Journal ArticleDOI
Myoclonic atonic epilepsy: Another generalized epilepsy syndrome that is “not so” generalized
John M. Zempel,Tadaaki Mano +1 more
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TLDR
MAE is typically treated with antiseizure medications that are used for generalized epilepsy syndromes, with perhaps a best response to valproate, felbamate, or the ketogenic diet.Abstract:
Myoclonic atonic/astatic epilepsy (MAE), first described well by Doose1 (pronounced dough sah: ), is a generalized electroclinical syndrome with early onset characterized by myoclonic, atonic/astatic, generalized tonic-clonic, and absence seizures (but not tonic seizures) in association with generalized spike-wave (GSW) discharges. Thought to have a genetic component that has proven to be complicated,2 MAE sometimes occurs in children who have otherwise been developing normally and has variable outcome. MAE is typically treated with antiseizure medications that are used for generalized epilepsy syndromes, with perhaps a best response to valproate, felbamate, or the ketogenic diet.3,4read more
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Diagnosis and management of childhood epilepsy.
TL;DR: A diagnostic and management approach to pediatric seizures and epilepsy syndromes and alternative treatment options for pharmacologically resistant patients such as ketogenic diet, vagal nerve stimulation, and surgery are discussed.
References
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Journal ArticleDOI
Dynamic time course of typical childhood absence seizures: EEG, behavior, and functional magnetic resonance imaging.
Xiaoxiao Bai,Matthew Vestal,Rachel Berman,Michiro Negishi,Marisa N. Spann,Clemente Vega,Matthew N. DeSalvo,Edward J. Novotny,Robert Todd Constable,Hal Blumenfeld +9 more
TL;DR: In this article, the authors acquired simultaneous EEG-fMRI in 88 typical childhood absence seizures from nine pediatric patients and investigated behavior concurrently using a continuous performance task or simpler repetitive tapping task.
Journal ArticleDOI
Simultaneous EEG-fMRI in drug-naive children with newly diagnosed absence epilepsy.
Friederike Moeller,Hartwig R. Siebner,Stephan Wolff,Hiltrud Muhle,Oliver Granert,Olav Jansen,Ulrich Stephani,Michael Siniatchkin +7 more
TL;DR: EEG‐fMRI is applied to investigate BOLD signal changes during absence seizures in children with newly diagnosed childhood absence epilepsy (CAE) and it is found that changes in regional neuronal activity to the occurrence of generalized spike‐and‐wave discharges are linked to absence seizures.
Journal ArticleDOI
The core network in absence epilepsy Differences in cortical and thalamic BOLD response
Patrick W. Carney,Richard A.J. Masterton,AS Harvey,Ingrid E. Scheffer,Samuel F. Berkovic,Graeme D. Jackson +5 more
TL;DR: In children with typical AS, a core network of structures involved in generalized epileptiform activity that includes the reticular structures of the brainstem is confirmed, suggesting the parietal cortex has a role in the initiation of epilepsy.
Journal ArticleDOI
Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress.
Sarah A. Kelley,Eric H. Kossoff +1 more
TL;DR: The components of this unique disorder including its incidence, clinical features, and electroencephalographic findings are discussed, and the most effective treatment reported to date appears to be the ketogenic diet.
Myoclonic-astatic epilepsy.
TL;DR: Discussion is presented of possible pitfalls in the classical syndromic approach to classifying epilepsies of early childhood, and of the advantages of a neurobiological view for understanding the immense variability of clinical manifestations of epilepsy.
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Epileptic encephalopathies with myoclonic seizures in infants and children (severe myoclonic epilepsy and myoclonic-astatic epilepsy).
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