{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["22(1)"],"submitter":["Reinecke R"],"funding":["Johann Wolfgang Goethe-Universität, Frankfurt am Main"],"pubmed_abstract":["<h4>Background</h4>Opsoclonus-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).<h4>Case presentation</h4>A 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.<h4>Conclusions</h4>This 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."],"journal":["BMC neurology"],"pagination":["507"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9801616"],"repository":["biostudies-literature"],"pubmed_title":["Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report."],"pmcid":["PMC9801616"],"pubmed_authors":["Reilander A","Voss M","Seiler A","Koch C","Reinecke R"],"additional_accession":[]},"is_claimable":false,"name":"Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report.","description":"<h4>Background</h4>Opsoclonus-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).<h4>Case presentation</h4>A 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.<h4>Conclusions</h4>This 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.","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Dec","modification":"2025-05-29T16:22:41.526Z","creation":"2025-04-19T18:09:45.528Z"},"accession":"S-EPMC9801616","cross_references":{"pubmed":["36581905"],"doi":["10.1186/s12883-022-03012-6"]}}