RADIOLOGY MCQ ---FRCR

189.Hypointense subependymal germinolytic cysts ,typically

 involving the caudothalamic grooves is seen on MR imaging of

a.Mucopolysaccharidoses

b.Zellweger Syndrome

c.Fabry Disease

d.Krabbe disease

e.MLD

ANS-----b --Zellweger Syndrome

Zellweger syndrome (ZS) is a peroxisomal disorder characterized by hepatomegaly, high levels of copper/iron, and visual symptoms

Hypointense subependymal germinolytic cysts (typically involving the caudothalamic grooves) is noted on on MR imaging of Zellweger Syndrome.Other associated MR finding are microgyria (predominantly in the frontal and perisylvian cortex), polymicrogyria, and pachygyria (particularly in the perirolandic and occipital regions).

MR imaging might be  useful for the in utero diagnosis of this condition (during the third trimester), showing abnormal cortical gyral patterns, abnormal myelin formation, and cerebral periventricular pseudocysts . MR spectra show similar peak heights of the two lipid peaks (CH2, CH3), elevated choline levels, and low NAA