{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:20:45Z","timestamp":1791566445596,"version":"4.3.4"},"reference-count":11,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,1,19]],"date-time":"2025-01-19T00:00:00Z","timestamp":1737244800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ther Apher Dial"],"published-print":{"date-parts":[[2025,6]]},"abstract":"Abstract<\/jats:title>Introduction<\/jats:title>Progressive encephalomyelitis with rigidity and myoclonus (PERM) is characterized by brainstem symptoms, muscle rigidity, and myoclonus. While autoantibodies to inhibitory neurons have been associated with the pathology, about 30% of cases are negative for autoantibodies. There are few reported cases of antibody\u2010negative PERM and its clinical course and prognosis are unknown.<\/jats:p><\/jats:sec>Methods<\/jats:title>We report three cases of antibody\u2010negative PERM in which plasma exchange was effective.<\/jats:p><\/jats:sec>Results<\/jats:title>Case 1 was a 68\u2010year\u2010old man, case 2 was a 27\u2010year\u2010old woman, and case 3 was a 47\u2010year\u2010old woman. In all three cases, steroid pulse therapy and intravenous immunoglobulin had limited effect and plasma exchange was markedly effective. All patients experienced disease relapse, which was effectively treated with plasma exchange.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>In the present cases of antibody\u2010negative PERM, plasma exchange was most effective and was maintained long\u2010term despite repeated relapses. Plasma exchange should be actively chosen in antibody\u2010negative PERM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1744-9987.14253","type":"journal-article","created":{"date-parts":[[2025,1,20]],"date-time":"2025-01-20T02:59:38Z","timestamp":1737341978000},"page":"512-515","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Efficacy of apheresis in antibody\u2010negative progressive encephalomyelitis with rigidity and myoclonus"],"prefix":"10.1111","volume":"29","author":[{"given":"Yohei","family":"Aoki","sequence":"first","affiliation":[{"name":"Department of Neurology Wakayama Medical University Wakayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mayumi","family":"Sakata","sequence":"additional","affiliation":[{"name":"Department of Neurology Wakayama Medical University Wakayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidekazu","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neurology Ikuwakai Memorial Hospital Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kohei","family":"Hori","sequence":"additional","affiliation":[{"name":"Department of Neurology Wakayama Medical University Wakayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junko","family":"Taruya","sequence":"additional","affiliation":[{"name":"Department of Neurology Wakayama Medical University Wakayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0349-9842","authenticated-orcid":false,"given":"Katsuichi","family":"Miyamoto","sequence":"additional","affiliation":[{"name":"Department of Neurology Wakayama Medical University Wakayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,19]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-6377-2"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13721"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awz297"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2021.106807"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000450"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2010.229104"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000200187"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awu142"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000372"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23756"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000200298"}],"container-title":["Therapeutic Apheresis and Dialysis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1744-9987.14253","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,4]],"date-time":"2025-05-04T23:33:41Z","timestamp":1746401621000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1744-9987.14253"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,19]]},"references-count":11,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,6]]}},"alternative-id":["10.1111\/1744-9987.14253"],"URL":"https:\/\/doi.org\/10.1111\/1744-9987.14253","archive":["Portico"],"relation":{},"ISSN":["1744-9979","1744-9987"],"issn-type":[{"value":"1744-9979","type":"print"},{"value":"1744-9987","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,19]]},"assertion":[{"value":"2024-09-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}