Alexander Disease - Pathology

Pathology

Alexander disease causes the affected person to slowly begin to lose body function and eventually the ability to talk. It also causes an overload of long chain fatty acids that the body cannot dispose of. This overload of fatty acids builds up in the brain and destroys the myelin sheath. The cause of Alexander disease is a mutation in the gene encoding glial fibrillary acidic protein.

CT shows:

  • decreased density of white matter
  • frontal lobe predominance
  • +/- dilated lateral ventricles

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