Mesial temporal sclerosis is uncommon in childhood but has been associated with febrile status epilepticus. SCN1A gene mutations are linked to multiple epilepsy syndromes with patients frequently presenting with prolonged febrile seizures. After observing mesial temporal sclerosis in a child with SCN1A gene mutation, we retrospectively reviewed magnetic resonance imaging (MRI) findings in all patients with SCN1A gene mutation identified between 2005 and 2010. We identified 20 patients with SCN1A mutations. Six patients had evidence of definite mesial temporal sclerosis with 2 patients having bilateral abnormalities. Another 4 patients were defined as having possible mesial temporal sclerosis. This patient group revealed that 50% had findings consistent with definite or possible mesial temporal sclerosis and many did not have a history of prolonged febrile seizures. We conclude that mesial temporal sclerosis is a common finding in children with SCN1A mutations. Many of these children will have Dravet syndrome but not all.

Object Functional mapping is important for determining surgical candidacy and also in epilepsy surgery planning. However, in young children and uncooperative patients, language mapping has been particularly challenging despite the advances in performing noninvasive functional studies. In this study the authors review a series of children with epilepsy who underwent language mapping with magnetoencephalography (MEG) while sedated or sleeping, to determine receptive language localization for presurgical evaluation. Methods The authors undertook a retrospective review of patients who underwent MEG between December 2007 and July 2009, and identified 15 individuals who underwent passive language testing as part of their presurgical evaluation because they were unable to participate in traditional language testing, such as Wada or functional MRI. Factors necessitating passive language testing included age and neurocognitive development. Results Three of the 15 patients were deemed candidates for epilepsy surgery based on the results from standard preoperative testing, including video electroencephalography, MRI, and passive receptive language testing using MEG technology. The MEG studies were used successfully to localize language in all 3 patients, creating opportunities for seizure freedom through surgery that would not otherwise have been available. All 3 patients then underwent resective epilepsy surgery without experiencing postoperative language deficits. Conclusions This case series is the first to look at language mapping during sleep (passive language mapping) in which MEG was used and is the first to evaluate passive language testing in a patient population with intracranial pathological entities. This case series demonstrates that MEG can provide an alternative method for receptive language localization in patients with barriers to more traditional language testing, and in these 3 cases surgery was performed safely based on the results.

Epilepsy, especially with refractory seizures, is thought to arise only from cortical lesions or substrate. The authors report on 2 patients with refractory epilepsy and cerebellar lesions. Depth electrodes were placed within the cerebellar lesions in both patients, and intracranial electroencephalographic recordings showed seizure origin from the cerebellar lesions. One patient eventually attained seizure control with antiepileptic drugs. The other case involved a child with generalized myoclonic epilepsy associated with a pilocytic astrocytoma of the cerebellum. This patient obtained seizure control following gross-total resection of the tumor.