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Figure Legends:
The presence of the molar tooth sign
on axial MRI through the malformed pontomesencephalic
junction (isthmus). The molar tooth sign consists of the
following triad: (1) deepening of the interpeduncular
fossa (*), (2) thick and straight superior cerebellar
peduncles (arrows), and (3) hypoplastic vermis.
Molar tooth sign on axial T1-weighted section through
the superior cerebellar peduncles. Joubert syndrome is
characterized by two features in particular: enlarged
superior cerebellar peduncles that do not decussate in
the isthmus portion of the brain stem and cerebellar
vermis dysplasia. This axial section illustrates the
thickened superior cerebellar peduncles (arrows). Since
they do not decussate, isthmus is then reduced and the
interpeduncular fossa is secondarily enlarged (*).
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