{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T17:10:08Z","timestamp":1730826608724,"version":"3.28.0"},"reference-count":16,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Epileptic spasms without hypsarrhythmia occur when patients do not display hypsarrhythmia on electroencephalogram (EEG) at the onset and throughout the clinical course. We report three patients of epileptic spasms in patients with early onset, all of whom experienced other types of seizures.<\/jats:p><jats:p>We detail three patients (two boys and one girl) of epileptic spasms without hypsarrhythmia, occurring between 1 and 3 months of age, with no abnormalities detected on neurometabolic analysis and brain magnetic resonance imaging. Long-term video-EEG monitoring revealed epileptic spasms with focal onset seizures in two patients, and epileptic spasms followed by generalized tonic-clonic seizures in one patient. Hypsarrhythmia was never observed in repeated EEG examinations. Two patients achieved seizure freedom and improved development through treatment with topiramate alone or in combination with valproate, without requiring hormonal therapies or vigabatrin. The remaining patient achieved seizure freedom following administration of antiseizure medications, including topiramate, after a trial of adrenocorticotropic hormone therapy.<\/jats:p><jats:p>We report the cases of three patients with early onset epileptic spasms without hypsarrhythmia. All patients achieved seizure freedom after topiramate treatment. Topiramate may be considered as a relatively effective antiseizure medication for early onset epileptic spasms without hypsarrhythmia.<\/jats:p>","DOI":"10.1055\/a-2298-0747","type":"journal-article","created":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T23:48:34Z","timestamp":1712101714000},"page":"250-254","source":"Crossref","is-referenced-by-count":0,"title":["Three Patients of the Early Onset Epileptic Spasms without Hypsarrhythmia"],"prefix":"10.1055","volume":"55","author":[{"given":"Ikko","family":"Ohshiro","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tohru","family":"Okanishi","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryo","family":"Ohta","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kento","family":"Ohta","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuto","family":"Arai","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sotaro","family":"Kanai","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayataka","family":"Fujimoto","sequence":"additional","affiliation":[{"name":"Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshihiro","family":"Maegaki","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,4,24]]},"reference":[{"issue":"09","key":"ref1","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.braindev.2013.10.008","article-title":"Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013","volume":"36","author":"P Pavone","year":"2014","journal-title":"Brain Dev"},{"issue":"07","key":"ref2","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1016\/S0387-7604(01)00263-7","article-title":"Epidemiological data of West syndrome in Finland","volume":"23","author":"R Riikonen","year":"2001","journal-title":"Brain Dev"},{"issue":"07","key":"ref3","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1111\/j.1528-1167.2009.02504.x","article-title":"Epileptic phenotypes in children with respiratory chain disorders","volume":"51","author":"S El Sabbagh","year":"2010","journal-title":"Epilepsia"},{"issue":"04","key":"ref4","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1111\/j.1528-1167.2009.02308.x","article-title":"Xp22.3 genomic deletions involving the CDKL5 gene in girls with early onset epileptic encephalopathy","volume":"51","author":"D Mei","year":"2010","journal-title":"Epilepsia"},{"issue":"12","key":"ref5","doi-asserted-by":"crossref","first-page":"2098","DOI":"10.1111\/epi.13937","article-title":"The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium","volume":"58","author":"S T Demarest","year":"2017","journal-title":"Epilepsia"},{"issue":"02","key":"ref6","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1684\/j.1950-6945.2003.tb00571.x","article-title":"Epileptic spasms in clusters without hypsarrhythmia in infancy","volume":"5","author":"R H Caraballo","year":"2003","journal-title":"Epileptic Disord"},{"issue":"05","key":"ref7","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1111\/j.1528-1167.2005.37504.x","article-title":"Effect of ACTH therapy for epileptic spasms without hypsarrhythmia","volume":"46","author":"H Oguni","year":"2005","journal-title":"Epilepsia"},{"issue":"04","key":"ref8","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1111\/epi.12951","article-title":"How should children with West syndrome be efficiently and accurately investigated? 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