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

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

doi:10.1186/s42466-020-00099-5

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.

Highlights

Lambert-Eaton myasthenic syndrome (LEMS) is a rare, autoimmune or paraneoplastic condition characterized by muscle weakness and fatigability
Hussman Institute for Human Genomics, University of Miami, Miller School of Medicine, Miami, Florida, USA Full list of author information is available at the end of the article the Lambert-Eaton myasthenic syndrome (LEMS) with 3,4-diaminopyridine and pyridostigmine
As 50% of LEMS cases arise in a paraneoplastic context [1], a PETCT revealed inguinal lymph nodes suspicious for malignancy

Summary

Introduction

Lambert-Eaton myasthenic syndrome (LEMS) is a rare, autoimmune or paraneoplastic condition characterized by muscle weakness and fatigability. * Correspondence: mdohrn@ukaachen.de; mfd83@miami.edu 1Department of Neurology, Medical Faculty of the RWTH Aachen University, Aachen, Germany 2Dr. John T. Hussman Institute for Human Genomics, University of Miami, Miller School of Medicine, Miami, Florida, USA Full list of author information is available at the end of the article the Lambert-Eaton myasthenic syndrome (LEMS) with 3,4-diaminopyridine and pyridostigmine. The patient received avelumab, a recently approved immune checkpoint inhibitor (ICI) [2], which led to tumor remission.

Results

Conclusion