TY - JOUR
T1 - Treatment of Epileptic Encephalopathies
T2 - Current State of the Art
AU - Nariai, Hiroki
AU - Duberstein, Susan
AU - Shinnar, Shlomo
N1 - Funding Information:
The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: S. Shinnar is funded by National Institutes of Health grants NS 2R37-NS043209, 2U01-NS045911, U10NS077308, and 1U-1NS08803. He serves on the editorial board of Pediatric Neurology and serves on a data and safety monitoring board for UCB Pharma. He has received personal compensation for serving on Scientific Advisory Boards for Accorda, AstraZeneca, Questcor, and Upsher Smith and for consulting for Accorda, AstraZeneca, Neurelis, Questcor, Upsher-Smith. and Xeris. He has received royalties from Elsevier for co-editing the book Febrile Seizures. Drs Duberstein and Nariai report no disclosures.
Publisher Copyright:
© 2017, © The Author(s) 2017.
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Childhood epileptic encephalopathies are age-dependent disorders of the brain whose hallmarks include loss of neurologic function over time, abnormal electroencephalographic findings, and seizures. Ictal and interictal electrographic activity are conjointly thought to be at the root of the often devastating neuropsychological deterioration, which is specific to the maturing brain. The goals of treatment are not only to control seizures, but also to prevent or reverse neurologic loss of function. In general, time is of the essence in diagnosis, and experienced specialists should promptly design a treatment plan. Hormonal and immune therapies are at the forefront of treatment in many cases, with traditional antiepileptic drugs and surgery (when an identifiable lesion is present) playing a limited role. However, gold standard evidence for treatment of epileptic encephalopathies remains limited. Ongoing clinical and basic research may lead to better understanding of these catastrophic conditions and to better and more effective therapies.
AB - Childhood epileptic encephalopathies are age-dependent disorders of the brain whose hallmarks include loss of neurologic function over time, abnormal electroencephalographic findings, and seizures. Ictal and interictal electrographic activity are conjointly thought to be at the root of the often devastating neuropsychological deterioration, which is specific to the maturing brain. The goals of treatment are not only to control seizures, but also to prevent or reverse neurologic loss of function. In general, time is of the essence in diagnosis, and experienced specialists should promptly design a treatment plan. Hormonal and immune therapies are at the forefront of treatment in many cases, with traditional antiepileptic drugs and surgery (when an identifiable lesion is present) playing a limited role. However, gold standard evidence for treatment of epileptic encephalopathies remains limited. Ongoing clinical and basic research may lead to better understanding of these catastrophic conditions and to better and more effective therapies.
KW - abnormal interictal EEG
KW - cognitive outcome
KW - hormonal therapy
KW - immune therapy
KW - intractable seizures
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U2 - 10.1177/0883073817690290
DO - 10.1177/0883073817690290
M3 - Article
C2 - 28134012
AN - SCOPUS:85038423691
SN - 0883-0738
VL - 33
SP - 41
EP - 54
JO - Journal of child neurology
JF - Journal of child neurology
IS - 1
ER -