Immunoglobulins to mitigate paraneoplastic Lambert Eaton Myasthenic Syndrome under checkpoint inhibition in Merkel cell carcinoma

Maike F Dohrn; Ulrike Schöne; Charlotte Küppers; Deborah Christen; Jörg B Schulz; Burkhard Gess; Simone Tauber

doi:10.1186/s42466-020-00099-5

Immunoglobulins to mitigate paraneoplastic Lambert Eaton Myasthenic Syndrome under checkpoint inhibition in Merkel cell carcinoma

Neurol Res Pract. 2020 Dec 9:2:52. doi: 10.1186/s42466-020-00099-5. eCollection 2020.

Authors

Maike F Dohrn^{1

2}, Ulrike Schöne¹, Charlotte Küppers¹, Deborah Christen³, Jörg B Schulz¹, Burkhard Gess¹, Simone Tauber¹

Affiliations

¹ Department of Neurology, Medical Faculty of the RWTH Aachen University, Aachen, Germany.
² Dr. John T. Macdonald Foundation, Department of Human Genetics and John P. Hussman Institute for Human Genomics, University of Miami, Miller School of Medicine, Miami, Florida USA.
³ Department of Oncology and Hematology, Medical Faculty of the RWTH Aachen University, Aachen, Germany.

Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is a rare, autoimmune or paraneoplastic condition characterized by muscle weakness and fatigability. In cancer therapy, immune checkpoint inhibitors (ICI) sensitize the immune system for tumor antigens. We report a 62-year-old, female patient with paraneoplastic LEMS as first manifestation of Merkel cell carcinoma. Under avelumab, the LEMS exacerbated with worsening of limb weakness and a severely reduced vital capacity (< 1 l). To treat this immunological side effect, we added a regimen with intravenous immunoglobulins. Hereby, the LEMS improved significantly. As we were able to continue the cancer treatment, the Merkel cell carcinoma has been in remission so far. This is the first description of paraneoplastic LEMS, avelumab, and Merkel cell carcinoma. We conclude that immunoglobulins are an option to control an ICI-associated deterioration of paraneoplastic symptoms.