MOLAR TOOTH SIGN - JOUBERT SYNDROME | Zendy

Dušica Ranđelović | Zendy; Tatjana Mikić-Ranđelović | Zendy; Darko Laketić | Zendy

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MOLAR TOOTH SIGN - JOUBERT SYNDROME

Author(s) -

Dušica Ranđelović,

Tatjana Mikić-Ranđelović,

Darko Laketić

Publication year - 2015

Publication title -

acta medica medianae

Language(s) - English

Resource type - Journals

eISSN - 1821-2794

pISSN - 0365-4478

DOI - 10.5633/amm.2015.0312

Subject(s) - joubert syndrome , cerebellar vermis , anatomy , fourth ventricle , agenesis , cerebellar hypoplasia (non human) , medicine , hypoplasia , cerebellum , molar , magnetic resonance imaging , lateral recess , biology , radiology , orthodontics , lumbar , cilium , microbiology and biotechnology

The molar tooth sign is seen in very few conditions and is a very rare pediatric central nervous system congenital anomaly. Molar tooth sign is the result of cerebellar vermis hypoplasia, thick and maloriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. In Joubert syndrome, this is seen in about 85% of patients. We present a case of a two-year old girl with flaccid paraparesis, regression of milestones and developmental delay. Magnetic resonance imaging (MRI) showed the characteristic molar tooth sign with apposition of cerebellar hemispheres, batwing-shaped fourth ventricle, cerebellar vermis agenesis and deep interpeduncular fossa consistent with the diagnosis of Joubert syndrome

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