Inborn metabolic brain disorders

Q.All are true regarding inborn metabolic brain disorders except

a. Alexander's disease shows  extensive white matter abnormality beginning in the frontal and periventricular white matter

b. l-2 hydroxyglutaricaciduria  involve   the periventricular white matter and corpus callosum

c. bilateral symmetrical involvement of the globus pallidus with sparing of the thalami is seen in methylmalonic acidaemia

d. Bilateral pallidal involvement is  seen as T2 hyperintensity  in Kearns–Sayer syndrome

e. Lorenzo's oil may delay disease progression of ALD

ANS .---b

The MRI findings of  l-2 hydroxyglutaricaciduria show white matter involvement with peripheral involvement, particularly of the subcortical U fibres, internal, external and extreme capsules, sparing of the periventricular white matter and corpus callosum, and with a slight frontal predominance. There is macrocephaly

Bilateral pallidal involvement is  seen as T2 hyperintensity in methylmalonic acidaemia ,GAMT (guanidinoacetate methyltransferase deficiency), Kearns–Sayer syndrome ,kernicterus and carbon monoxide poisoning