Pre-existing Lambert-Eaton Myasthenic Syndrome and scleroderma in a patient with neuroendocrine carcinoma undergoing immune checkpoint inhibitor cancer immunotherapy

Abstract

IntroductionParaneoplastic neurological syndromes (PNS) can worsen with immune checkpoint inhibitor (ICI) cancer immunotherapy. Case reportA 66-year-old female with paraneoplastic Lambert-Eaton Myasthenic Syndrome (LEMS), which led to the diagnosis of metastatic neuroendocrine carcinoma, was treated with intravenous immune globulin (IVIg) (with minimal response), chemotherapy, and radiation, resulting in neurological improvement. However, sclerodermatous changes developed after a year. Due to cancer progression, dual ICI therapy was initiated, and the patient remained stable for eight months until the progression of both LEMS and cancer, ultimately leading to death. DiscussionThis case highlights the challenges of managing pre-existing PNS during ICI therapy, emphasizing the need for a multidisciplinary approach and the consideration of unusual clinical presentations in therapeutic decision-making.