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Alexander disease

Alexander disease (dysmyelogenic leukodystrophy) is a progressive fatal neurodegenerative disease of childhood characterized by a deficit in axonal myelination affecting the white matter of the brain with Rosenthal fibres formation.

The condition is associated with mutations in the coding region of the GFAP gene on chromosome 17q21. Most cases are sporadic.

Last updated: May 31, 2012, 12:59 pm EST

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