PMID- 29062978
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240321
IS  - 2470-9239 (Print)
IS  - 2470-9239 (Electronic)
IS  - 2470-9239 (Linking)
VI  - 2
IP  - 3
DP  - 2017 Sep
TI  - INTERNEURONOPATHIES AND THEIR ROLE IN EARLY LIFE EPILEPSIES AND 
      NEURODEVELOPMENTAL DISORDERS.
PG  - 284-306
LID - 10.1002/epi4.12062 [doi]
AB  - GABAergic interneurons control the neural circuitry and network activity in the 
      brain. The advances in genetics have identified genes that control the 
      development, maturation and integration of GABAergic interneurons and implicated 
      them in the pathogenesis of epileptic encephalopathies or neurodevelopmental 
      disorders. For example, mutations of the Aristaless-Related homeobox X-linked 
      gene (ARX) may result in defective GABAergic interneuronal migration in infants 
      with epileptic encephalopathies like West syndrome (WS), Ohtahara syndrome or 
      X-linked lissencephaly with abnormal genitalia (XLAG). The concept of 
      "interneuronopathy", i.e. impaired development, migration or function of 
      interneurons, has emerged as a possible etiopathogenic mechanism for epileptic 
      encephalopathies. Treatments that enhance GABA levels, may help seizure control 
      but do not necessarily show disease modifying effect. On the other hand, 
      interneuronopathies can be seen in other conditions in which epilepsy may not be 
      the primary manifestation, such as autism. In this review, we plan to outline 
      briefly the current state of knowledge on the origin, development, and migration 
      and integration of GABAergic interneurons, present neurodevelopmental conditions, 
      with or without epilepsy, that have been associated with interneuronopathies and 
      discuss the evidence linking certain types of interneuronal dysfunction with 
      epilepsy and/or cognitive or behavioral deficits.
FAU - Katsarou, Anna-Maria
AU  - Katsarou AM
AD  - Saul R. Korey Department of Neurology, Laboratory of Developmental Epilepsy, 
      Albert Einstein College of Medicine, Bronx, New York, U.S.A.
FAU - Moshe, Solomon L
AU  - Moshe SL
AD  - Saul R. Korey Department of Neurology, Laboratory of Developmental Epilepsy, 
      Albert Einstein College of Medicine, Bronx, New York, U.S.A.
AD  - Montefiore/Einstein Epilepsy Center, Dominick P. Purpura Department of 
      Neuroscience, Albert Einstein College of Medicine, Bronx, New York, U.S.A.
AD  - Department of Pediatrics, Albert Einstein College of Medicine, Bronx, New York, 
      U.S.A.
FAU - Galanopoulou, Aristea S
AU  - Galanopoulou AS
AD  - Saul R. Korey Department of Neurology, Laboratory of Developmental Epilepsy, 
      Albert Einstein College of Medicine, Bronx, New York, U.S.A.
AD  - Montefiore/Einstein Epilepsy Center, Dominick P. Purpura Department of 
      Neuroscience, Albert Einstein College of Medicine, Bronx, New York, U.S.A.
LA  - eng
GR  - R01 NS091170/NS/NINDS NIH HHS/United States
GR  - U54 NS100064/NS/NINDS NIH HHS/United States
PT  - Journal Article
DEP - 20170628
PL  - United States
TA  - Epilepsia Open
JT  - Epilepsia open
JID - 101692036
PMC - PMC5650248
MID - NIHMS876053
OTO - NOTNLM
OT  - GABA
OT  - West syndrome
OT  - autism
OT  - interneuronopathy
OT  - lissencephaly
OT  - neurodevelopmental
OT  - schizophrenia
EDAT- 2017/10/25 06:00
MHDA- 2017/10/25 06:01
PMCR- 2017/06/28
CRDT- 2017/10/25 06:00
PHST- 2017/10/25 06:00 [entrez]
PHST- 2017/10/25 06:00 [pubmed]
PHST- 2017/10/25 06:01 [medline]
PHST- 2017/06/28 00:00 [pmc-release]
AID - EPI412062 [pii]
AID - 10.1002/epi4.12062 [doi]
PST - ppublish
SO  - Epilepsia Open. 2017 Sep;2(3):284-306. doi: 10.1002/epi4.12062. Epub 2017 Jun 28.