Very-late-onset adrenoleukodystrophy: possible precipitation of demyelination by cerebral contusion

Neurology. 1992 Feb;42(2):367-70. doi: 10.1212/wnl.42.2.367.

Abstract

We report the clinical history, neuroradiology, and autopsy findings in a patient with very late onset, at age 57, of adrenoleukodystrophy (ALD) presenting with dementia, hemianopia, and gait apraxia. Open brain biopsy, elevated plasma very-long-chain fatty acids, and autopsy confirmed the diagnosis of ALD. Demyelinative lesions were most extensive at the site of a recent cerebral contusion. CNS trauma may have precipitated or accelerated demyelination in this patient carrying the ALD gene.

Publication types

  • Case Reports

MeSH terms

  • Adrenoleukodystrophy / diagnostic imaging
  • Adrenoleukodystrophy / pathology
  • Adrenoleukodystrophy / physiopathology*
  • Age Factors
  • Brain Concussion / complications*
  • Demyelinating Diseases / etiology*
  • Fatty Acids / blood
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Tomography, X-Ray Computed

Substances

  • Fatty Acids