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Lambert-Eaton myasthenic syndrome

Key sources
The following summarized guidelines for the evaluation and management of Lambert-Eaton myasthenic syndrome are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the German Neurological Society (DGN 2023), the American Academy of Neurology (AAN 2012), and the European Federation of Neurological Societies (EFNS 2010; 2008).
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Guidelines

1.Diagnostic investigations

Respiratory evaluation: obtain pulmonary function testing to assist with management decisions in patients with neuromuscular disease at risk for respiratory complications.
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2.Respiratory support

Airway clearance: consider offering glossopharyngeal breathing for lung volume recruitment and airway clearance in patients with hypoventilation.
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  • Noninvasive ventilation

  • Invasive ventilation

3.Medical management

Symptomatic treatment: as per DGN 2023 guidelines, offer amifampridine and pyridostigmine for symptomatic therapy in patients with LEMS.
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  • Immunosuppressants

  • IVIG

4.Therapeutic procedures

Plasmapheresis/immunoadsorption: perform plasmapheresis/immunoadsorption if appropriate for the management of crisis worsening.
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5.Specific circumstances

Patients with paraneoplastic Lambert-Eaton syndrome: as per DGN 2023 guidelines, treat the primary tumor in patients with paraneoplastic LEMS as usual.