Unique combination of anti‐paraneoplastic antigen Ma2 antibody‐positive Ophelia syndrome and opsoclonus‐myoclonus syndrome in an adult Hodgkin’s lymphoma patient

Abstract

AbstractBackgroundParaneoplastic neurological syndromes are a rare presentation of malignant lymphomas at diagnosis or relapse. The most frequent form is the cerebellar degeneration associated with Hodgkin’s lymphoma (HL), but several other syndromes, such as limbic encephalitis, sensorimotor neuropathies and dermatomyositis, have also been described in association with both HL and non‐Hodgkin’s lymphomas.Case presentationHere, we describe a unique case of a 64‐year‐old man with HL presenting at diagnosis with two distinct well‐defined paraneoplastic neurological syndromes – autoimmune limbic encephalitis with memory loss and opsoclonus‐myoclonus syndrome. Intriguingly, the patient tested positive for anti‐paraneoplastic antigen Ma2/Ta antibody, which is almost exclusively detected in solid cancers, such as testicular germ cell tumors and small cell lung cancer. The patient had early‐stage HL, and showed rapid improvement of neurological symptoms after initiation of corticosteroid treatment and specific lymphoma therapy with the adriamycin, bleomycin, vinblastine and dacarbazine regimen.ConclusionsThis case supports the general view of the complex immune system deregulation in HL, which can trigger various autoimmune phenomena, even in early‐stage disease.