Serial MR imaging, diffusion tensor imaging, and MR spectroscopic findings in a child with progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome

AJNR Am J Neuroradiol. 2006 Aug;27(7):1555-8.

Abstract

PEHO syndrome is a rare symptom complex of severe progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy. Disease onset is in early infancy. The current case report presents and discusses serial conventional MR imaging findings and serial functional studies including diffusion tensor imaging and quantitative MR spectroscopy findings in a 6-year-old child with PEHO.

Publication types

  • Case Reports

MeSH terms

  • Aspartic Acid / analogs & derivatives
  • Aspartic Acid / analysis
  • Atrophy
  • Brain Diseases / diagnosis*
  • Brain Edema / diagnosis
  • Cerebellum / pathology
  • Child
  • Choline / analysis
  • Creatine / analysis
  • Diffusion Magnetic Resonance Imaging*
  • Edema / diagnosis*
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Lactic Acid / analysis
  • Magnetic Resonance Imaging*
  • Magnetic Resonance Spectroscopy*
  • Neurodegenerative Diseases / diagnosis
  • Optic Atrophy / diagnosis*
  • Spasms, Infantile / diagnosis*
  • Syndrome

Substances

  • Aspartic Acid
  • Lactic Acid
  • N-acetylaspartate
  • Creatine
  • Choline