MR imaging of pelizaeus-merzbacher disease

Monte W. Penner, King C. Li, Stephen S. Gebarski, Richard J. Allen

Research output: Contribution to journalArticlepeer-review

Abstract

Pelizaeus-Merzbacher disease (PMD) is a rare, slowly progressive, sex-linked dysmyelinating disorder generally classified with the sudanophilic leukodystrophies. The onset is most often in the pediatric age group and may be diagnosed as cerebral palsy because of the subtle onset. Cranial magnetic resonance (MR) imaging of two patients with PMD showed reversal of the normal gray/white matter signal relationships, consistent with dysmyelination, as well as low intensity lentiform nuclei and thalami possibly suggesting pathologic iron deposition. Magnetic resonance also better demonstrated low volume brain without the beam hardening limitations of X-ray CT. Although our MR findings correlate well with the pathophysiology of PMD, the MR characteristics are not specific. The diagnosis of PMD remains one of clinical and laboratory exclusion.

Original languageEnglish (US)
Pages (from-to)591-593
Number of pages3
JournalJournal of Computer Assisted Tomography
Volume11
Issue number4
DOIs
StatePublished - Jan 1 1987
Externally publishedYes

Keywords

  • Central nervous system, diseases
  • Leukodystrophy
  • Magnetic resonance imaging
  • Merzbacher disease
  • Pelizaeus

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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