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Article

Molar tooth sign of the midbrain–hindbrain junction: Occurrence in multiple distinct syndromes

7 Aug 2003

Abstract excerpt

The Molar Tooth Sign (MTS) is defined by an abnormally deep interpeduncular fossa; elongated, thick, and mal-oriented superior cerebellar peduncles; and absent or hypoplastic cerebellar vermis that together give the appearance of a "molar tooth" on axial brain MRI through the junction of the midbrain and hindbrain (isthmus region). It was first described in Joubert syndrome (JS) where it is present in the vast...

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Identifiers

PMID: 14981712
DOI: 10.1002/ajmg.a.20437

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Source receipt: pubmed, retrieved 7 Sept 2026.

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