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Hemiconvulsion-hemiplegia-epilepsy syndrome. A clinical, electroencephalographic and neuroradiological study
M A Salih1, M Kabiraj, A S Al-Jarallah
1Department of Paediatrics, College of Medicine, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
Insights
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome in children involves prolonged seizures and hemiplegia. Neuroimaging reveals cerebral hemiatrophy and ipsilateral hypoperfusion, with epilepsy often manageable by medication.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare neurological disorder.
- Characterized by prolonged seizures, hemiplegia, and subsequent epilepsy in children.
Abstract:
Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemiconvulsion in three of the children and generalized tonic-clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.