Affiliations: Department of Pediatric Neurology, Children's Hospital
Sheffield, Sheffield, UK | Department of Neuroradiology, Children's Hospital
Sheffield, Sheffield, UK
Note: [] Correspondence: Dr. Santosh R. Mordekar,
Ryegate Children's Centre, Tapton Crescent, Sheffield S10 5DD, UK.
Tel.: +44 (0) 114 2260675; Fax: +44 (0) 114 2678296; E-mail:
santosh.mordekar@sch.nhs.uk
Abstract: We describe four children with an apparently unique form of easily
treatable early onset epilepsy. All had an unremarkable family history,
pregnancy and delivery. One child presented with neonatal seizures. None of
them had neonatal jaundice. They presented with developmental impairment,
cortical visual agnosia or squint, and clusters of epileptic spasms [3]
beginning in infancy. Electroencephalogram showed features of modified
(atypical) hypsarrhythmia. Neuroimaging in infancy showed unilateral or
bilateral signal changes in the globus pallidus. All infants were treated with
high dose vigabatrin (VGB). The seizure disorder responded well to treatment
and VGB was discontinued. Repeat magnetic resonance imaging brain scan showed
complete resolution of the signal changes in the globus pallidi. All remained
seizure free but continued to show severe intellectual disability. We suggest
that VGB treatment was associated with "reversible" signal changes in the
globus pallidi in these children.
