Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Saluja, Alvee, Verma, Jyoti, Anees, Shahbaz, Nangia, Anita, Dhamija, Rajinder K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2022
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
_version_ 1784881716283834368
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
work_keys_str_mv AT salujaalvee morvanssyndromeanunusualpresentationofasolidpseudopapillarypancreatictumor
AT vermajyoti morvanssyndromeanunusualpresentationofasolidpseudopapillarypancreatictumor
AT aneesshahbaz morvanssyndromeanunusualpresentationofasolidpseudopapillarypancreatictumor
AT nangiaanita morvanssyndromeanunusualpresentationofasolidpseudopapillarypancreatictumor
AT dhamijarajinderk morvanssyndromeanunusualpresentationofasolidpseudopapillarypancreatictumor