Abstract
Dysphagia occurs in a variety of neuromuscular disorders, including motor neuron diseases, myasthenia gravis (MG) and myopathies. Limited data exist regarding myopathies with early/ prominent dysphagia. We conducted a retrospective study over 16 years to identify myopathy patients, in whom dysphagia was the initial symptom or was disproportionately severe compared to limb weakness (evidence of aspiration on swallowing study or requirement of diet modification, procedural intervention or feeding tube while ambulatory).. We identified 29 patients, [inclusion body myositis (IBM) (n=13), immune-mediated necrotizing myopathy (IMNM) with (n=2)/without (n=3) immune checkpoint inhibitor exposture, oculopharyngeal muscular dystrophy (OPMD) (n=4), nonspecific myositis (n=2), mitochondrial myopathy (n=2), and single cases of amyloid myopathy, sporadic late onset nemaline myopathy, and congenital myopathy], dysphagia was the initial symptom in 15. Median age at diagnosis was 65 years (range 36-80). At time of evaluation, 19 required diet alterations, 3 required feeding tubes and 5 had aspiration pneumonia. Twenty eight patients had also weakness of facial (12), neck flexor (11), neck extensor (6) and limb muscles (27). Ten patients (4 IMNM, 4 OPMD, 1 IBM and 1 mitochondrial myopathy) had ophthalmoparesis or ptosis. Median time from dysphagia onset to diagnosis was 36 months, (range 1–240). Dysphagia evolved rapidly within 6 months in 4 patients (3 IMNM and 1 nonspecific myositis). Follow-up data was available in 20 patients: 4 required feeding tube (3 SIBM, 1 nonspecific myositis), 2 SIBM patients had esophageal dilatation and 4 SIBM patients underwent cricopharyngeal myotomy. Eight patients (4 IMNM, 3 IBM, and 1 nonspecific myositis), received immunotherapy resulting in marked improvement only in patients with rapidly progressive dysphagia. This study identifies IBM and IMNM as the 2 most common causes of acquired myopathies with early or prominent dysphagia, accounting for approximately two-thirds of the patients in our cohort. Rapidly progressive course of dysphagia may predict immunotherapy responsiveness
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