Abstract:
BACKGROUND:Limited data exist regarding myopathies with early or prominent dysphagia. METHODS:A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness. RESULTS:Thirty-two patients were identified. The median age at diagnosis was 65 y (range, 36-80 y). Inclusion body myositis (IBM) (n = 15), immune-mediated necrotizing myopathy (IMNM) (n = 5), and oculopharyngeal muscular dystrophy (n = 4), were the most common diagnoses. In 4 patients (3 IMNM and 1 nonspecific myositis) dysphagia evolved rapidly. At evaluation, 21 patients required diet alterations, 5 required feeding tubes, and 8 had aspiration pneumonia. Follow-up data were available for 20 patients (median, 24 mo). Eight patients received immunosuppressive therapies with improvement in 7, including 3 of 4 with rapidly progressive dysphagia. CONCLUSIONS:IBM and IMNM accounted for approximately two-thirds of patients with early or prominent dysphagia at our institution. Rapidly progressive dysphagia may predict immunotherapy responsiveness.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Triplett JD,Pinto MV,Hosfield EA,Milone M,Liewluck Tdoi
10.1002/mus.26996subject
Has Abstractpub_date
2020-09-01 00:00:00pages
344-350issue
3eissn
0148-639Xissn
1097-4598journal_volume
62pub_type
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