Animal Models of Neurological Disease, II Metabolic Encephalopathies and Epilepsies

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A first step to further explore this hypothesis would be to study stiripentol and topiramate, and evaluate their effects on both types of seizures. It would also be interesting to study lamotrigine to identify whether this model may recapitulate the worsening effect observed in humans [ ].

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The use of the DS model with a deletion of SCN1A is interesting for such a translational approach because more than mutations have been described among patients with DS [ ]. Regarding the study of new treatment strategies, the animal model of DS generated with a stop codon could be an interesting tool; it would permit exploration of treatments for the suppression of nonsense mutations. Genetic and biochemical studies have demonstrated that aminoglycoside antibiotics bind a specific site in ribosomal RNA and disturb codon-anticodon recognition at the aminoacyl-tRNA Transfer RNA acceptor site.

As a result, the ribosome introduces missense mutations and translates through termination codons in bacteria, as well as eukaryotic cells. Aminoglycoside antibiotics are able to disrupt translational fidelity in both species. There are now data showing that the growth of mammalian cells in the presence of an aminoglycoside antibiotic can suppress nonsense mutations [ ]. In vitro and in vivo studies have suggested that this strategy may be relevant for human diseases.

In a mouse model of Duchenne muscular dystrophy, gentamicin was used to disrupt translational fidelity to phenotypically correct a nonsense mutation [ ].

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This type of treatment might be used only with mouse models that use a codon stop. The need for pediatric epilepsy models has been stressed for many years [ 13 ]. Ideal models recapitulating all aspects of each pediatric epilepsy syndrome are not to be expected. However, increasing the number of animal models would enable approaches for the various aspects of each syndrome.

The models of infantile spasms are a good example.

To understand and treat early onset epileptic encephalopathies caused by KCNQ2 mutations.

Studies using several models would permit evaluation of the effects of treatment in models mimicking various etiologies. There are still epilepsy syndromes without any relevant model e. An improvement of our understanding of the various pediatric epilepsy syndromes through more cellular and molecular data using genetic, human tissue, imaging investigations is required to develop new animal models of pediatric epilepsy.

Moreover, a better understanding of the relationship between brain development and epilepsy is also a necessity. Finally, we need to improve our knowledge regarding the presence of therapeutic targets in both laboratory rodent and human brains across development to decrease the gap between preclinical data and successful clinical trials. Disclosure forms provided by the authors are available with the online version of this article. Skip to main content Skip to sections.

Animal Models of Neurological Disease, II

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Novel Animal Models of Pediatric Epilepsy. Review First Online: 31 March Introduction Epilepsy is suspected when there is a repetition of seizures. Given the wide diversity of epilepsy types that present at different developmental ages, certain clinical questions must be considered to identify the models that are needed. Animal models of pediatric epilepsy should permit the study of epilepsy syndromes with high incidences. To illustrate the current need for animal models of pediatric epilepsies, Fig.

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Hyperthermic seizures induced in postnatal day 10 or 11 PP11 rat pups are used to mimic prolonged febrile seizure [ 18 ]. Hypoxic-ischemic brain insults can also be induced in rat pups to model hypoxic-ischemic encephalopathy, which is a prominent cause of mortality in neonates and morbidities, including epilepsy, in children [ 19 ].

Animal models involving hypoxia in P7 to P10 rats have been used to study both acute neonatal seizures and the subsequent development of epilepsy [ 20 , 21 ]. Status epilepticus SE induced by pilocarpine is used in rats from P14 to adulthood to induce significant epileptogenesis and resulting spontaneous seizures [ 22 , 23 ].

For many years, the development of an IS model appeared to be a real challenge [ 13 ].

Epileptic Encephalopathy in Infants and Children - Europe PMC Article - Europe PMC

Recently, several new infantile spasm models have been published, creating new hope for better understanding of the underlying mechanisms of IS. Four of these models are chemically induced, and 2 are based on genetically modified mice. These models permit a representation of the etiological spectrum that is observed in children with IS. The models that are induced by endogenous and exogenous toxins or stress hormones represent acquired causes, whereas genetic causes are represented by Ts65Dn mice, which are a model of Down syndrome or the Aristaless-related homeobox ARX model.

Methylazoxymethanol Acetate In utero exposure of rats to MAM results in reproducible brain abnormalities that include microcephaly, heterotopic cell clusters in the hippocampus and cortex, and abnormally located cell clusters in the periventricular region [ 73 ].

Novel Animal Models of Pediatric Epilepsy

In Utero Irradiation The use of in utero radiation on postnatal brain structures and its behavioral consequences are dependent on both the timing and the dose of radiation [ 84 , 85 , 86 ]. Focally Induced Lesions Because MAM produces widespread lesions, some authors have developed a model using unique focal lesions. Required Author Forms Disclosure forms provided by the authors are available with the online version of this article.

Guerrini R. Epilepsy in children. Lancet ;— Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, — Epilepsia ;— Proposal for revised clinical and electroencephalographic classification of epileptic seizures. CrossRef Google Scholar.

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Epilepsy Behav ;7 suppl 3 :SS Jensen FE. Pediatric epilepsy models. Epilepsy Res ;— Engel J, Schwartzkroin PA. What should be modeled? Models of seizures and epilepsy. Amsterdam: Elsevier, — Google Scholar. Models of pediatric epilepsies: startegies and opportunities. Sarkisian MR. Overview of the current animal models for human seizure and epileptic disorders.


Epilepsy Behav ;— Epileptogenesis in experimental models. Epilepsia ;48 suppl 2 — Does acquired epileptogenesis in the immature brain require neuronal death. Epilepsy Curr ;— Is neuronal death necessary for acquired epileptogenesis in the immature brain? Complex febrile seizure-An experimental model in immature rodents. Oxford, UK: Elsevier, — Wallace SJ. Epilepsy in cerebral palsy. Dev Med Child Neurol ;— Continuous electroencephalographic monitoring with radio-telemetry in a rat model of perinatal hypoxia-ischemia reveals progressive post-stroke epilepsy. J Neurosci ;— Development of later life spontaneous seizures in a rodent model of hypoxia-induced neonatal seizures.

Patterns of status epilepticus-induced neuronal injury during development and long-term consequences.


PubMed Google Scholar. Epilepsy after early-life seizures can be independent of hippocampal injury. Ann Neurol ;— Seizure-induced neuronal injury: vulnerability to febrile seizures in an immature rat model. Mossy fiber plasticity and enhanced hippocampal excitability, without hippocampal cell loss or altered neurogenesis, in an animal model of prolonged febrile seizures.