Muscle disorders.doc

Muscle disorders affecting oral and pharyngeal swallowing Safwan Jaradeh, M.D.Key Points Dysphagia is often underestimated in neuromuscular disorders. It can be prominent in dystrophies, inflammatory myopathy, mitochondrial myopathy, myasthenia, motor neuron diseases, and peripheral neuropathy. Among muscular dystrophies, oculopharyngeal muscular dystrophy and myotonic dystrophy are most commonly associated with dysphagia. Among inflammatory myopathies, the polymyositis associated with connective tissue disease and inclusion body myositis are most likely associated with dysphagia. Myasthenia leads to dysphagia with significant oral, masticatory, and pharyngeal components. Among motor neuron disorders, amyotrophic lateral sclerosis (ALS) is a leading cause of swallowing impairment, even in the absence of overt bulbar symptoms. Swallowing is usually intact in peripheral nerve disorders, except for polyradiculoneuropathies. Introduction Patients with progressive difficulties of swallowing present a challenge to the gastroenterologist, otolaryngologist, and neurologist. Besides adding a significant level of disability, certain neuromuscular conditions leading to dysphagia such as amyotrophic lateral sclerosis (ALS) can be fatal, and bulbar presentation may be the predominant feature, requiring a diligent workup and intervention to prevent unwanted complications such as aspiration pneumonia. Feeding problems in neuromuscular disorders are often underestimated. In a survey of 451 patients with 409 responders, the overall prevalence of feeding disability (oral or pharyngeal) was 35%.1 A better understanding of the swallowing problems associated with these disorders may help in guiding treatment, choosing technical aids, modifying the consistency of foods, swallowing rehabilitation, and nutritional support by the nonoral route. It is important to evaluate the respiratory function in neuromuscular diseases leading to dysphagia, because many of these disorders disrupting de