Mandibular dysfunction as a reflection of bulbar involvement in SMA type 2 and 3

Neurology
H Willemijn van BruggenMichel H Steenks

Abstract

In a cross-sectional study, we aimed to determine (1) the effect of spinal muscular atrophy (SMA) type 2 and 3 on mandibular function reflected as masticatory performance, mandibular range of motion, and bite force and (2) the predictors of mandibular dysfunction. Sixty patients with SMA type 2 and 3 (mean age 32.3 years, SD 17.4 years) and 60 age-matched controls filled out questionnaires about impairments of mandibular function. All participants underwent detailed clinical examination to document the mandibular range of motion including maximal mouth opening, bite force, and masticatory function. All mandibular movements, including mouth opening, lateral range of motion, and protrusion of the mandible, were reduced in patients with SMA type 2 and 3 compared to healthy controls (p < 0.001). Maximal bite force was 19% lower in patients than controls, and more in patients with SMA type 2 than type 3. The strongest predictive factor was SMA type for impairment of mandibular range of motion (R(2) = 0.82) and weakness of neck muscles for bite force (R(2) = 0.47). Reduced mandibular mobility and bite force are common complications in SMA. SMA type and neck muscle strength are important correlates of these complications. We provide f...Continue Reading

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Citations

Sep 3, 2019·Journal of Neuromuscular Diseases·A M B van der HeulL van den Engel-Hoek
Jul 6, 2018·European Journal of Anaesthesiology·Camiel A WijngaardeW Ludo van der Pol
Mar 14, 2020·Orphanet Journal of Rare Diseases·Hamish W Y WanMichelle A Farrar
Sep 19, 2019·European Journal of Orthodontics·Valentina PucciarelliChiarella Sforza
Jan 18, 2021·Neuromuscular Disorders : NMD·Renske I WadmanFrancesco Muntoni
Apr 7, 2021·American Journal of Speech-language Pathology·Katlyn Elizabeth McGrattanBasil T Darras

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