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September 16, 2014; 83 (12) Article

Bulbar muscle MRI changes in patients with SMA with reduced mouth opening and dysphagia

Renske I. Wadman, H. Willemijn van Bruggen, Theo D. Witkamp, Stanimira I. Sparreboom-Kalaykova, Marloes Stam, Leonard H. van den Berg, Michel H. Steenks, W. Ludo van der Pol
First published August 13, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000796
Renske I. Wadman
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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H. Willemijn van Bruggen
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Theo D. Witkamp
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Stanimira I. Sparreboom-Kalaykova
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Marloes Stam
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Leonard H. van den Berg
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Michel H. Steenks
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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W. Ludo van der Pol
From the Department of Neurology and Neurosurgery and Spieren voor Spieren Kindercentrum and Brain Center Rudolf Magnus (R.I.W., M.S., L.H.v.d.B., W.L.v.d.P.), Department of Oromaxillofacial Surgery and Special Dental Care (H.W.v.B.), and Department of Radiology (T.D.W.), University Medical Center Utrecht, the Netherlands; Radboud University Medical Center (H.W.v.B., S.I.S.-K., M.H.S.), Nijmegen, the Netherlands; and Center for Evidence Based Practice (H.W.v.B., S.I.S.-K., M.H.S.), Department of Oral Function and Prosthetic Dentistry, College of Dental Sciences, Nijmegen, the Netherlands.
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Citation
Bulbar muscle MRI changes in patients with SMA with reduced mouth opening and dysphagia
Renske I. Wadman, H. Willemijn van Bruggen, Theo D. Witkamp, Stanimira I. Sparreboom-Kalaykova, Marloes Stam, Leonard H. van den Berg, Michel H. Steenks, W. Ludo van der Pol
Neurology Sep 2014, 83 (12) 1060-1066; DOI: 10.1212/WNL.0000000000000796

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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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