Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report

BMC Neurology

Case Report

Christine Koch¹  Alexander Seiler²  Annemarie Reiländer²  Raphael Reinecke³  Martin Voss³ 

[1] Department of Gastroenterology, Hepatology and Endocrinology, Goethe University Frankfurt, Frankfurt am Main, Germany;Department of Neurology, Goethe University Frankfurt, Frankfurt am Main, Germany;Department of Neurology, Goethe University Frankfurt, Frankfurt am Main, Germany;Dr. Senckenberg Institute of Neurooncology, Goethe University Frankfurt, Frankfurt am Main, Germany;

关键词: Opsoclonus-myoclonus syndrome;    Pancreatic neuroendocrine tumor;    Paraneoplastic neurological syndromes;    Plasmapheresis;   

DOI  :  10.1186/s12883-022-03012-6

 received in 2022-02-02, accepted in 2022-12-06,  发布年份 2022

来源: Springer

【 摘 要 】

BackgroundOpsoclonus-myoclonus syndrome (OMS) is a rare, immune-mediated neurological disorder. In adults, the pathogenesis can be idiopathic, post-infectious or paraneoplastic, the latter etiology belonging to the ever-expanding group of defined paraneoplastic neurological syndromes (PNS). In contrast to other phenotypes of PNS, OMS cannot be ascribed to a single pathogenic autoantibody. Here, we report the first detailed case of paraneoplastic, antibody-negative OMS occurring in association with a pancreatic neuroendocrine tumor (pNET).Case presentationA 33-year-old female presented with a two-week history of severe ataxia of stance and gait, dysarthria, head tremor, myoclonus of the extremities and opsoclonus. Her past medical history was notable for a metastatic pancreatic neuroendocrine tumor, and she was subsequently diagnosed with paraneoplastic opsoclonus-myoclonus syndrome. Further workup did not reveal a paraneoplastic autoantibody. She responded well to plasmapheresis, as she was refractory to the first-line therapy with corticosteroids.ConclusionsThis case expands current knowledge on tumors associated with paraneoplastic opsoclonus-myoclonus syndrome and the age group in which it can occur. It further adds evidence to the effectiveness of plasmapheresis in severe cases of opsoclonus-myoclonus syndrome with a lack of response to first-line therapy.

【 授权许可】

CC BY   
© The Author(s) 2022

【 图 表 】

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