Abstract
In Genetic Absence Epilepsy Rats from Strasbourg (GAERS), 100% of the animals present recurrent generalized non-convulsive seizures characterized by bilateral and synchronous spike-and-wave discharges (SWD) accompanied with behavioural arrest, staring and sometimes twitching of the vibrissae. Spontaneous SWD (7–11 cps) start and end abruptly on a normal background EEG at a mean frequency of 1.5 per min. Drugs effective against absence seizures in humans suppress the SWD dose-dependently, whereas drugs specific for convulsive or focal seizures are ineffective. Depth EEG recordings and lesion experiments show that SWD in GAERS depend on cortical and thalamic structures with a possible rhythmic triggering by the lateral thalamus; GABAA and GABAB receptors seem to play a critical role. SWD are genetically determined with an autosomal dominant inheritance. The variable expression of SWD in offsprings from GAERS and control reciprocal crosses may be due to the existence of multiple genes.
Sommario
Il 100% degli animali appartenenti al ceppo GAERS (Genetic Absence Epilepsy Rats from Strasbourg) presenta crisi generalizzate non convulsive ricorrenti caratterizzate da scariche di punta-onda (PO) accompagnate da arresto comportamentale, immobilità e talora movimenti rapidi delle vibrisse. Le scariche spontanee di PO (7–11 Hz) iniziano e cessano bruscamente su una attività di fondo EEG normale alla frequenza media di 1.5 per minuto. I farmaci efficaci nella epilessia con assenze tipo piccolo male dell'uomo sopprimono le PO in modo dose-dipendente, mentre i farmaci specifici per le crisi convulsive o parziali sono inefficaci. Registrazioni con elettrodi profondi ed esperimenti di lesione dimostrano che le PO nei GAERS dipendono da strutture corticali e talamiche con una possibile origine del ritmo nella porzione laterale del talamo; i recettori GABAA sembrano giocare un ruolo critico. Le PO sono geneticamente determinate con una ereditarietà autosomica dominante. La variabile espressività delle PO nei nati da incroci tra GAERS e controlli può essere dovuta all'esistenza di geni multipli.
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Marescaux, C., Vergnes, M. Genetic Absence Epilepsy in Rats from Strasbourg (GAERS). Ital J Neuro Sci 16, 113–118 (1995). https://doi.org/10.1007/BF02229083
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DOI: https://doi.org/10.1007/BF02229083