Swallowing problems in neuromuscular disorders.
Feeding problems in patients with neuromuscular diseases are frequently underestimated and poorly analyzed. To gain a better understanding of the most common complaints, we surveyed 451 patients and received 409 responses representing seven disorders. Difficulties in the pre-oral phase of swallowing were encountered primarily in Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy (LGMD), facio-scapulo-humoral muscular dystrophy (FSHMD), and spinal muscular atrophy (SMA). A limitation in the ability to open the mouth was also noted in SMA. Some features are characteristic of certain diseases such as macroglossia in DMD and dryness of the mouth in dermatomyositis and polymyositis (DMPM) and myasthenia gravis (MG). Posterior swallowing time is especially affected in MG, dermatomyositis and polymyositis, LGMD, and SMA. Overall prevalence of feeding disability in five disorders (SMA, myotonic dystrophy [MD], DMPM, FSHMD, MG) was 34.9%. A better understanding of the swallowing problems associated with these disorders may help in choosing treatment possibilities, technical aids, adaptation of the consistency of foods, swallowing rehabilitation, and nutritional support by the non-oral route.
AFM, Hôpital Tenon, Paris, France.
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TN
Willig
J
J
Paulus
J
J
Lacau Saint Guily
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C
Béon
J
J
Navarro
D000293:Adolescent; D000328:Adult; D003680:Deglutition Disorders / Q000209:etiology*; D003882:Dermatomyositis / Q000150:complications; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000150:complications; D009136:Muscular Dystrophies / Q000150:complications; D009157:Myasthenia Gravis / Q000150:complications; D009468:Neuromuscular Diseases / Q000150:complications* / Q000503:physiopathology / Q000534:rehabilitation; D009752:Nutritional Status; D018529:Nutritional Support; D017285:Polymyositis / Q000150:complications; D013704:Temporomandibular Joint / Q000503:physiopathology