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[A case of intractable epilepsy with pachygyria: the effect of TRH-T treatment]
H Takahashi1, T Nakazawa, C Kanazawa
1Department of Pediatrics, Juntendo University School of Medicine, Chiba.
Insights
Thyrotropin-releasing hormone treatment (TRH-T) effectively controlled intractable seizures in a child with pachygyria. This suggests TRH-T may benefit similar epilepsy cases, potentially via dopaminergic pathways.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Pachygyria, a cortical malformation, can present with intractable seizures.
- Severe Myoclonic Epilepsy in Infancy (SMEI) is a severe form of epilepsy with specific clinical features.
- Thyrotropin-releasing hormone treatment (TRH-T) has been explored for various neurological conditions.
Observation:
- A 1-year and 9-month-old girl with left hemispheric pachygyria experienced intractable seizures mimicking SMEI.
- The patient underwent multiple admissions and received intramuscular TRH-T (0.5-1.0 mg daily).
Findings:
- Intramuscular TRH-T successfully controlled seizures within 21, 10, and 5 days during three treatment courses.
- Subsequent oral TRH-T (1-4 mg daily) was continued.
- A decrease in plasma prolactin levels post-TRH-T suggests dopaminergic neuron involvement in seizure control.
Implications:
- TRH-T demonstrates potential efficacy in managing intractable seizures associated with pachygyria.
- This treatment approach may offer an alternative for patients with pachygyria-related epilepsy refractory to standard therapies.
- The findings suggest a role for dopaminergic modulation in seizure control for these specific cases.
Abstract:
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.