A model of symptomatic infantile spasms syndrome

Morris H. Scantlebury, Aristea S. Galanopoulou, Lenka Chudomelova, Emmanuel Raffo, David Betancourth, Solomon L. Moshe

Research output: Contribution to journalArticle

75 Citations (Scopus)

Abstract

Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.

Original languageEnglish (US)
Pages (from-to)604-612
Number of pages9
JournalNeurobiology of Disease
Volume37
Issue number3
DOIs
StatePublished - Mar 2010

Fingerprint

Infantile Spasms
Spasm
Adrenocorticotropic Hormone
Electroencephalography
Fenclonine
Vigabatrin
Grooming
Neurobiology
Autistic Disorder
Doxorubicin
Lipopolysaccharides
Epilepsy
Seizures
Stroke
Learning
Pathology
Therapeutics

Keywords

  • Animal model
  • Autism
  • Doxorubicin
  • Epilepsy
  • Infantile spasms
  • Lipopolysaccharide
  • Neonatal rat
  • p-Chlorophenylalanine
  • West syndrome

ASJC Scopus subject areas

  • Neurology

Cite this

A model of symptomatic infantile spasms syndrome. / Scantlebury, Morris H.; Galanopoulou, Aristea S.; Chudomelova, Lenka; Raffo, Emmanuel; Betancourth, David; Moshe, Solomon L.

In: Neurobiology of Disease, Vol. 37, No. 3, 03.2010, p. 604-612.

Research output: Contribution to journalArticle

Scantlebury, MH, Galanopoulou, AS, Chudomelova, L, Raffo, E, Betancourth, D & Moshe, SL 2010, 'A model of symptomatic infantile spasms syndrome', Neurobiology of Disease, vol. 37, no. 3, pp. 604-612. https://doi.org/10.1016/j.nbd.2009.11.011
Scantlebury, Morris H. ; Galanopoulou, Aristea S. ; Chudomelova, Lenka ; Raffo, Emmanuel ; Betancourth, David ; Moshe, Solomon L. / A model of symptomatic infantile spasms syndrome. In: Neurobiology of Disease. 2010 ; Vol. 37, No. 3. pp. 604-612.
@article{3eaf1e980d3c424b9ff58aeebe5fa7b7,
title = "A model of symptomatic infantile spasms syndrome",
abstract = "Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.",
keywords = "Animal model, Autism, Doxorubicin, Epilepsy, Infantile spasms, Lipopolysaccharide, Neonatal rat, p-Chlorophenylalanine, West syndrome",
author = "Scantlebury, {Morris H.} and Galanopoulou, {Aristea S.} and Lenka Chudomelova and Emmanuel Raffo and David Betancourth and Moshe, {Solomon L.}",
year = "2010",
month = "3",
doi = "10.1016/j.nbd.2009.11.011",
language = "English (US)",
volume = "37",
pages = "604--612",
journal = "Neurobiology of Disease",
issn = "0969-9961",
publisher = "Academic Press Inc.",
number = "3",

}

TY - JOUR

T1 - A model of symptomatic infantile spasms syndrome

AU - Scantlebury, Morris H.

AU - Galanopoulou, Aristea S.

AU - Chudomelova, Lenka

AU - Raffo, Emmanuel

AU - Betancourth, David

AU - Moshe, Solomon L.

PY - 2010/3

Y1 - 2010/3

N2 - Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.

AB - Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.

KW - Animal model

KW - Autism

KW - Doxorubicin

KW - Epilepsy

KW - Infantile spasms

KW - Lipopolysaccharide

KW - Neonatal rat

KW - p-Chlorophenylalanine

KW - West syndrome

UR - http://www.scopus.com/inward/record.url?scp=75949111993&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=75949111993&partnerID=8YFLogxK

U2 - 10.1016/j.nbd.2009.11.011

DO - 10.1016/j.nbd.2009.11.011

M3 - Article

VL - 37

SP - 604

EP - 612

JO - Neurobiology of Disease

JF - Neurobiology of Disease

SN - 0969-9961

IS - 3

ER -