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The 'molar tooth sign' in Joubert syndrome.
The molar tooth sign is seen in very few conditions and is a very rare paediatric 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.
Rehman, Imaad ur +4 more
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Joubert syndrome and related disorders (JSRDs) are a group of anomalies characterized by hypotonia, ataxia, developmental delay, intellectual disability, abnormal eye movements, and apnea and hyperpnea in infancy with multiorgan involvement in which the ...
Fatemeh Farahmand +3 more
doaj
P18.01: Joubert syndrome at 11–13 weeks: molar tooth sign and associated brain anomalies [PDF]
F. Ushakov
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Background Joubert’s syndrome is a rare condition affecting an estimated 1:80,000–1:100,000 individuals. There is underdevelopment of the cerebellar vermis resulting in a characteristic molar tooth sign on cross sectional axial magnetic resonance imaging.
Grace M. Musiime +3 more
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P30.08: Severe ventriculomegaly, cerebellar hypoplasia and molar tooth sign in a case of Joubert syndrome [PDF]
Mariangela Cialdella +3 more
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The Molar Tooth Sign of Brain Mri: A Case Report of Joubert Syndrome
Fadoua Ijim +2 more
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OP05.05: The prenatal visualisation of the “molar tooth sign” in case of Joubert syndrome and related disorders using 3D ultrasound and/or MRI [PDF]
E. Quarello +4 more
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The aggressive growth and high recurrence rate of juvenile ossifying fibroma pose significant challenges in diagnosis and treatment. The lesion can expand substantially, involving the orbit and causing tooth displacement, leading to complications.
Niloofar Ghadimi, DDS +4 more
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Joubert Syndrome: Imaging Findings and Report of a Case
Background: Joubert syndrome (JS) is a very rare autosomal recessive disorder characterized by agenesis of cerebellar vermis, abnormal eye movements, respiratory irregularities, and delayed generalized motor development.
Qays A. Hassan, Asmaa H. Alsharea
doaj
Molar tooth sign: Neuroimaging characteristic of Joubert syndrome [PDF]
Nagpal, Tarun, Pande, Sanjay
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