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Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy
Rosa Martinez-Mañas1, Roy Thomas Daniel, Damien Debatisse
1Department of Neurosurgery, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 46, Lausanne 1011, Switzerland.
Seizure
|August 25, 2004
Summary
Infantile hemiplegia seizure syndrome (IHSS) with reflex audiogenic epilepsy, caused by birth injury, was successfully treated with hemispherotomy. This case highlights an uncommon presentation and effective surgical intervention for intractable seizures.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Infantile hemiplegia seizure syndrome (IHSS) is a severe neurological disorder.
- Reflex audiogenic epilepsy is a rare seizure subtype triggered by sound.
- Iatrogenic birth injuries can lead to significant neurological deficits.
Observation:
- A unique case of IHSS presented with intractable reflex audiogenic startle epilepsy.
- The condition stemmed from an iatrogenic brain injury during a cesarean section.
- Peri-insular hemispherotomy was performed for seizure management.
Findings:
- Hemispherotomy achieved complete seizure control in this patient.
- The study reviews mechanisms of birth injury and physiopathogenesis of hemispheric damage.
- This is the first reported instance of intractable reflex audiogenic epilepsy in IHSS treated with hemispherotomy.
Implications:
- Hemispherotomy is a viable surgical option for intractable reflex audiogenic epilepsy in IHSS.
- Understanding birth injury mechanisms is crucial for preventing neurological sequelae.
- This case expands the understanding of IHSS and its diverse clinical presentations.