Clinical Perspective

Molar tooth sign − looking beyond the obvious

Nasreen Mahomed, Sanjay P. Prabhu
South African Journal of Radiology | Vol 17, No 3 | a275 | DOI: https://doi.org/10.4102/sajr.v17i3.275 | © 2013 Nasreen Mahomed, Sanjay P. Prabhu | This work is licensed under CC Attribution 4.0
Submitted: 24 February 2013 | Published: 01 August 2013

About the author(s)

Nasreen Mahomed, Department of Radiology, University of the Witwatersrand, Johannesburg, South Africa
Sanjay P. Prabhu, Department of Radiology, Boston Childrens Hospital, Harvard Medical School, Boston, United States


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Abstract

The molar tooth sign was initially identified in Joubert syndrome, named after Marie Joubert who first described it in 1968 as a rare autosomal recessive disorder characterised by the neuroradiological hallmark of the molar tooth sign caused by cerebellar vermian hypoplasia. Subsequently, it emerged that the molar tooth sign encompasses many syndromes that are now grouped together and termed Joubert syndrome and related disorders (JSRDs). Knowledge of the newer classification system and the subtypes is important and helps to direct and interpret imaging studies based on clinical signs so as to avoid delay in diagnosis of the hepatic oculorenal and renal subtypes of JSRDs in patients in whom the molar tooth sign is identified on brain MRI.

Keywords

Joubert syndrome; molar tooth sign

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