Water apparent diffusion coefficient and T2 changes in the acute stage of maple syrup urine disease: Evidence of intramyelinic and vasogenic-interstitial edema

Andrea Righini, Luca A. Ramenghi, Rossella Parini, Fabio Triulzi, Fabio Mosca

Research output: Contribution to journalArticlepeer-review

Abstract

Background. The acute phase of the neonatal classical form of maple syrup urine disease (MSUD) is usually associated with generalized brain edema. Methods and Results. The authors present the case of a new-born infant in the acute stage of the classical form of MSUD in whom a remarkable decrease in the water apparent diffusion coefficient (ADC) in advanced myelinating white matter areas was associated with an increase in the T2 signal. This diffusion magnetic resonance imaging (MRI) pattern appears to be compatible with a rare kind of cytotoxic edema, the so-called intramyelinic edema. At the same time, an increase in the ADC was seen in unmyelinated areas together with an increase in the T2 signal, a sign of a coexistent vasogenic-interstitial edema. Conclusions. ADC measurements in MSUD provide more specific information than conventional MRI about the pathophysiology of white matter changes.

Original languageEnglish
Pages (from-to)162-165
Number of pages4
JournalJournal of Neuroimaging
Volume13
Issue number2
DOIs
Publication statusPublished - Apr 2003

Keywords

  • Diffusion-weighted imaging
  • Maple syrup urine disease
  • Neonate

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Neuroscience(all)
  • Radiological and Ultrasound Technology

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