Vacuolating megalencephalic leukoencephalopathy with mild clinical course validated by diffusion tensor imaging and MR spectroscopy

Yi Fang Tu, Cheng Yu Chen, Chao Ching Huang, Chang Shin Lee

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

We report a case of presumed vacuolating megalencephalic leukoencephalopathy (VML) in a 5-year-old boy that was diagnosed when the patient was 34 months old. The patient presented clinically with megalencephaly and delayed onset of slowly progressive neurologic dysfunction. Serial MR imaging and biochemical analysis were performed to differentiate VML from other disease entities with megalencephaly and leukoencephalopathy of infantile onset. Information obtained with diffusion tensor MR imaging revealed nearly intact white matter anisotropy and vectors in parietal and posterior temporal lobes in which hyperintense abnormality was shown on conventional T2-weighted images, and proton MR spectra showed a significant decrease in N-acetylaspartate-creatine + phosphocreatine ratio.

Original languageEnglish
Pages (from-to)1041-1045
Number of pages5
JournalAmerican Journal of Neuroradiology
Volume25
Issue number6
Publication statusPublished - Jun 2004
Externally publishedYes

Fingerprint

Diffusion Tensor Imaging
Magnetic Resonance Spectroscopy
Megalencephaly
Phosphocreatine
Creatine
Anisotropy
Temporal Lobe
Neurologic Manifestations
Protons
Megalencephalic leukoencephalopathy with subcortical cysts

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

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abstract = "We report a case of presumed vacuolating megalencephalic leukoencephalopathy (VML) in a 5-year-old boy that was diagnosed when the patient was 34 months old. The patient presented clinically with megalencephaly and delayed onset of slowly progressive neurologic dysfunction. Serial MR imaging and biochemical analysis were performed to differentiate VML from other disease entities with megalencephaly and leukoencephalopathy of infantile onset. Information obtained with diffusion tensor MR imaging revealed nearly intact white matter anisotropy and vectors in parietal and posterior temporal lobes in which hyperintense abnormality was shown on conventional T2-weighted images, and proton MR spectra showed a significant decrease in N-acetylaspartate-creatine + phosphocreatine ratio.",
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AB - We report a case of presumed vacuolating megalencephalic leukoencephalopathy (VML) in a 5-year-old boy that was diagnosed when the patient was 34 months old. The patient presented clinically with megalencephaly and delayed onset of slowly progressive neurologic dysfunction. Serial MR imaging and biochemical analysis were performed to differentiate VML from other disease entities with megalencephaly and leukoencephalopathy of infantile onset. Information obtained with diffusion tensor MR imaging revealed nearly intact white matter anisotropy and vectors in parietal and posterior temporal lobes in which hyperintense abnormality was shown on conventional T2-weighted images, and proton MR spectra showed a significant decrease in N-acetylaspartate-creatine + phosphocreatine ratio.

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