Late-onset double-seronegative myasthenia gravis syndrome and myasthenic crisis due to nivolumab use for Hodgkin's lymphoma

Abstract

Introduction: Insofar, use of programmed cell death-1 (PD-1) immune checkpoint inhibitors in oncology has been linked with several immune-mediated neurologic effects. However, grade 3 to 4 adverse events such as myasthenic crisis have been vanishingly rare.Case presentation: We present herein a unique patient with Hodgkin lymphoma who developed late-onset double-seronegative myasthenia gravis syndrome followed by myasthenic crisis after 16 weeks of therapy with nivolumab. One day prior to this event, she developed ptosis, diplopia, bulbar symptoms of dysphagia, dysarthria, orthopnea as well as extremity weakness. She required intubation, mechanical ventilation, plasmapheresis and steroid therapy.Management and outcome: She gradually achieved a near-complete resolution of neurologic symptoms over the next several weeks. On a follow-up visit eight weeks later, she only has some residual diplopia. Restaging scans showed a continued decrease in size of the mediastinal mass, without abnormal uptake. She remains on prednisone 10 mg orally daily.

Discussion: Prompt recognition of this rare phenomenon, immediate discontinuation of checkpoint inhibitor therapy and subsequent management with immunosuppressive therapy are necessary steps in order to minimize the considerable rates of morbidity and mortality.

Keywords: Myasthenia gravis; PD-1; adverse event; checkpoint inhibitor; nivolumab.