{"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":1783440805200,"version":"3.54.6"},"reference-count":10,"publisher":"Department of Medicine, Warren Alpert Medical School at Brown University","issue":"2","license":[{"start":{"date-parts":[[2022,6,3]],"date-time":"2022-06-03T00:00:00Z","timestamp":1654214400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>A woman in her 50s presented with hyper-religiosity, auditory hallucinations, episodes of staring associated with unresponsiveness and stiffening of her entire body. A broad workup excluded autoimmune encephalitis, sarcoidosis, and prion disease. She was diagnosed with PERM (progressive encephalomyelitis with rigidity and myoclonus) syndrome which was supported by elevated cerebrospinal fluid (CSF) autoantibodies against glutamic acid decarboxylase (GAD) glutamic acid decarboxylase. She responded to treatment with corticosteroids and weekly rituximab therapy over four weeks. PERM is an autoimmune condition which is a rare variant of stiff person syndrome involving rigidity, dysautonomia and encephalopathy. PERM requires the clinician to have a high index of clinical suspicion to recognize and treat.<\/jats:p>","DOI":"10.56305\/001c.36139","type":"journal-article","created":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T15:56:31Z","timestamp":1730130991000},"source":"Crossref","is-referenced-by-count":1,"title":["Progressive Encephalomyelitis with Rigidity and Myoclonus Treated with Rituximab: Case Report and Review of the Literature"],"prefix":"10.56305","volume":"1","author":[{"given":"Carlin","family":"Chuck","sequence":"first","affiliation":[{"name":"Warren Alpert Medical School at Brown University","department":["Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5647-4569","authenticated-orcid":false,"given":"Karl","family":"Herman","sequence":"additional","affiliation":[{"name":"Warren Alpert Medical School at Brown University","department":["Division of Hospital Medicine, Department of Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sleiman","family":"El Jamal","sequence":"additional","affiliation":[{"name":"Warren Alpert Medical School at Brown University","department":["Neurology"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"35509","published-online":{"date-parts":[[2022,6,3]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"116993","DOI":"10.1016\/j.jns.2020.116993","article-title":"Therapeutic considerations in a case of progressive encephalomyelitis with rigidity and myoclonus","volume":"416","author":"Lauren Gluck","year":"2020","journal-title":"Journal of the Neurological Sciences","ISSN":"https:\/\/id.crossref.org\/issn\/0022-510X","issn-type":"print"},{"issue":"11","key":"ref2","doi-asserted-by":"publisher","first-page":"3166","DOI":"10.1093\/brain\/awq253","article-title":"Stiff person syndrome-associated autoantibodies to amphiphysin mediate reduced GABAergic inhibition","volume":"133","author":"Christian Geis","year":"2010","journal-title":"Brain","ISSN":"https:\/\/id.crossref.org\/issn\/1460-2156","issn-type":"print"},{"issue":"5","key":"ref3","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1212\/wnl.0b013e318227b176","article-title":"Progressive encephalomyelitis with rigidity and myoclonus: glycine and NMDA receptor antibodies","volume":"77","author":"M. R. Turner","year":"2011","journal-title":"Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"issue":"1","key":"ref4","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1093\/brain\/99.1.27","article-title":"Progressive encephalomyelitis with rigidity","volume":"99","author":"A. M. Whiteley","year":"1976","journal-title":"Brain","ISSN":"https:\/\/id.crossref.org\/issn\/0006-8950","issn-type":"print"},{"key":"ref5","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":"Anna Chang","year":"2021","journal-title":"Clinical Neurology and Neurosurgery","ISSN":"https:\/\/id.crossref.org\/issn\/0303-8467","issn-type":"print"},{"issue":"4","key":"ref6","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1001\/jamaneurol.2013.1872","article-title":"Progressive Encephalomyelitis With Rigidity and Myoclonus: The First Pediatric Case With Glycine Receptor Antibodies","volume":"70","author":"Joana Dam\u00e1sio","year":"2013","journal-title":"JAMA Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/2168-6149","issn-type":"print"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"1017","DOI":"10.3389\/fneur.2020.01017","article-title":"Progressive Encephalomyelitis With Rigidity and Myoclonus With Thymoma: A Case Report and Literature Review","volume":"11","author":"Yana Su","year":"2020","journal-title":"Frontiers in Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/1664-2295","issn-type":"print"},{"issue":"16","key":"ref8","doi-asserted-by":"publisher","first-page":"1291","DOI":"10.1212\/01.wnl.0000327606.50322.f0","article-title":"Progressive encephalomyelitis, rigidity, and myoclonus: a novel glycine receptor antibody","volume":"71","author":"M. Hutchinson","year":"2008","journal-title":"Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"issue":"17","key":"ref9","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":"B. Balint","year":"2014","journal-title":"Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"issue":"3","key":"ref10","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":"Sarah J. Crisp","year":"2017","journal-title":"Current Opinion in Neurology","ISSN":"https:\/\/id.crossref.org\/issn\/1350-7540","issn-type":"print"}],"container-title":["Journal of Brown Hospital Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/bhm.scholasticahq.com\/article\/36139-progressive-encephalomyelitis-with-rigidity-and-myoclonus-treated-with-rituximab-case-report-and-review-of-the-literature","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,18]],"date-time":"2025-02-18T18:36:55Z","timestamp":1739903815000},"score":1,"resource":{"primary":{"URL":"https:\/\/bhm.scholasticahq.com\/article\/36139-progressive-encephalomyelitis-with-rigidity-and-myoclonus-treated-with-rituximab-case-report-and-review-of-the-literature"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,3]]},"references-count":10,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.56305\/001c.36139","relation":{},"ISSN":["2994-5593"],"issn-type":[{"value":"2994-5593","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,3]]}}}