A Case of Intractable Epilepsy with Pachygyria: the Effect of TRH-T Treatment.

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We report a 1-year and 9-month old girl with a pachygyria in the left cerebral hemisphere who showed intractable seizures mimicking severe myoclonic epilepsy in infancy (SMEI) treated with TRH-T. The patient has been admitted to our department 3 times and then treated with intramuscular TRH-T (0.5-1.0 mg daily) successfully. Seizures had been controlled with TRH-T in 21, 10 and 5 days, and thereafter ad-on therapy with peroral TRH-T (1-4 mg daily) has been continued. The drop of plasma prolactin level after TRH-T treatment suggests a possible participation of the inhibitory action of dopaminergic neurons in seizure control. Pachygyria is known to have microdysgenesis of the cerebral cortex, which can be the epileptic focus of the case. The TRH-T can be beneficial in the treatment of intractable seizures mimicking SMEI seen in patients with pacygyria.
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