Q.All are true
regarding malformations except
a. The
posterior fossa is of normal size in cerebellar hypoplasia
b. Torcular–lambdoid
inversion on MRI is seen in Dandy –Walker
syndrome
syndrome
d.The Molar
tooth appearance is noted in rhombencephalosynapsis
feature of L'Hermitte-Duclos
ANS .---d
Joubert's syndrome is considered as generalized developmental disorder of the midbrain and hindbrain. The imaging findings reflect a failure of formation of the decussation of the superior cerebellar peduncles, lack of the pyramidal decussations and other anomalies of the midbrain crossing tracts and their nuclei. On cross-sectional imaging the fourth ventricle is enlarged with a ‘batwing appearance’ and there is a cleft in the vermis. The midbrain is small. The ‘molar tooth’ appearance seen on axial images arises from the lack of the superior cerebellar decussation and the superior peduncles also appear enlarged.
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