Q.All are true regarding Alexander disease except
a.enlarged
brain
b. substantial accumulation
of Rosenthal fibers
c. The basal ganglia and cortex usually relatively preserved
d. sparing of the subcortical U fibers
e. Cavitation common
ANS.---d
There
is no sparing of the subcortical U fibers
in Alexander disease. The cerebellum is less often affected than in
other leukodystrophies. MR findings classically demonstrate increased signal
intensity on the T2-weighted images in the frontal white matter .The occipital
white matter and cerebellum are usually spared.
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