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Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disease of unknown etiology, and most patients with PERM are positive for anti-glycine receptor (GlyR) antibody. In this case study, we report a clinical case of a varicella-zoster virus-infected patient who deve...

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Autores principales: Yuan, Jing, Wang, Aihua, Hou, Yunfeng, Xu, Xuxu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9744753/
https://www.ncbi.nlm.nih.gov/pubmed/36523342
http://dx.doi.org/10.3389/fneur.2022.1042988
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author Yuan, Jing
Wang, Aihua
Hou, Yunfeng
Xu, Xuxu
author_facet Yuan, Jing
Wang, Aihua
Hou, Yunfeng
Xu, Xuxu
author_sort Yuan, Jing
collection PubMed
description Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disease of unknown etiology, and most patients with PERM are positive for anti-glycine receptor (GlyR) antibody. In this case study, we report a clinical case of a varicella-zoster virus-infected patient who developed anti-GlyR antibody-positive PERM. He initially suffered from herpes zoster and gradually developed symptoms of impaired brainstem functions including hoarse voice and dysphagia, accompanied by paroxysmal sympathetic hyperactivity. The patient also suffered from severe spasms, which were easily triggered by external stimuli. Glycine receptor antibodies were then found to be positive in serum and cerebrospinal fluid, and the diagnosis of PERM was confirmed. Methylprednisolone and gamma globulin treatments were given, and spasms were improved after treatment. Unfortunately, the patient's family insisted on automatic discharge and the patient passed away several days later.
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spelling pubmed-97447532022-12-14 Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus Yuan, Jing Wang, Aihua Hou, Yunfeng Xu, Xuxu Front Neurol Neurology Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disease of unknown etiology, and most patients with PERM are positive for anti-glycine receptor (GlyR) antibody. In this case study, we report a clinical case of a varicella-zoster virus-infected patient who developed anti-GlyR antibody-positive PERM. He initially suffered from herpes zoster and gradually developed symptoms of impaired brainstem functions including hoarse voice and dysphagia, accompanied by paroxysmal sympathetic hyperactivity. The patient also suffered from severe spasms, which were easily triggered by external stimuli. Glycine receptor antibodies were then found to be positive in serum and cerebrospinal fluid, and the diagnosis of PERM was confirmed. Methylprednisolone and gamma globulin treatments were given, and spasms were improved after treatment. Unfortunately, the patient's family insisted on automatic discharge and the patient passed away several days later. Frontiers Media S.A. 2022-11-29 /pmc/articles/PMC9744753/ /pubmed/36523342 http://dx.doi.org/10.3389/fneur.2022.1042988 Text en Copyright © 2022 Yuan, Wang, Hou and Xu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Yuan, Jing
Wang, Aihua
Hou, Yunfeng
Xu, Xuxu
Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title_full Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title_fullStr Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title_full_unstemmed Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title_short Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
title_sort case report: varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9744753/
https://www.ncbi.nlm.nih.gov/pubmed/36523342
http://dx.doi.org/10.3389/fneur.2022.1042988
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