Treatment of Lambert-Eaton Myasthenic Syndrome in the Setting of Associated Malignancy

Lambert-Eaton myasthenic syndrome (LEMS) may arise as a paraneoplastic condition in patients with an underlying malignancy. This paraneoplastic form (pLEMS) presents a distinct clinical scenario that shapes the overall management approach.

Clinical scenario

LEMS can be idiopathic (iLEMS) or, in the context of an associated malignancy — particularly small-cell lung cancer (SCLC), typically in long-time smokers — as a paraneoplastic disease (pLEMS). When malignancy is present, it is central to the management strategy.

Treatment approach (overview only)

In pLEMS, addressing the underlying malignancy takes priority — tumor-directed treatment forms the cornerstone of care. Symptomatic therapy is continued alongside oncological management. The complete regimen — including specific agents, dosing guidance, and sequencing — is available in the full protocol.

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References

LEMS can be idiopathic (iLEMS; mostly women younger than 50 years) or, less commonly – especially in small-cell lung cancer (SCLC) – as a paraneoplastic disease (pLEMS; mostly in long-time smokers).

For pLEMS, tumor treatment must be performed as usual.

Therapy of pLEMS focuses primarily on effective treatment of the tumor by means of (neo-)adjuvant chemotherapy, radiation, and, if necessary, surgical removal.

An initiated and effective symptomatic treatment with amifampridine and, if necessary, pyridostigmine should be continued.

DOI: 10.1177/17562864231213240

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