Alexanders Disease

Rare Leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the Brain. Rosenthal fibers are Glial Fibrillary Acidic Protein aggregates found in Astrocytes. Juvenile- and Adult-onset types show progressive Atrophy of the lower Brainstem instead. De novo Mutations in the GFAP Gene are associated with the Disease with propensity for paternal inheritance.