Objective: To evaluate the clinical features, treatment strategies, and outcome of dysphagia in patients with inclusion body myositis.
Design: Retrospective review of all 26 patients (20 women, 6 men, mean age of 72.2 yrs) with inclusion body myositis-associated dysphagia seen in 1997-2001 at our institution.
Results: Twenty-four patients (92%) had a dysphagia evaluation. Cricopharyngeal muscle dysfunction was noted in all nine patients who had barium swallow studies. Eighteen patients (69%) underwent one or more interventional procedures: cricopharyngeal myotomy (10), pharyngoesophageal dilation (6), percutaneous endoscopic gastrostomy (6), and botulinum injection of the upper esophageal sphincter (2). Dysphagia tended to worsen with time. Symptomatic improvement was noted with cricopharyngeal myotomy (63%) and pharyngoesophageal dilation (33%). The Mendelsohn maneuver seemed helpful in maintaining oral intake in the three patients in whom it was recommended. Thirteen patients died during follow-up at a mean age of 81 yrs. The cause of death was identified in eight and in all cases was because of the respiratory complications of aspiration.
Conclusions: Dysphagia is a progressive condition in patients with inclusion body myositis and often leads to death from aspiration pneumonia. Treatment targeting cricopharyngeal muscle dysfunction, such as the Mendelsohn maneuver, will benefit from further investigation.