Molar tooth sign − looking beyond the obvious

SA Journal of Radiology

Field Value
Title Molar tooth sign − looking beyond the obvious
Creator Mahomed, Nasreen Prabhu, Sanjay P.
Subject Radiology Joubert syndrome; molar tooth sign
Description 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.
Publisher AOSIS
Date 2013-08-01
Type info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion — —
Format text/html application/pdf
Identifier 10.4102/sajr.v17i3.275
Source South African Journal of Radiology; Vol 17, No 3 (2013); 102-103 2078-6778 1027-202X
Language eng
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Rights Copyright (c) 2013 Nasreen Mahomed, Sanjay P. Prabhu