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"ALEXANDER DISEASE"

“ Presents in infancy. Histology shows increased density of Rosenthal fibres. MRI •   Progressive demyelination beginning with the frontal l...

Presents in infancy. Histology shows increased density of Rosenthal fibres.
MRI

•  Progressive demyelination beginning with the frontal lobes and eventually involving the entire hemispheres

•  Enhancement following contrast administration is seen in Alexander’s disease
•  Brain enlargement,macrocephaly