N-acetylaspartylglutamate in CNS hypomyelination

Neuropediatrics. 2011 Feb;42(2):74-7. doi: 10.1055/s-0031-1277176. Epub 2011 May 3.


CSF N-acetylaspartylglutamate (NAAG) has been found to be elevated in some hypomyelinating disorders. This study addressed the question whether it could be used as a marker for hypomyelination and as a means to distinguish between hypomyelinating disorders biochemically. We have measured CSF NAAG in a cohort of 28 patients with hypomyelination with known and unknown aetiology. NAAG was found to be elevated in 7 patients, but was normal in the majority, including patients with defined hypomyelinating disorders. CSF NAAG is not a universal marker of hypomyelination, and the mechanism of its elevation remains poorly understood.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cohort Studies
  • Demyelinating Diseases / cerebrospinal fluid*
  • Dipeptides / cerebrospinal fluid*
  • Female
  • Humans
  • Infant
  • Leukoencephalopathies / cerebrospinal fluid*
  • Magnetic Resonance Imaging
  • Male
  • Tritium / cerebrospinal fluid
  • Young Adult


  • Dipeptides
  • Tritium
  • isospaglumic acid