{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:17:47Z","timestamp":1787840267232,"version":"build-2784847793"},"reference-count":11,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2025,3,20]],"date-time":"2025-03-20T00:00:00Z","timestamp":1742428800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Acute intermittent porphyria (AIP) is a dominant mendelian disorder caused due to deficiency of the enzyme porphobilinogen deaminase. It classically presents with pain abdomen, hypertensive crisis, electrolyte imbalance, mostly hyponatremia, and neuropsychiatric involvement. We report a case of a 12-year-old boy with AIP who experienced an acute crisis and later developed altered sensorium and seizures. Upon evaluation, he was found to have severe hyponatremia, which was secondary to the syndrome of inappropriate antidiuretic hormone secretion. His condition was corrected with intravenous hypertonic saline, and his sodium levels normalized over 2\u20133 days. Despite the successful correction of sodium levels, he developed extrapyramidal symptoms a week later. Magnetic resonance imaging of the brain revealed extrapontine myelinolysis. He was treated with intravenous steroids, which led to significant improvement. At 1-month follow-up, there were no neurological deficits.<\/jats:p>","DOI":"10.4103\/aian.aian_920_24","type":"journal-article","created":{"date-parts":[[2025,3,22]],"date-time":"2025-03-22T19:58:06Z","timestamp":1742673486000},"page":"437-439","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Unmasking Osmotic Demyelination Syndrome\/Extrapontine Myelinolysis in Acute Intermittent Porphyria: Preventable Complications\u2013Challenges in Diagnosis and Management"],"prefix":"10.4103","volume":"28","author":[{"given":"Vykuntaraju K","family":"Gowda","sequence":"first","affiliation":[{"name":"Department of Paediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priyanka A","family":"Nayak","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uddhava V","family":"Kinhal","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amaresh","family":"Roy","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Varunvenkat M","family":"Srinivasan","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,3,20]]},"reference":[{"key":"R1-20250614","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.ymgme.2016.10.005","article-title":"Acute intermittent porphyria in children:A case report and review of the literature","volume":"119","author":"Balwani","year":"2016","journal-title":"Mol Genet Metab"},{"key":"R3-20250614","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.21037\/atm-20-1529","article-title":"Management of hyponatremia associated with acute porphyria-proposal for the use of tolvaptan","volume":"8","author":"Solares","year":"2020","journal-title":"Ann Transl Med"},{"key":"R4-20250614","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1056\/NEJMra1608634","article-title":"Porphyria","volume":"377","author":"Bissell","year":"2017","journal-title":"N Engl J Med"},{"key":"R5-20250614","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1002\/mdc3.13250","article-title":"The spectrum of movement disorders in cases with osmotic demyelination syndrome","volume":"8","author":"Sindhu","year":"2021","journal-title":"Mov Disord Clin Pract"},{"key":"R6-20250614","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1681\/ASN.V481522","article-title":"Neurologic sequelae after treatment of severe hyponatremia:A multicenter perspective","volume":"4","author":"Sterns","year":"1994","journal-title":"J Am Soc Nephrol"},{"key":"R7-20250614","first-page":"383","article-title":"Therapeutic relowering of the serum sodium in a patient after excessive correction of hyponatremia","volume":"51","author":"Soupart","year":"1999","journal-title":"Clin Nephrol"},{"key":"R8-20250614","doi-asserted-by":"crossref","first-page":"733","DOI":"10.2169\/internalmedicine.56.7491","article-title":"The improvement of the outcome of osmotic demyelination syndrome by plasma exchange","volume":"56","author":"Kumon","year":"2017","journal-title":"Intern Med"},{"key":"R9-20250614","first-page":"005","article-title":"Complete recovery of chronic osmotic demyelination syndrome with plasma exchange","volume":"2","author":"Mahmood","year":"2018","journal-title":"J Clin Nephrol"},{"key":"R10-20250614","doi-asserted-by":"crossref","first-page":"106811","DOI":"10.1016\/j.clineuro.2021.106811","article-title":"Osmotic demyelination syndrome improving after immune-modulating treatment:Case report and literature review","volume":"208","author":"Kalampokini","year":"2021","journal-title":"Clin Neurol Neurosurg"},{"key":"R11-20250614","doi-asserted-by":"crossref","first-page":"2291","DOI":"10.2169\/naika.96.2291","article-title":"[Complete recovery of central pontine myelinolysis by high dose pulse therapy with methylprednisolone","volume":"96","author":"Sakamoto","year":"2007","journal-title":"Nihon Naika Gakkai Zasshi"},{"key":"R12-20250614","doi-asserted-by":"crossref","first-page":"30","DOI":"10.5049\/EBP.2015.13.1.30","article-title":"A case of osmotic demyelination presenting with severe hypernatremia","volume":"13","author":"Han","year":"2015","journal-title":"Electrolyte Blood Press"}],"container-title":["Annals of Indian Academy of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/aian.aian_920_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T13:19:04Z","timestamp":1787836744000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/aian.aian_920_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,20]]},"references-count":11,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025]]}},"URL":"https:\/\/doi.org\/10.4103\/aian.aian_920_24","relation":{},"ISSN":["0972-2327","1998-3549"],"issn-type":[{"value":"0972-2327","type":"print"},{"value":"1998-3549","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,20]]},"assertion":[{"value":"2024-11-03","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-02-14","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}