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The möbius sequence: a relook.
1Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois at Chicago, 60612, USA. marimill@uic.edu
Summary
Mobius sequence, a developmental disorder, involves cranial nerve deficits and diverse malformations. Associated findings offer insights into the embryonic insult
Area of Science:
- Neurology
- Developmental Biology
- Ophthalmology
Background:
- Mobius syndrome is re-conceptualized as Mobius sequence, indicating secondary events post-embryonic insult.
- Classic signs include cranial nerve VI and VII deficits, with limb, craniofacial, and other cranial nerve malformations.
Purpose of the Study:
- To investigate the clinical spectrum and associated findings in patients with Mobius sequence.
- To explore the developmental disturbances underlying Mobius sequence.
Main Methods:
- A prospective study of 25 patients with characteristic Mobius sequence findings in Sweden.
- Clinical assessment of ocular motility, facial nerve function, and associated systemic anomalies.
Main Results:
- Ocular motility showed varied patterns, including limited abduction and horizontal gaze paresis; 10/25 had straight eyes.
- Nineteen patients had diminished facial expression; associated anomalies included limb malformations (8), micrognathia (8), tongue malformations (17), and autism spectrum disorder (7).
Conclusions:
- Associated findings in Mobius sequence provide clues to the timing and location of developmental disturbances.
- The diverse ocular motility patterns challenge the notion of a lesion solely in the sixth nerve nucleus.