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Abstract
Objective: We performed a study in patients with proximal spinal muscular atrophy (SMA) to determine the prevalence of reduced maximal mouth opening (MMO) and its association with dysphagia as a reflection of bulbar dysfunction and visualized the underlying mechanisms using MRI.
Methods: We performed a cross-sectional study of MMO in 145 patients with SMA types 1–4 and 119 healthy controls and used MRI in 12 patients to visualize mandibular condylar shape and sliding and the anatomy of muscle groups relevant for mouth opening and closing. We analyzed associations of reduced MMO with SMA severity and complaints of dysphagia.
Results: Reduced MMO was defined as an interincisal distance ≤35 mm and was found in none of the healthy controls and in 100%, 79%, 50%, and 7% of patients with SMA types 1, 2, 3a, and 3b/4, respectively. MRI showed severe fatty degeneration of the lateral pterygoid muscles that mediate mouth opening by allowing mandibular condylar sliding but relatively mild involvement of the mouth closing muscles in patients with reduced MMO. Reduced MMO was associated with SMA type, age, muscle weakness, and dysphagia (p < 0.05).
Conclusions: Reduced MMO is common in SMA types 1–3a and is mainly caused by fatty degeneration of specific mouth opening muscles. Reduced MMO is a sign of bulbar dysfunction in SMA.
GLOSSARY
- AUC=
- area under the curve;
- HFMSE=
- Hammersmith Functional Motor Scale Expanded;
- MMO=
- maximal mouth opening;
- MRC=
- Medical Research Council;
- PV=
- predictive value;
- ROC=
- receiver operating characteristic;
- SMA=
- spinal muscular atrophy;
- SMN=
- survival motor neuron;
- TMJ=
- temporomandibular joint;
- VFSS=
- videofluoroscopic swallowing study
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received December 27, 2013.
- Accepted in final form June 24, 2014.
- © 2014 American Academy of Neurology
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