Saturday 4 June 2016

Pelizaeus-Merzbacher disease

Q.All are true regarding Pelizaeus-Merzbacher disease except

a.due to lack of lipophilin

b.patchy demyelination with sparing of perivascular white matter

c.preserved internal capsule and subcortical U fibres

d.diffuse low signal on T2w

e.hypointense basal ganglia and thalamus on T2W

ANS.---d


Severe cases of Pelizaeus-Merzbacher disease show near –total 

lack of normal myelination with  diffuse high signal on T2W image

 that extends peripherally to involve the arcuate fibres .

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