Intravenous ganaxolone in pediatric super-refractory status epilepticus: A single hospital experience.

Synaptic GABA receptor (GABAR) internalization contributes to the drug resistant nature of super-refractory status epilepticus (SRSE). Ganaxolone is a 3β-methylated synthetic analog of the endogenous neuroactive steroid, allopregnanolone, that has positive allosteric modulatory activity on synaptic and extrasynaptic GABA receptors. Ganaxolone is currently in clinical trials to treat rare pediatric seizure disorders and established and refractory SE.

Drug-Drug Interactions Between Cannabidiol and Lithium.

Epidiolex (Cannabidiol- CBD) is approved for epilepsy associated with Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) in patients over 2 years of age. Common side effects include somnolence and diarrhea. Recent studies have demonstrated interactions between cannabidiol and several other antiseizure medications.

Cerebellar seizures.

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.

Passive language mapping with magnetoencephalography in pediatric patients with epilepsy.

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.

Mesial temporal sclerosis in a cohort of children with SCN1A gene mutation.

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.

Alexander disease: An important mimicker of focal brainstem glioma.

We report the case of a 6-year-old male who was referred to a tertiary oncology center with a focal brainstem lesion which was presumed to be neoplastic. Due to the symmetric nature of the lesion on magnetic resonance imaging, the evaluation was expanded to investigate other possible causes and eventual diagnosis of Alexander's disease (AD) was made. AD is a neurodegenerative disease which must be included in the differential for tumor-like lesions within the posterior fossa.