{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T16:13:25Z","timestamp":1783440805259,"version":"3.54.6"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,11,29]],"date-time":"2022-11-29T00:00:00Z","timestamp":1669680000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3389\/fneur.2022.1042988","type":"journal-article","created":{"date-parts":[[2022,11,29]],"date-time":"2022-11-29T05:07:19Z","timestamp":1669698439000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":9,"title":["Case report: Varicella-zoster virus infection triggering progressive encephalomyelitis with rigidity and myoclonus"],"prefix":"10.3389","volume":"13","author":[{"given":"Jing","family":"Yuan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aihua","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunfeng","family":"Hou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuxu","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,11,29]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1159\/000342237","article-title":"Progressive encephalomyelitis with rigidity and myoclonus: a syndrome with diverse clinical features and antibody responses","volume":"69","author":"Shugaiv","year":"2013","journal-title":"Eur Neurol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1290","DOI":"10.1111\/ene.13721","article-title":"Antiglycine receptor antibody related disease: a case series and literature review","volume":"25","author":"Swayne","year":"2018","journal-title":"Eur J Neurol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"2178","DOI":"10.1093\/brain\/awu142","article-title":"Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes","volume":"137","author":"Carvajal-Gonzalez","year":"2014","journal-title":"Brain."},{"key":"B4","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1186\/s12883-022-02555-y","article-title":"Inspiratory laryngeal stridor as the main feature of progressive encephalomyelitis with rigidity and myoclonus: a case report and literature review","volume":"22","author":"Yao","year":"2022","journal-title":"BMC Neurol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1212\/WNL.0000000000000372","article-title":"Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies","volume":"82","author":"Balint","year":"2014","journal-title":"Neurology."},{"key":"B6","doi-asserted-by":"publisher","first-page":"661","DOI":"10.1136\/jnnp.2003.010504","article-title":"Progressive encephalomyelitis with rigidity associated with anti-amphiphysin antibodies","volume":"75","author":"Ishii","year":"2004","journal-title":"J Neurol Neurosurg Psychiatry."},{"key":"B7","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1097\/WCO.0000000000000450","article-title":"Redefining progressive encephalomyelitis with rigidity and myoclonus after the discovery of antibodies to glycine receptors","volume":"30","author":"Crisp","year":"2017","journal-title":"Curr Opin Neurol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"2510","DOI":"10.1002\/acn3.50946","article-title":"Antecedent infections in Guillain-Barre syndrome: a single-center, prospective study","volume":"6","author":"Hao","year":"2019","journal-title":"Ann Clin Transl Neurol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1214","DOI":"10.1016\/S0140-6736(21)00517-1","article-title":"Guillain-Barre syndrome","volume":"397","author":"Shahrizaila","year":"2021","journal-title":"Lancet."},{"key":"B10","doi-asserted-by":"publisher","first-page":"760","DOI":"10.1016\/S1474-4422(18)30244-8","article-title":"Frequency, symptoms, risk factors, and outcomes of autoimmune encephalitis after herpes simplex encephalitis: a prospective observational study and retrospective analysis","volume":"17","author":"Armangue","year":"2018","journal-title":"Lancet Neurol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1016\/j.clim.2019.07.007","article-title":"West Nile Virus infection triggering autoimmune encephalitis: pathophysiological and therapeutic implications","volume":"207","author":"Karagianni","year":"2019","journal-title":"Clin Immunol."},{"key":"B12","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1016\/S1473-3099(10)70222-X","article-title":"Causes of encephalitis and differences in their clinical presentations in England: a multicentre, population-based prospective study","volume":"10","author":"Granerod","year":"2010","journal-title":"Lancet Infect Dis."},{"key":"B13","doi-asserted-by":"publisher","first-page":"2309","DOI":"10.1212\/WNL.0000000000001072","article-title":"VZV brainstem encephalitis triggers NMDA receptor immunoreaction","volume":"83","author":"Schabitz","year":"2014","journal-title":"Neurology."},{"key":"B14","doi-asserted-by":"publisher","first-page":"703050","DOI":"10.3389\/fneur.2021.703050","article-title":"Case report: dexmedetomidine for intractable clusters of myoclonic jerks and paroxysmal sympathetic hyperactivity in progressive encephalomyelitis with rigidity and myoclonus","volume":"12","author":"Fujino","year":"2021","journal-title":"Front Neurol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/S0091-6749(97)70236-7","article-title":"Conversion of the CD4+ T cell profile from T(H2)-dominant type to T(H1)-dominant type after varicella-zoster virus infection in atopic dermatitis","volume":"100","author":"Fujimura","year":"1997","journal-title":"J Allergy Clin Immunol."},{"key":"B16","doi-asserted-by":"publisher","first-page":"526","DOI":"10.1007\/s13365-014-0274-2","article-title":"Robust pro-inflammatory and lesser anti-inflammatory immune responses during primary simian varicella virus infection and reactivation in rhesus macaques","volume":"20","author":"Traina-Dorge","year":"2014","journal-title":"J Neurovirol."},{"key":"B17","doi-asserted-by":"publisher","first-page":"106807","DOI":"10.1016\/j.clineuro.2021.106807","article-title":"Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature","volume":"208","author":"Chang","year":"2021","journal-title":"Clin Neurol Neurosurg."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2022.1042988\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,11,29]],"date-time":"2022-11-29T05:07:23Z","timestamp":1669698443000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2022.1042988\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,29]]},"references-count":17,"alternative-id":["10.3389\/fneur.2022.1042988"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2022.1042988","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,29]]},"article-number":"1042988"}}