Molar tooth sign of the midbrain-hindbrain junction: occurrence in multiple distinct syndromes.

@article{Gleeson2004MolarTS,
  title={Molar tooth sign of the midbrain-hindbrain junction: occurrence in multiple distinct syndromes.},
  author={Joseph G. Gleeson and Lesley C Keeler and Melissa A Parisi and Sarah E. Marsh and Phillip F. Chance and Ian A Glass and John M. Jr Graham and B. L. Maria and A. James Barkovich and William B. Dobyns},
  journal={American journal of medical genetics. Part A},
  year={2004},
  volume={125A 2},
  pages={
          125-34; discussion 117
        }
}
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 majority of patients with this diagnosis. We previously showed that the MTS is a… CONTINUE READING

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