Hemispherotomy in an infant with hemimegalencephaly

Abstract

Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain. The epilepsy pattern in HME can be partial seizures or may present as spasms as in epileptic encephalopathy. Epilepsy associated with HME is usually resistant to antiepileptic drugs and requires surgical intervention. Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay. We report a case of HME, who underwent a two-stage hemispherotomy due to complications in the first surgery. She had more than 90% reduction of seizures with good developmental outcome on follow-up. Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

Refractory neonatal seizures caused by hemimegalencephaly

“…Similarly, Kulkarni et al 5 reported a term infant with HME who had more than 90% reduction in seizures with good developmental outcome on follow-up after hemispherectomy. In addition, Chand and colleagues8 reported a neonate who presented with focal seizures.…”

“…HME is a hamartomatous malformation of the brain 3. Kulkarni et al 5 reported a prevalence ranging from 1 to 3 cases per 1000 children with epilepsy and 1%–14% among children with cortical developmental abnormalities. Abnormalities of cortical development can be classified as follows: abnormal neural proliferation (diffuse cortical dysplasia, microlissencephaly and HME), abnormal neural migration (nodular heterotopia, double cortex, band heterotopia, pachygyria-agyria and lissencephaly) and abnormal postmigrational development (polymicrogyria, focal cortical dysplasia and schizencephaly) 6…”

A case of epilepsia partialis continua due to linear nevus syndrome with hemimegalencephaly

“…[ 14 ] Despite the risks, early surgery should be preferred for major reduction in seizure frequency and better developmental outcome. [ 15 ]…”

Hemimegalencephaly: Evolution From an Atypical Focal Early Appearance on Fetal MRI to More Conventional MR Findings

“…Hemimegalencephaly is a sporadic disorder. The prevalence is distributed equally by sex and is believed to be 1-3 per 1,000 epileptic children [1,9]. It is an isolated finding in the majority of cases, but may also be associated with neurocutaneous syndromes, including epidermal nevus syndrome, proteus syndrome, hypomelanosis of Ito, neurofibromatosis type I, Klippel-Trenaunay syndrome, and tuberous sclerosis [1,2,4].…”