Joubert’s syndrome with Dandy walker variant (MR).

PROTOCOL:

T1W – Sagittal & axial scans
T2W – Coronal & Axial scans
FLAIR – Axial & sagittal scans

FINDINGS:

-Abnormal morphology of the brain stem and cerebellum in the form of the following:

• Molar tooth sign of the brain stem.
• Hypoplasia of the cerebellar vermis.
• Formation of a big cyst behind the cerebellum in the posterior fossa.
• Abnormal cerebellar Foleys.
- Evidence of enlargement of the cerebral gyri is seen.
-Lateral and third ventricles are normal in size and position. Septum is in midline.  There is no midline shift.
-Basal cisterns, cortical sulci and gyri are normal in appearance.
-Corpus callosum shows normal MR morphology.
-Dilated fourth ventricle.


OPINION:
• Picture is suggestive of Joubert’s syndrome with Dandy walker variant; please correlate with clinical picture.
• Evidence of Pachy gyri malformation.


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