Ohtahara syndrome - early infantile epileptic encephalopathy with characteristic pattern on electroencephalograph - "suppression burst"

Rana epileptička encefalopatija ili Ohtahara sindrom (OS) jedan je od najranijih oblika dobno ovisne encefalopatije, koja se javlja u prva tri mjeseca života, a obilježena je učestalim toničkim spazmima, suppression burst (S-B) zapisom u EEG-u i tvrdokornošću na terapiju antiepilepticima. Cilj našeg rada je opisati kliničku sliku, EEG osobitosti, etiologiju napadaja te karakterističan prijelaz u druge oblike epileptičkih sindroma. Opisali smo devetero djece s Ohtahara sindromom. Najučestaliji uzrok bile su kortikalne razvojne anomalije mozga, a tip napadaja tonički spazmi. Gotovo u sve djece bio je prisutan S-B zapis u EEG-u. Pratili smo i evaluaciju u druge sindrome: Westov sindrom (WS) i Lenox-Gastautov sindrom (L-GS). Ohtahara sindrom je encefalopatija rane dječje dobi s karakterističnom etiologijom i kliničkom slikom te specifičnim suppression-burst zapisom (S-B) u EEG-u, različitim odstupanjima u neurorazvojnom ishodu te zahtijeva posebne i detaljne pretrage.Ohtahara syndrome (OS) is the earliest type of infantile epileptic encephalopathy, the age of onset is during the first three months of age. It is characterized by frequent tonic spasms, suppression- burst pattern in electroencephalograph (EEG) recording and resistance to drug treatment. The aim of this study is to evaluate clinical symptoms, EEG characteristics, etiology and transition into other epileptic syndromes. We describe 9 children with OS. The most common cause of OS were anomalies of the cortical development and the most common type of seizures tonic spasms. The EEG pattern of OS was a suppression burst (S-B) pattern in all children. Follow-up during infancy showed that OS evolved into West syndrome (WS) and Lenox Gastaut syndrome (L-GS). OS is early epileptic encephalopathy with a characteristic clinic picture, heterogeneic etiology, characteristic EEG pattern of suppression – burst, and different neurologic outcome requiering special and detailed investigation

Ohtahara syndrome - early infantile epileptic encephalopathy with characteristic pattern on electroencephalograph - "suppression burst"

Rana epileptička encefalopatija ili Ohtahara sindrom (OS) jedan je od najranijih oblika dobno ovisne encefalopatije, koja se javlja u prva tri mjeseca života, a obilježena je učestalim toničkim spazmima, suppression burst (S-B) zapisom u EEG-u i tvrdokornošću na terapiju antiepilepticima. Cilj našeg rada je opisati kliničku sliku, EEG osobitosti, etiologiju napadaja te karakterističan prijelaz u druge oblike epileptičkih sindroma. Opisali smo devetero djece s Ohtahara sindromom. Najučestaliji uzrok bile su kortikalne razvojne anomalije mozga, a tip napadaja tonički spazmi. Gotovo u sve djece bio je prisutan S-B zapis u EEG-u. Pratili smo i evaluaciju u druge sindrome: Westov sindrom (WS) i Lenox-Gastautov sindrom (L-GS). Ohtahara sindrom je encefalopatija rane dječje dobi s karakterističnom etiologijom i kliničkom slikom te specifičnim suppression-burst zapisom (S-B) u EEG-u, različitim odstupanjima u neurorazvojnom ishodu te zahtijeva posebne i detaljne pretrage.Ohtahara syndrome (OS) is the earliest type of infantile epileptic encephalopathy, the age of onset is during the first three months of age. It is characterized by frequent tonic spasms, suppression- burst pattern in electroencephalograph (EEG) recording and resistance to drug treatment. The aim of this study is to evaluate clinical symptoms, EEG characteristics, etiology and transition into other epileptic syndromes. We describe 9 children with OS. The most common cause of OS were anomalies of the cortical development and the most common type of seizures tonic spasms. The EEG pattern of OS was a suppression burst (S-B) pattern in all children. Follow-up during infancy showed that OS evolved into West syndrome (WS) and Lenox Gastaut syndrome (L-GS). OS is early epileptic encephalopathy with a characteristic clinic picture, heterogeneic etiology, characteristic EEG pattern of suppression – burst, and different neurologic outcome requiering special and detailed investigation