Ki Yeol

AU - Seol, Hae Young

AU - Cha, Sang Hoon

PY - 2004/2

Y1 - 2004/2

N2 - 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.

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.

KW - Brain

KW - Diffusion-weighted MRI

KW - Maple syrup urine disease

KW - Metabolic

KW - Neonate

UR - http://www.scopus.com/inward/record.url?scp=1042291995&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=1042291995&partnerID=8YFLogxK

U2 - 10.1007/s00247-003-1058-7

DO - 10.1007/s00247-003-1058-7

M3 - Article

C2 - 14504844

AN - SCOPUS:1042291995

VL - 34

SP - 163

EP - 166

JO - Pediatric Radiology

JF - Pediatric Radiology

SN - 0301-0449

IS - 2

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Cite this