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Effect on maximal mouth opening in children with spinal muscular atrophy treated with onasemnogene abeparvovec
  1. Nidhi Beri1,
  2. Lekha Kapoor2,
  3. Deepak Parashar3,
  4. Vivek Mundada4
  1. 1 Special Need Dentistry, Aster DM Healthcare, Dubai, UAE
  2. 2 ENT, Aster DM Healthcare, Dubai, UAE
  3. 3 Department of Health Science, University of Warwick Warwick Medical School, Coventry, UK
  4. 4 Paediatric Neurology, Aster DM Healthcare, Dubai, UAE
  1. Correspondence to Dr Nidhi Beri, Special Need Dentistry, Aster DM Healthcare, Dubai, UAE; nidhi.beri{at}icloud.com

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Spinal muscular atrophy (SMA) is a progressive, autosomal recessive neuromuscular disorder caused by a mutation of the SMN1 gene. This causes progressive muscle weakness and muscle atrophy. The voluntary muscles are affected, including those involved in swallowing and breathing. The muscles of mastication are also affected in them.

Adequate mouth opening is required to maintain good oral hygiene and adequate mastication. Tools like maximum tongue pressure and maximum mouth opening (MMO) are often used as clinical and sensitive outcome measures for assessing oral function tests.1 MMO is the maximal interincisal distance on unassisted active mouth opening, reflecting the mandibular range of motion.2

Onasemnogene abeparvovec is the US Food and Drug Administration (FDA)-approved gene replacement therapy for SMA. There is little or no …

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Footnotes

  • Twitter @vbmundada

  • Contributors VM designed the study. NB conducted the study. NB and LK prepared the manuscript under supervision of VM while DP has done the statistical analysis.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.