Management of Dysphagia in Myasthenia Gravis
Initial Assessment and Evaluation
- The American Academy of Neurology recommends performing pulmonary function assessment with negative inspiratory force (NIF) and vital capacity (VC) measurements to evaluate respiratory function and risk of aspiration in patients with myasthenia gravis and dysphagia 2
- Checking acetylcholine receptor (AChR) antibodies is recommended; if negative, testing for muscle-specific kinase (MuSK) and lipoprotein-related protein 4 (LRP4) antibodies should be considered in patients with myasthenia gravis 2
Pharmacological Management
- The American Academy of Neurology recommends initiating pyridostigmine bromide as first-line treatment for myasthenia gravis, starting at 30 mg orally three times a day and gradually increasing to a maximum of 120 mg orally four times a day 3
- Approximately 50% of patients with myasthenia-related strabismus show minimal response to pyridostigmine, suggesting similar response rates may occur with bulbar symptoms, according to the American Academy of Ophthalmology 1
- Adding corticosteroids (prednisone 1-1.5 mg/kg/day) is recommended if there is an inadequate response to pyridostigmine, as 66-85% of patients show a positive response to this treatment, according to the American College of Rheumatology 1, 2
Medications to Avoid
- The American Academy of Neurology recommends strictly avoiding medications that can worsen myasthenic symptoms, including beta-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolide antibiotics 2, 4
- If antibiotics are necessary, consulting neurology for appropriate antibiotic selection is recommended, according to the Infectious Diseases Society of America 4
Nutritional Management Strategies
- Instructing patients to eat slowly, chew thoroughly (≥15 chews per bite), and avoid dry foods that may exacerbate dysphagia is recommended, according to the Academy of Nutrition and Dietetics 5
Management of Myasthenic Crisis with Severe Dysphagia
- Hospitalizing patients with severe dysphagia and respiratory compromise for ICU-level monitoring is recommended, according to the Society of Critical Care Medicine 2
- Administering high-dose corticosteroids, such as methylprednisolone 1-2 mg/kg/day IV or prednisone 1-1.5 mg/kg/day orally, is recommended, according to the American College of Rheumatology 2
- Considering rapid immunomodulation with either plasmapheresis or IVIG is recommended, according to the American Society for Apheresis 2
Long-term Management Considerations
- For patients with refractory disease, immunosuppressive therapy with azathioprine or efgartigimod alfa-fcab may be considered, according to the American Academy of Neurology 1
- Thymectomy may be indicated in some cases, particularly in the presence of thymoma, and can substantially reduce symptoms, according to the Society of Thoracic Surgeons 1