PMID- 27527919
OWN - NLM
STAT- MEDLINE
DCOM- 20170605
LR  - 20181113
IS  - 1528-1167 (Electronic)
IS  - 0013-9580 (Print)
IS  - 0013-9580 (Linking)
VI  - 57
IP  - 10
DP  - 2016 Oct
TI  - Continuous spike-waves during slow-wave sleep in a mouse model of focal cortical 
      dysplasia.
PG  - 1581-1593
LID - 10.1111/epi.13501 [doi]
AB  - OBJECTIVE: To examine if mice with focal cortical dysplasia (FCD) develop 
      spontaneous epileptic seizures and, if so, determine the key 
      electroencephalography (EEG) features. METHODS: Unilateral single freeze lesions 
      to the S1 region (SFLS1R) were made in postnatal day 0-1 pups to induce a 
      neocortical microgyrus in the right cortical hemisphere. Continuous 24-h 
      recordings with intracranial EEG electrodes and behavioral tests were performed 
      in adult SFLS1R and sham-control mice to assess neurologic status. RESULTS: A 
      high percentage of adult SFLS1R animals (89%, 40/45) exhibited at least one or 
      more spontaneous nonconvulsive seizure events over the course of 24 h. Of these 
      animals, 60% (27/45) presented with a chronic seizure state that was persistent 
      throughout the recording session, consisting of bursts of rhythmic high-amplitude 
      spike-wave activities and primarily occurring during periods of slow-wave sleep. 
      In comparison, none of the control, age-matched, mice (0/12) developed seizures. 
      The epileptic discharge pattern closely resembled a pattern of continuous 
      spike-waves during slow-wave sleep (CSWS) of the human syndrome described as an 
      electrical status epilepticus during slow-wave sleep (ESES). Key findings in the 
      SFLS1R model indicated that the observed CSWS (1) were more prevalent in female 
      (18/23) versus male (9/22, p < 0.05), (2) were strongest in the right S1 region 
      although generalized to other brain regions, (3) were associated with significant 
      cognitive and behavioral deficits, (4) were temporarily alleviated by 
      ethosuximide treatment or optogenetic activation of cortical gamma-aminobutyric acid 
      (GABA)ergic neurons, and (5) theta and alpha band rhythms may play a key role in 
      the generalization of spike-wave activities. SIGNIFICANCE: This is the first 
      report of an in vivo animal FCD model that induces chronic spontaneous 
      electrographic brain seizures. Further characterization of the abnormal 
      oscillations in this mouse model may lead to a better understanding of the 
      mechanisms of CSWS/ESES.
CI  - Wiley Periodicals, Inc. (c) 2016 International League Against Epilepsy.
FAU - Sun, Qian-Quan
AU  - Sun QQ
AD  - Department of Zoology and Physiology, University of Wyoming, Laramie, Wyoming, 
      U.S.A. neuron@uwyo.edu.
FAU - Zhou, Chen
AU  - Zhou C
AD  - Department of Zoology and Physiology, University of Wyoming, Laramie, Wyoming, 
      U.S.A.
FAU - Yang, Weiguo
AU  - Yang W
AD  - Department of Zoology and Physiology, University of Wyoming, Laramie, Wyoming, 
      U.S.A.
FAU - Petrus, Daniel
AU  - Petrus D
AD  - Department of Zoology and Physiology, University of Wyoming, Laramie, Wyoming, 
      U.S.A.
LA  - eng
GR  - P20 GM103432/GM/NIGMS NIH HHS/United States
GR  - R01 NS094550/NS/NINDS NIH HHS/United States
GR  - R21 NS084182/NS/NINDS NIH HHS/United States
PT  - Journal Article
DEP - 20160816
PL  - United States
TA  - Epilepsia
JT  - Epilepsia
JID - 2983306R
RN  - 0 (Channelrhodopsins)
RN  - 0 (Vesicular Inhibitory Amino Acid Transport Proteins)
RN  - 0 (vesicular GABA transporter)
SB  - IM
MH  - Animals
MH  - Animals, Newborn
MH  - Brain Mapping
MH  - Brain Waves/*physiology
MH  - Channelrhodopsins
MH  - Disease Models, Animal
MH  - Electroencephalography
MH  - Epilepsy, Generalized/*etiology
MH  - Exploratory Behavior
MH  - Female
MH  - Freezing/adverse effects
MH  - Functional Laterality/physiology
MH  - Male
MH  - Malformations of Cortical Development/*complications/etiology
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Transgenic
MH  - Sleep Stages/*physiology
MH  - Vesicular Inhibitory Amino Acid Transport Proteins/genetics/metabolism
PMC - PMC5423360
MID - NIHMS860176
OTO - NOTNLM
OT  - Cortical malformation
OT  - Freeze lesion
OT  - Optogenetic
OT  - Spike-wave seizure
COIS- Disclosure of Conflicts of Interest: None. Ethical Publication Statement: we 
      confirm that we have read the Journal's position on issues involved in ethical 
      publication and affirm that this report is consistent with those guidelines.
EDAT- 2016/08/17 06:00
MHDA- 2017/06/06 06:00
PMCR- 2017/05/09
CRDT- 2016/08/17 06:00
PHST- 2016/07/21 00:00 [accepted]
PHST- 2016/08/17 06:00 [pubmed]
PHST- 2017/06/06 06:00 [medline]
PHST- 2016/08/17 06:00 [entrez]
PHST- 2017/05/09 00:00 [pmc-release]
AID - 10.1111/epi.13501 [doi]
PST - ppublish
SO  - Epilepsia. 2016 Oct;57(10):1581-1593. doi: 10.1111/epi.13501. Epub 2016 Aug 16.