Immunoglobulins to mitigate paraneoplastic Lambert Eaton Myasthenic Syndrome under checkpoint inhibition in Merkel cell
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(2020) 2:52
Neurological Research and Practice
LETTER TO THE EDITOR
Open Access
Immunoglobulins to mitigate paraneoplastic Lambert Eaton Myasthenic Syndrome under checkpoint inhibition in Merkel cell carcinoma Maike F. Dohrn1,2* , Ulrike Schöne1, Charlotte Küppers1, Deborah Christen3, Jörg B. Schulz1, Burkhard Gess1 and Simone Tauber1 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.
Case A 62-year-old female patient presented with bilateral ptosis, dysphagia, and combined distal and proximal muscle weakness and fatigability. The clinical examination revealed a positive Simpson-test, hand muscle weakness, and an impaired proximal endurance. The 3 Hz-repetitive stimulation showed a significant decrement of compound muscle action potentials (CMAP) of the left trapezius and both anconeus muscles. PQ-type VGCC antibodies were elevated in serum by 387.6 pmol/ l (< 40), while antibodies against the acetylcholine receptor were negative. We began a symptomatic treatment of * Correspondence: [email protected]; [email protected] 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 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. A biopsy disclosed lymphogenic metastases of Merkel cell carcinoma, the primary origin of which remained unknown. The patient received avelumab, a recently approved immune checkpoint inhibitor (ICI) [2], which led to tumor remission. To enhance the immune response, avelumab targets the programmed cell death ligand (PD-L1), which is known to be upregulated by tumor cells to escape the recognition of T cells. In this patient, ICI treatment led to a significant worsening of limb weakness requiring the use of a walker, and a severely reduced vital capacity (< 1 l). The risks of both re
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