Treatment of Epileptic Encephalopathies

Current State of the Art

Hiroki Nariai, Susan Duberstein, Shlomo Shinnar

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)41-54
Number of pages14
JournalJournal of Child Neurology
Volume33
Issue number1
DOIs
StatePublished - Jan 1 2018

Fingerprint

Brain Diseases
Nervous System
Seizures
Anticonvulsants
Stroke
Brain
Therapeutics
Research

Keywords

  • abnormal interictal EEG
  • cognitive outcome
  • hormonal therapy
  • immune therapy
  • intractable seizures

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Treatment of Epileptic Encephalopathies : Current State of the Art. / Nariai, Hiroki; Duberstein, Susan; Shinnar, Shlomo.

In: Journal of Child Neurology, Vol. 33, No. 1, 01.01.2018, p. 41-54.

Research output: Contribution to journalArticle

Nariai, Hiroki ; Duberstein, Susan ; Shinnar, Shlomo. / Treatment of Epileptic Encephalopathies : Current State of the Art. In: Journal of Child Neurology. 2018 ; Vol. 33, No. 1. pp. 41-54.
@article{3371f64b730a4ce692c92d51f0491bb9,
title = "Treatment of Epileptic Encephalopathies: Current State of the Art",
abstract = "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.",
keywords = "abnormal interictal EEG, cognitive outcome, hormonal therapy, immune therapy, intractable seizures",
author = "Hiroki Nariai and Susan Duberstein and Shlomo Shinnar",
year = "2018",
month = "1",
day = "1",
doi = "10.1177/0883073817690290",
language = "English (US)",
volume = "33",
pages = "41--54",
journal = "Journal of Child Neurology",
issn = "0883-0738",
publisher = "SAGE Publications Inc.",
number = "1",

}

TY - JOUR

T1 - Treatment of Epileptic Encephalopathies

T2 - Current State of the Art

AU - Nariai, Hiroki

AU - Duberstein, Susan

AU - Shinnar, Shlomo

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

UR - http://www.scopus.com/inward/record.url?scp=85038423691&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85038423691&partnerID=8YFLogxK

U2 - 10.1177/0883073817690290

DO - 10.1177/0883073817690290

M3 - Article

VL - 33

SP - 41

EP - 54

JO - Journal of Child Neurology

JF - Journal of Child Neurology

SN - 0883-0738

IS - 1

ER -