Neurology®
The most widely read and highly cited peer-reviewed Neurology journal
Quick Search
Advanced Search
This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Data Supplement
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by van den Engel-Hoek, L.
Right arrow Articles by de Groot, I. J.M.
PubMed
Right arrow PubMed Citation
Right arrow Articles by van den Engel-Hoek, L.
Right arrow Articles by de Groot, I. J.M.
Related Collections
Right arrow Nutritional
Right arrow Anterior nerve cell disease
Right arrow All Rehabilitation
Right arrow EMG
NEUROLOGY 2009;73:1787-1791
© 2009 American Academy of Neurology

Dysphagia in spinal muscular atrophy type II

More than a bulbar problem? L. van den Engel-Hoek, MSc, C. E. Erasmus, MD, H. W. van Bruggen, MSc, B.J.M. de Swart, PhD, L. T.L. Sie, MD, PhD, M. H. Steenks, DDS, PhD and I. J.M. de Groot, MD, PhD

From the Department of Rehabilitation (L.v.d.E.-H., B.J.M.d.S., I.J.M.d.G.), Department of Child Neurology (C.E.E., L.T.L.S.), and Department Dental Care (H.W.v.B., M.H.S.), Radboud University Nijmegen Medical Centre, Nijmegen; and Department Oromaxillofacial Surgery and Special Dental Care (M.H.S.), University Medical Centre Utrecht, Utrecht, the Netherlands.

Address correspondence and reprint requests to L. van den Engel-Hoek, Department of Rehabilitation, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands, P.O. Box 9101, 818, 6500 HB Nijmegen, the Netherlands L.vandenengel{at}cukz.umcn.nl

Objective: In patients with spinal muscular atrophy (SMA) type II, feeding problems and dysphagia are common, but the underlying mechanisms of these problems are not well defined. This case control study was designed to determine the underlying mechanisms of dysphagia in SMA type II.

Methods: Six children with SMA type II and 6 healthy matched controls between 6.4 and 13.4 years of age were investigated during swallowing liquid and solid food in 2 different postures using surface EMG (sEMG) of the submental muscle group (SMG) and a video fluoroscopic swallow study (VFSS).

Results: The VFSS showed postswallow residue of solid food in the vallecula and above the upper esophageal sphincter (UES), which can be responsible for indirect aspiration. Better results in swallowing were achieved in a more forward head position. These findings were supported by the sEMG measurements of the SMG during swallowing.

Conclusions: Dysphagia in spinal muscular atrophy type II is due to a neurologic dysfunction (lower motor neuron problems from the cranial nerves in the brainstem) influencing the muscle force and efficiency of movement of the tongue and the submental muscle group in combination with a biomechanical component (compensatory head posture). The results suggest an integrated treatment with an adapted posture during meals and the advice of drinking water after meals to prevent aspiration pneumonias.

Abbreviations: DDS = Dysphagia Disorder Survey; DSW = Digital Swallowing Workstation; FP = forward posture; NDS = Nijmegen Dysarthria Scale; RP = retracted posture; sEMG = surface EMG; SLP = speech language pathologist; SMA = spinal muscular atrophy; SMG = submental muscle group; TOM = Therapy Outcome Measure; UES = upper esophageal sphincter; VFSS = video fluoroscopic swallow study.

Supplemental data at www.neurology.org

The study was done at the Department of Speech Language Therapy (part of the Department of Rehabilitation) of the Children Hospital of the Radboud University Nijmegen Medical Centre, Nijmegen (the Netherlands), as a part of clinical work of the specialized Swallowing Team.

Disclosure: The authors report no disclosures.

Received March 24, 2009. Accepted in final form August 19, 2009.