Hemiconvulsion Hemiplegia Epilepsy (HHE) Syndrome with Iron Deficiency Anemia among Children

Two cases of hemiconvulsion hemiplegia syndrome with iron deficiency anemia have been presented. Hemiconvulsion hemiplegia epilepsy (HHE) syndrome is a rare complication of prolonged focal seizures in children and is usually idiopathic but may be associated with structural, infective, traumatic, and degenerative diseases. The pathophysiology of this syndrome still remains poorly understood and the long-term cognitive outcomes are still unclear. Magnetic Resonance Imaging (MRI) of the brain in the acute stages characteristically shows gross cerebral edema in the contralateral hemisphere, which later turns into atrophy. Two cases with initial presentation as left-sided focal status epilepticus, later developing left-sided hemiparesis with iron deficiency anemia, have been presented. This is the first association of iron deficiency with hemiconvulsion-hemiplegia-syndrome illustrating the importance of testing for iron deficiency and neuroimaging in each case of status epilepticus. More importantly, these cases also raise the possibility that deficiency in iron could be a contributing factor in cases of HHE. IDA may be a potential trigger to develop seizure and subsequently status epilepticus which may eventually lead to a catastrophic entity like Hemiconvulsion-Hemiplegia Syndrome.