Joubert syndrome

Case contributed by Dr Hani Salam


No clinical information available.

Patient Data

Age: Child

MRI brain

MRI of a child demonstrates absence of the cerebellar vermis with the cerebellar hemispheres apposed in the midline, without fusion. The mid brain has a characteristic molar tooth configuration. 

Annotated image

Molar tooth and bat wing signs

Joubert syndrome is home to two signs: the molar tooth sign of the midbrain and the bat wing configuration of the 4th ventricle. 

  • Molar tooth photo credit: Gleam (more information here)
  • Fruit bat photo credit: Marion Schneider & Christoph Aistleitner (more information here)

Case Discussion

This case illustrates the typical features of Joubert syndrome, with both the molar tooth appearance of the midbrain and bat wing configuration of the 4th ventricle. 

PlayAdd to Share

Case information

rID: 8595
Published: 15th Feb 2010
Last edited: 3rd Feb 2020
Tag: brain
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.