{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T02:19:45Z","timestamp":1790129985150,"version":"4.0.1"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T00:00:00Z","timestamp":1719964800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T00:00:00Z","timestamp":1719964800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Pediatr"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and life-threatening autoimmune disease of the central nervous system. So far, only ten cases of PERM have been reported in children worldwide, including the one in this study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>We report a case of an 11-year-old boy with PERM with an initial presentation of abdominal pain, skin itching, dysuria, urinary retention, truncal and limb rigidity, spasms of the trunk and limbs during sleep, deep and peripheral sensory disturbances, and dysphagia. A tissue-based assay using peripheral blood was positive, demonstrated by fluorescent staining of mouse cerebellar sections. He showed gradual and persistent clinical improvement after immunotherapy with intravenous immunoglobulin, steroids, plasmapheresis and rituximab.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We summarized the diagnosis and treatment of a patient with PERM and performed a literature review of pediatric PERM to raise awareness among pediatric neurologists. A better comprehension of this disease is required to improve its early diagnosis, treatment, and prognosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12887-024-04899-5","type":"journal-article","created":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T09:03:47Z","timestamp":1719997427000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Progressive encephalomyelitis with rigidity and myoclonus: a pediatric case report and literature review"],"prefix":"10.1186","volume":"24","author":[{"given":"Yu","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing-wen","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi-hui","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruo-hao","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiang-yang","family":"Luo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhan-wen","family":"He","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,3]]},"reference":[{"issue":"5","key":"4899_CR1","doi-asserted-by":"publisher","first-page":"853","DOI":"10.1002\/mds.10279","volume":"17","author":"HM Meinck","year":"2002","unstructured":"Meinck HM, Thompson PD. Stiff man syndrome and related conditions. Mov Disord. 2002;17(5):853\u201366.","journal-title":"Mov Disord"},{"key":"4899_CR2","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1159\/000212375","volume":"26","author":"ME Duddy","year":"2009","unstructured":"Duddy ME, Baker MR. Stiff person syndrome. Front Neurol Neurosci. 2009;26:147\u201365.","journal-title":"Front Neurol Neurosci"},{"issue":"Pt 8","key":"4899_CR3","doi-asserted-by":"publisher","first-page":"2178","DOI":"10.1093\/brain\/awu142","volume":"137","author":"A Carvajal-Gonzalez","year":"2014","unstructured":"Carvajal-Gonzalez A, Leite MI, Waters P, Woodhall M, Coutinho E, Balint B, Lang B, Pettingill P, Carr A, Sheerin UM, et al. Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. 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Informed consent was obtained from parents of the participants included in this study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent for publication of the patient\u2019s clinical details was obtained from the guardian of the patient. Written consent is available for review, please contact the corresponding author if necessary.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"427"}}