Molar Tooth Sign

Imaging brain A finding on transverse CT and MRI images acquired at the midbrain in patients with Joubert syndrome. The term molar tooth refers to the characteristic appearance of an enlarged and horizontally directed tubular structure on each side of the midline emerging from the midbrain, which is due to an absent decussation of the superior cerebellar peduncular fiber tracts
Imaging urinary bladder A descriptive term referring to the appearance of perivesicular contrast on contrast-enhanced CT in extraperitoneal bladder rupture
References in periodicals archive ?
Valente, brancati, and dallapiccola proposed a clinical classification of JS in which molar tooth sign was consider as obligatory criteria and hypotonia, developmental delay, ataxia, abnormal eye movements were considered as primary criteria.
A brain MRI showed severe vermis hypoplasia, molar tooth sign, agenesis of corpus callosum, retrocerebellar enlargement (variant Dandy Walker syndrome?
The deleted region involved a new gene that could be responsible for the clinical manifestations including cleft palate and Molar tooth sign, pyruvate dehydrogenase phosphatase regulatory subunit (PDPR).
Joubert Syndrome can be identified on axial magnetic resonance imaging as the molar tooth sign (MTS); other criteria include intellectual disability, hypotonia, and often, abnormal respiratory pattern and/or abnormal eye movements.
Joubert Syndrome: The Molar Tooth Sign of the Mid-Brain, Ann Med Health Sci Res 2013, Apr-Jun 3(2): 291-294.
There are classic imaging findings in Joubert syndrome, including the molar tooth sign and a bat-wing configuration of the fourth ventricle seen on axial MRI or CT.
Traditionally, all patients with the molar tooth sign on axial imaging were given the diagnosis of Joubert syndrome.
Brain MRI examination revealed that all cases had molar tooth sign, bat-wing appearance and vermian cleft.
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.
Joubert syndrome (JS) is a rare genetic disorder that affects the cerebellum, controlling balance, and coordination, and it usually presents a molar tooth sign on magnetic resonance imaging.
All three cases had also molar tooth sign in his brain MRI.
Molar tooth sign in fetal brain magnetic resonance imaging leading to the prenatal diagnosis of Joubert syndrome and related disorders.