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Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor
Morvan’s syndrome is a rare anti-contactin-associated protein-like 2 (CASPR2) antibody-mediated autoimmune disorder. The clinical features of this syndrome include muscular twitching, insomnia, dysautonomia, peripheral nerve hyperexcitability, and fluctuating delirium. An underlying tumor is commonl...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Scientific Scholar
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9894319/ https://www.ncbi.nlm.nih.gov/pubmed/36743755 http://dx.doi.org/10.25259/JNRP-2022-3-49 |
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author | Saluja, Alvee Verma, Jyoti Anees, Shahbaz Nangia, Anita Dhamija, Rajinder K. |
author_facet | Saluja, Alvee Verma, Jyoti Anees, Shahbaz Nangia, Anita Dhamija, Rajinder K. |
author_sort | Saluja, Alvee |
collection | PubMed |
description | Morvan’s syndrome is a rare anti-contactin-associated protein-like 2 (CASPR2) antibody-mediated autoimmune disorder. The clinical features of this syndrome include muscular twitching, insomnia, dysautonomia, peripheral nerve hyperexcitability, and fluctuating delirium. An underlying tumor is commonly found among Morvan’s syndrome cases, with thymoma being the most frequent association. We describe an unusual case of a 39-year-old female with excruciating bilateral leg pain, insomnia, hyperhidrosis, peripheral nerve hyperexcitability, serum anti-CASPR2 antibody positivity, and a solid pseudopapillary tumor of the pancreas on histopathology. Furthermore, the patient’s symptoms improved after receiving intravenous immunoglobulin (0.4 g/kg per day for 5 days). To the best of our knowledge, this is the first case of Morvan syndrome associated with a solid pseudopapillary pancreatic tumor to be reported in the literature to date. Our case adds to the spectrum of malignancies that are associated with Morvan’s syndrome. The recognition of this rare syndrome and its various associations are important for the neurologist, as it is a potentially treatable condition. |
format | Online Article Text |
id | pubmed-9894319 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Scientific Scholar |
record_format | MEDLINE/PubMed |
spelling | pubmed-98943192023-02-03 Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor Saluja, Alvee Verma, Jyoti Anees, Shahbaz Nangia, Anita Dhamija, Rajinder K. J Neurosci Rural Pract Case Report Morvan’s syndrome is a rare anti-contactin-associated protein-like 2 (CASPR2) antibody-mediated autoimmune disorder. The clinical features of this syndrome include muscular twitching, insomnia, dysautonomia, peripheral nerve hyperexcitability, and fluctuating delirium. An underlying tumor is commonly found among Morvan’s syndrome cases, with thymoma being the most frequent association. We describe an unusual case of a 39-year-old female with excruciating bilateral leg pain, insomnia, hyperhidrosis, peripheral nerve hyperexcitability, serum anti-CASPR2 antibody positivity, and a solid pseudopapillary tumor of the pancreas on histopathology. Furthermore, the patient’s symptoms improved after receiving intravenous immunoglobulin (0.4 g/kg per day for 5 days). To the best of our knowledge, this is the first case of Morvan syndrome associated with a solid pseudopapillary pancreatic tumor to be reported in the literature to date. Our case adds to the spectrum of malignancies that are associated with Morvan’s syndrome. The recognition of this rare syndrome and its various associations are important for the neurologist, as it is a potentially treatable condition. Scientific Scholar 2022-12-16 2022 /pmc/articles/PMC9894319/ /pubmed/36743755 http://dx.doi.org/10.25259/JNRP-2022-3-49 Text en © 2022 Published by Scientific Scholar on behalf of Journal of Neurosciences in Rural Practice https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Saluja, Alvee Verma, Jyoti Anees, Shahbaz Nangia, Anita Dhamija, Rajinder K. Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title | Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title_full | Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title_fullStr | Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title_full_unstemmed | Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title_short | Morvan’s syndrome: An unusual presentation of a solid pseudopapillary pancreatic tumor |
title_sort | morvan’s syndrome: an unusual presentation of a solid pseudopapillary pancreatic tumor |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9894319/ https://www.ncbi.nlm.nih.gov/pubmed/36743755 http://dx.doi.org/10.25259/JNRP-2022-3-49 |
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