Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report
- Background: 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). Case presentation: 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. Conclusions: 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.
Author: | Raphael ReineckeORCiDGND, Annemarie Reiländer, Alexander SeilerORCiDGND, Christine KochGND, Martin VoßORCiDGND |
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URN: | urn:nbn:de:hebis:30:3-860142 |
DOI: | https://doi.org/10.1186/s12883-022-03012-6 |
ISSN: | 1471-2377 |
Parent Title (English): | BMC neurology |
Publisher: | BioMed Central ; Springer |
Place of publication: | London ; Berlin ; Heidelberg |
Document Type: | Article |
Language: | English |
Date of Publication (online): | 2022/12/30 |
Date of first Publication: | 2022/12/30 |
Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
Release Date: | 2024/08/08 |
Tag: | Opsoclonus-myoclonus syndrome; Pancreatic neuroendocrine tumor; Paraneoplastic neurological syndromes; Plasmapheresis |
Volume: | 22 |
Issue: | art. 507 |
Article Number: | 507 |
Page Number: | 4 |
First Page: | 1 |
Last Page: | 4 |
Note: | Open Access funding enabled and organized by Projekt DEAL. |
Note: | Gefördert durch den Open-Access-Publikationsfonds der Goethe-Universität. |
Note: | The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
HeBIS-PPN: | 520853482 |
Institutes: | Medizin |
Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
Sammlungen: | Universitätspublikationen |
Open-Access-Publikationsfonds: | Medizin |
Licence (German): | ![]() |