Summary:Purpose: Children with autism are commonly referred for video-EEG monitoring to determine the precise nature of their seizure-like events.Methods: We studied 32 children with autism by using continuous video-EEG telemetry (VEEG) monitoring at a tertiary care referral center.Results: Of the 32 total patients, 22 were primarily referred for seizure evaluation and 10 for 24-h interictal EEG recording. Studies in two additional patients were prematurely terminated because of intolerance (they are not included in the analyses). The median monitoring duration was 1 day (range, 1-7 days). Of 22 patients referred for seizure evaluation, 15 had recorded events, but none was an epileptic seizure; the other seven patients had no recorded events. Interictal epileptiform EEG abnormalities were detected in 19 (59%) of 32 patients. These abnormalities included focal sharp waves (in eight patients), multifocal sharp waves (in six patients), generalized spike-wave complexes (in 11 patients), and generalized paroxysmal fast activity/polyspikes (in two patients). Focal/multifocal and generalized epileptiform abnormalities coexisted in six patients. Notably, 11 (73%) of the 15 patients with nonepileptic events had interictal epileptiform EEG abnormalities.Conclusions: Video-EEG evaluation of children with autism reveals epileptiform EEG abnormalities in the majority. However, many recorded seizure-like events are not epileptic, even in children with epileptiform EEG abnormalities.

MEG studies can now be performed on a routine basis as a clinical tool. MEG is now indicated for: 1) localization of the irritative zone in lesional and nonlesional epilepsy surgery patients, 2) functional mapping of eloquent cortex, and 3) assessment of normal and abnormal language development. In the future MEG may help the understanding of normal development and reorganization after brain injury. The neurologist can use MEG data to complement structural and metabolic imaging techniques.

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We evaluated our clinical experience with zonisamide, a broad-spectrum antiepileptic drug, in a group of children with predominantly medically refractory epilepsy. A retrospective chart review was conducted on patients at our tertiary referral center following Institutional Review Board approval. Observers documented reports of seizure frequency, and seizure types were identified either clinically or by prior video-electroencephalography monitoring. We identified 68 patients (age range 1.9-18.1 years [median 6.9 years]; male to female ratio 1.3:1) treated with zonisamide for 0.7 to 28.9 months; at the last visit, 22% and 78% were on monotherapy and adjunctive therapy, respectively. The median duration of treatment and maintenance dose at the end of the follow-up were 11.2 months and 8.0 mg/kg/day, respectively. Seizure types included generalized (primary generalized tonic-clonic, myoclonic, tonic, atonic, absence) and partial (simple, complex, and secondarily generalized tonic-clonic seizures); 10 (15%) patients had both partial and generalized seizures. Sixteen (25.8%) patients were seizure free, although five of them were already in remission prior to starting zonisamide. Thirteen (21.0%) patients had a > 50% seizure reduction, 10 (16.1%) patients had a < 50% seizure reduction, 14 (22.6%) had no improvement in baseline seizures, and 9 (14.5%) reported having increased seizures. The latter were mostly associated with dosage alterations in concomitant antiepileptic drugs. Common side effects were central nervous system related, including behavioral or psychiatric (23.5%), cognitive dysfunction (12.0%), and sedation (10.3%). Eleven (16.2%) patients ultimately discontinued zonisamide, but only five were strictly due to side effects. Zonisamide is clinically effective against multiple seizure types in a significant proportion of children with epilepsy across a broad age range. Drug discontinuation as a result of side effects is uncommon.

Summary:The treatment of complex partial status epilepticus continues to be controversial, especially with regard to the intensity of the treatment. Medical therapy and drug-induced coma are sometimes required. Rarely this may not be effective. A healthy 4-year old girl was first seen in complex partial status epilepticus. She had a 1-year history of cryptogenic partialonset seizures. Detailed magnetic resonance imaging (MRI) studies were normal. Her course was refractory to multiple medical therapies and multiple subpial transection (MST). An urgent epilepsy surgery evaluation resulted in a focal cortical resection being performed over the right mesial parietal region with resultant seizure freedom and no significant neurologic deficit 2 years later. This patient illustrates the need to consider occult focal cortical dysplasia as a cause of nonconvulsive status epilepticus (NCSE) in children, and if it is not responsive to medical management, the utility of performing an urgent epilepsy surgery evaluation.