{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T23:02:13Z","timestamp":1790550133533,"version":"4.1.0"},"reference-count":13,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,12,27]],"date-time":"2018-12-27T00:00:00Z","timestamp":1545868800000},"content-version":"vor","delay-in-days":26,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epileptic Disorders"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Hemiconvulsion\u2010Hemiplegia\u2010Epilepsy initially involves an infantile presentation of febrile focal motor status epilepticus, with subsequent hemiplegia of the initially affected side. Months to years later, affected children go on to develop a chronic epilepsy with recurrent focal seizures which are often refractory to treatment. This uncommon paediatric epilepsy syndrome is poorly understood, with only a very small minority of cases associated with an underlying genetic or metabolic abnormality.<\/jats:p><jats:p>We present a four\u2010year\u2010old girl with genetic cobalamin C deficiency who had a dramatic presentation with Hemiconvulsion\u2010Hemiplegia\u2010Epilepsy. She had febrile focal status epilepticus, with right hemiconvulsive seizures for nearly 10 hours, ultimately requiring a midazolam infusion. Over subsequent days, she developed progressively worsening cerebral oedema, leading to herniation and requiring a craniectomy to relieve pressure.<\/jats:p><jats:p>This girl's presentation is the first association of cobalamin deficiency with hemiconvulsion\u2010hemiplegia\u2010epilepsy; and illustrates the importance of considering this entity when patients with this metabolic disorder present with acute deterioration. More importantly, the case also raises the possibility that derangements of cobalamin metabolism could be a contributing factor in cases of hemiconvulsion\u2010hemiplegia\u2010epilepsy, as well as febrile seizures in general.<\/jats:p>","DOI":"10.1684\/epd.2018.1017","type":"journal-article","created":{"date-parts":[[2019,8,2]],"date-time":"2019-08-02T13:24:29Z","timestamp":1564752269000},"page":"545-550","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Hemiconvulsion\u2010Hemiplegia\u2010Epilepsy in a girl with cobalamin C deficiency"],"prefix":"10.1002","volume":"20","author":[{"given":"Kenneth A.","family":"Myers","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal Canada"},{"name":"Department of Neurology and Neurosurgery, McGill University, Montreal Canada"},{"name":"Research Institute of the McGill University Health Centre, Montreal Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roy WR.","family":"Dudley","sequence":"additional","affiliation":[{"name":"Research Institute of the McGill University Health Centre, Montreal Canada"},{"name":"Division of Neurosurgery, Department of Pediatric Surgery, Montreal Children's Hospital, McGill University Health Centre, Montreal Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam","family":"Srour","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal Canada"},{"name":"Department of Neurology and Neurosurgery, McGill University, Montreal Canada"},{"name":"Research Institute of the McGill University Health Centre, Montreal Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,12,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2012.01.007"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2002.24001.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13827"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1959.tb04278.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2011.06.001"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2018.01.005"},{"key":"e_1_2_7_8_1","first-page":"345","article-title":"Serum vitamin B12, folic acid, and homocysteine levels in children with febrile seizure","volume":"57","author":"Ozkale Y.","year":"2015","journal-title":"Turk J Pediatr"},{"key":"e_1_2_7_9_1","volume-title":"MedLink Neurology","author":"Panayiotopoulos CP","year":"2016"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2012.08.007"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-nutr-071715-050947"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9217-0"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2013.07.018"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199804093381507"}],"container-title":["Epileptic Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1684\/epd.2018.1017","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,18]],"date-time":"2024-03-18T16:06:11Z","timestamp":1710777971000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1684\/epd.2018.1017"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,12]]},"references-count":13,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2018,12]]}},"alternative-id":["10.1684\/epd.2018.1017"],"URL":"https:\/\/doi.org\/10.1684\/epd.2018.1017","archive":["Portico"],"relation":{},"ISSN":["1294-9361","1950-6945"],"issn-type":[{"value":"1294-9361","type":"print"},{"value":"1950-6945","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,12]]},"assertion":[{"value":"2018-05-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-08-22","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-12-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}