An avian model of genetic reflex epilepsy.
The Fayoumi strain of chickens (Fepi) carries a recessive autosomal gene mutation in which homozygotes are afflicted with a photogenic and audiogenic reflex epilepsy. Seizures consist of stimulus-locked motor symptoms followed by generalized self sustained convulsions. EEG recordings show spikes and spike and waves patterns at rest which are suppressed during seizures and replaced by a desynchronized pattern of activity. Neurones of the prosencephalon discharge in bursts at rest, while neurones of the mesencephalon are bursting during seizures. Living neural chimeras were obtained by replacing specific embryonic brain vesicles in a normal chicken embryo with equivalent vesicles from a Fepi donor. These chimeras show that the epileptic phenotype can be totally or partially transferred from the Fepi to the normal chickens. Total transfer of photogenic and audiogenic seizures was obtained by substitution of both the prosencephalon and mesencephalon, while substitution of the prosencephalon alone resulted in transfer of interictal paroxysmal activity and substitution of the mesencephalon alone resulted principally in transfer of ictal motor symptoms. Increased expression of the c-fos protooncogene, as revealed by the western blot technique, confirmed the distinct encephalic localizations of the symptoms of the photogenic and audiogenic reflex epilepsy of the Fepi shown with the methods of electrophysiology and brain chimeras. We conclude that the Fepi is a good model of brain stem reflex epilepsy and suggest that the brain stem is a generator of some other animal and human genetic reflex "epileptic syndromes".
Batini
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LGN, CNRS UMR 7091, Université Pierre et Marie Curie, CHU Pitié Salpetrière, Batiment Cervi, 91 Bd. de l'Hopital, 75634, Paris, France.
Teillet
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Naquet
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D000200:Action Potentials / Q000235:genetics; D000818:Animals; D001921:Brain / Q000196:embryology / Q000254:growth & development / Q000503:physiopathology*; D002642:Chick Embryo; D002645:Chickens / Q000235:genetics* / Q000254:growth & development; D002678:Chimera / Q000235:genetics; D004195:Disease Models, Animal; D004569:Electroencephalography; D020195:Epilepsy, Reflex / Q000151:congenital* / Q000235:genetics* / Q000503:physiopathology; D005808:Genes, Recessive; D006801:Humans; D010775:Photic Stimulation / Q000009:adverse effects; D016760:Proto-Oncogene Proteins c-fos / Q000235:genetics / Q000378:metabolism
D016760:Proto-Oncogene Proteins c-fos