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Cerebrospinal fluid tested positive for VZV, MRI showed no signs of myelitis and EEG was negative for epilepsy. However, on clinical presentation, progressive encephalomyelitis with rigidity and myoclonus, a variant of the more well-known stiff person syndrome, was suspected and later supported by positive serum testing for associated anti-glycine receptor antibodies. Initially established antiviral, anti-epileptic and symptomatic treatment was then extended with intravenous immunoglobulin therapy. Only additional corticosteroid treatment led to a reduction in myoclonus, and uptitration of benzodiazepines decreased rigidity.<\/jats:p>","DOI":"10.1136\/bcr-2024-262431","type":"journal-article","created":{"date-parts":[[2024,12,24]],"date-time":"2024-12-24T22:55:28Z","timestamp":1735080928000},"page":"e262431","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Varicella zoster infection-associated progressive encephalomyelitis with rigidity and myoclonus in a 6-month follow-up"],"prefix":"10.1136","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2576-9727","authenticated-orcid":false,"given":"Iris","family":"Wiederkehr","sequence":"first","affiliation":[{"name":"Neurology, Neurologische Praxis Luzern, Luzern, Switzerland"},{"name":"Internal Medicine, Hirslanden Klinik Sankt Anna, Luzern, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyun","family":"Hor","sequence":"additional","affiliation":[{"name":"Neurology, Neurologische Praxis Luzern, Luzern, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Max","family":"Wiederkehr","sequence":"additional","affiliation":[{"name":"Neurology, Neurologische Praxis Luzern, Luzern, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esther","family":"Bachli","sequence":"additional","affiliation":[{"name":"Internal Medicine, Hirslanden Klinik Sankt Anna, Luzern, Switzerland"},{"name":"Medicine, Universit\u00e4t Z\u00fcrich, Zurich, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2024,12,24]]},"reference":[{"key":"2025082708003247000_17.12.e262431.1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10279"},{"key":"2025082708003247000_17.12.e262431.2","doi-asserted-by":"crossref","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":"2025082708003247000_17.12.e262431.3","doi-asserted-by":"crossref","unstructured":"Lancaster E . 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