{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:19:33Z","timestamp":1753877973493,"version":"3.41.2"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2024,4,5]],"date-time":"2024-04-05T00:00:00Z","timestamp":1712275200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,4,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Familial hemiplegic migraine (FHM), an autosomal dominant subtype of hemiplegic migraine, is a channelopathy presenting with severe headache, visual field defect, paresthesia, unilateral motor deficit, encephalopathy, seizures and aphasia. This cross-sectional study was conducted over 10\u2009months in children aged 1\u201318\u2009years suspected of hemiplegic migraine at a tertiary care pediatric hospital. Fourteen children were screened and five children with genetically confirmed FHM were included. The symptoms in the study population were paroxysmal hemiparesis (5\/5), headache (5\/5) and focal seizures (1\/5). The hemiplegia episodes lasted from 4\u2009h to 7\u2009days. The mean age at the onset of neurological symptoms was 6.8\u2009\u00b1\u20090.7\u2009years and the mean age at diagnosis was 12.8\u2009\u00b1\u20091.7\u2009years, with a mean delay of 6.1\u2009\u00b1\u20091.9\u2009years for the diagnosis. Neuroimaging during acute episodes revealed accentuated gray, white differentiation in the contralateral cerebral hemisphere with mild effacement of sulcal spaces in T2\/fluid-attenuated inversion recovery (FLAIR) images. Genetic testing revealed ATP1A2 mutations (FHM2) in 4\/5 and SCN1A (FHM3) in 1\/5 patients. All of them (5\/5) were initiated on oral topiramate and had favorable treatment responses with a mean follow-up duration of 7\u2009\u00b1\u20091.4\u2009months. Diagnosis of FHM is mainly clinical and can be confirmed by genetic analysis. Perfusion and diffusion-weighted MRI should be considered during acute headache episodes, as it is mostly normal in symptom-free periods. Routine MRI sequences like T1 weighted, T2 weighted, FLAIR and contrast remain normal even during acute attacks.<\/jats:p>","DOI":"10.1093\/tropej\/fmae008","type":"journal-article","created":{"date-parts":[[2024,4,6]],"date-time":"2024-04-06T00:53:14Z","timestamp":1712364794000},"source":"Crossref","is-referenced-by-count":1,"title":["Familial hemiplegic migraine in Indian children\u2014a tertiary center experience"],"prefix":"10.1093","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3153-0736","authenticated-orcid":false,"given":"Lokesh","family":"Saini","sequence":"first","affiliation":[{"name":"Department of Pediatrics, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3494-8863","authenticated-orcid":false,"given":"Pradeep Kumar","family":"Gunasekaran","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarbesh","family":"Tiwari","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4289-4415","authenticated-orcid":false,"given":"Bharat","family":"Choudhary","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sujatha","family":"Manjunathan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashna","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, All India Institute of Medical Sciences , Jodhpur 342005, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,4,5]]},"reference":[{"key":"2024040523415952700_fmae008-B1","first-page":"1993","volume-title":"GeneReviews\u00ae","author":"Jen","year":"2001"},{"key":"2024040523415952700_fmae008-B2","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1016\/S1474-4422(11)70048-5","article-title":"Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management","volume":"10","author":"Russell","year":"2011","journal-title":"Lancet Neurol"},{"key":"2024040523415952700_fmae008-B3","doi-asserted-by":"crossref","first-page":"12697","DOI":"10.3390\/ijms241612697","article-title":"Genetic mechanisms of migraine: insights from monogenic migraine mutations","volume":"24","author":"Gosalia","year":"2023","journal-title":"Int J Mol Sci"},{"key":"2024040523415952700_fmae008-B4","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1111\/head.14582","article-title":"Familial hemiplegic migraine in pediatric patients: a genetic, clinical, and follow-up study","volume":"63","author":"Mangano","year":"2023","journal-title":"Headache"},{"key":"2024040523415952700_fmae008-B5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/0333102417738202","article-title":"Headache classification committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition","volume":"38","author":"Olesen","year":"2018","journal-title":"Cephalalgia"},{"key":"2024040523415952700_fmae008-B6","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1007\/s11940-012-0208-3","article-title":"Familial and sporadic hemiplegic migraine: 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