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Journal of Child Neurology
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Is Head Balance a Major Determinant for Swallowing Problems in Patients With Spinal Muscular Atrophy Type 2?

Lenie van den Engel-Hoek, BA

Department of Rehabilitation, Radboud University Nijmegen Medical Centre, L.vandenEngel{at}cukz.umcn.nl

Bert J. M. de Swart, PhD

Department of Rehabilitation, Radboud University Nijmegen Medical Centre

Corrie E. Erasmus, MD

Interdisciplinary Centre for Child Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

Imelda J. M. de Groot, MD, PhD

Department of Rehabilitation, Radboud University Nijmegen Medical Centre

A child with spinal muscular atrophy type 2 was referred for evaluation of eating and swallowing problems. The dysphagia evaluation demonstrated coughing during eating and drinking and occasionally stertorous when eating solid food. The videofluoroscopic swallow study showed a late upper esophageal sphincter opening with hypopharyngeal residue, more with solid food than with thin liquid. His lumbar lordosis associated with anterior tilted pelvis and his problems with head balance due to weak neck musculature caused compensatory behavior like a retracted neck and mandible. This position negatively influences the opening of the upper esophageal sphincter. This case supported the idea that dysphagia in spinal muscular atrophy type 2 is caused by both a bulbar component as well as a treatable posture component.

Key Words: spinal muscular atrophy • swallowing problems

This version was published on August 1, 2008

Journal of Child Neurology, Vol. 23, No. 8, 919-921 (2008)
DOI: 10.1177/0883073808315418
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L. van den Engel-Hoek, C. E. Erasmus, H. W. van Bruggen, B.J.M. de Swart, L. T.L. Sie, M. H. Steenks, and I. J.M. de Groot
Dysphagia in spinal muscular atrophy type II: More than a bulbar problem?
Neurology, November 24, 2009; 73(21): 1787 - 1791.
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