Twenty to thirty percent of children with epilepsy continue to have seizures despite anti-epileptic drugs (AEDs) treatment and are defined as medically intractable epilepsy. Intractable epilepsy frequently has developmental impact, causing permanent neurodevelopmental deficits in children. Recently developed neurodiagnostic studies and treatment modalities have substantially altered the management of patients with intractable epilepsy. The newly developed AEDs are frequently used for the treatment of patients unresponsive to conventional AEDs. Nonpharmacologic options for intractable epilepsy include dietary therapy such as ketogenic diet and modified Atkins diet, epilepsy surgery, and neuromodulatory treatments such as vagus nerve stimulation. Ketogenic diet is an effective and safe treatment for epilepsy in children; increasing use has led to modification of protocol for higher efficacy and better tolerability. When epilepsy is intractable to medical treatment, epilepsy surgery including surgical resection of epileptogenic area, or palliative surgery can be considered. Vagal Nerve Stimulation is recommended for patients who are not candidates for resective surgery. These nonpharmcologic treatments could result in resumption of developmental progress in patients with childhood intractable epilepsy.Active application of newly developed medical and surgical treatments could provide better outcome in seizure control and developmental progress in patients with intractable epilepsy.

View full abstract

Purpose: To demonstrate the preliminary result of surgical treatment in a university-based hospital in Thailand.
Methods: The medical records of children and adolescents who received surgical treatment for epilepsy at Ramathibodi Hospital, Bangkok, Thailand from October 2005 till July 2007 were reviewed.
Results: 38 children and adolescents (21 males and 17 females; ages ranged from 14 months to 18 ½ years, mean 9.5 years, median 9.75 years) were included in this study. 18 and 20 were classified into symptomatic and cryptogenic/idiopathic groups, respectively. Lennox-Gastaut syndrome, West syndrome, tuberous sclerosis and Sturge-Weber syndrome were diagnosed in 10, 3, 2 and 1 patients, respectively. Presurgical evaluation consisted of ictal/interictal video-monitored EEG recording, MRI of brain and ictal/interictal SPECT scans. Surgical treatment were lesionectomy, lobectomy, corpus callosotomy, corpus callosotomy with lobectomy, anterior temporal lobectomy with amydalohippocampectomy, functional hemishperectomy and hemispherotomy in 11, 9, 8, 2, 5, 2 and 1 patients, respectively. Follow-up durations ranged from 1 to 19 months. The outcomes were freedom from seizure, significant seizure reduction, minimal seizure reduction, and no frequency change in 22, 11, 3 and 2 patients, respectively. One antiepileptic drug was removed from the treatment regimen in 11 patients. No patient deteriorated after treatment. The majority of parents reported improvement of social interaction and motor functions.
Conclusion: Although this is a report of a small number of patients with short follow-up duration, the preliminary result is in favor of surgery as an option for the treatment of childhood and adolescent intractable epilepsy in Thailand.

View full abstract

We present a surgical case of a 53-year-old man with lesion-related epilepsy associated with medullary venous malformation (MVM) and cavernous malformation (CM) in the right frontal lobe. He had frequent secondary generalized seizures since developing a MVM-related intracerebral hemorrhage in the right frontal lobe at the age of 44. Intraoperative electrocorticography demonstrated frequent paroxysmal activities on the yellowish cortex above the CM. Anterior frontal lobectomy including the irritative cortex, CM, MVM and its tributaries was performed. The patient became seizure free after surgery. Pathologically, the irritative cortex above the CM had hemosiderin deposits and astrogliosis. The CM was surrounded by dilated veins of various sizes, indicating MVM, which confirmed the radiological findings of so-called caput medusae. Epileptogenicity in patients with CM and MVM is thought to be due to repeated hemorrhage from the CM. Surgical strategy is discussed.

View full abstract

Purpose: While many patients with childhood-onset epilepsy go into remission before reaching adulthood, a significant number of patients continue to suffer from refractory epilepsy. The purpose of this study was to clarify the long-term outcome of childhood-onset epilepsies.
Subjects and Methods: We retrospectively studied 445 adult patients with childhood-onset epilepsies who were still being treated at the Department of Child Neurology, Okayama University Hospital.
Results: Age at onset of epilepsy was < 7 years in 66% of the patients; 7 to 14 years in 30%; and 15 to 19 years in 5%. We classified the subjects into three groups: Group PE (289 patients) with partial epilepsy; Group GE (60 patients) with generalized epilepsy; Group RE (70 patients) consisting of patients with a history of West syndrome, Lennox-Gastaut syndrome, Doose syndrome and related epileptic syndromes; and 26 other unclassified cases. At follow-up, frequent seizures (≥ per month) were observed in 26%, 13% and 63% of patients in Groups PE, GE and RE, respectively. Of 168 patients in remission across these three groups, AEDs were discontinued or being reduced in 21% each, while 36% had experienced relapse of seizures, mainly caused by AED withdrawal.
Discussion: This study indicated that these adult patients could be classified into two types: patients who still have frequent seizures even after reaching adulthood, and patients in remission or with rare seizures. For long-term management of these patients, an efficient system with cooperation between medical and comedical staff, and a comprehensive care system are essential.

View full abstract

Febrile seizure (FS), autosomal dominant epilepsy with FS plus (ADEFS+), and severe myoclonic epilepsy of infancy (SMEI) are all triggered by high fever at the onset of disorder, but are clinically distinct from each other. Although various identified gene mutations account for approximately 15% of families with ADEFS+ and 80% of patients with SMEI, the responsible gene for FS is essentially unknown. The present study was conducted to identify the loci associated with the risk of these 3 disorders. Among 154 patients (77 with SMEI, 34 with ADEFS+ and 43 with FS), we found 21, 24 and 27 novel susceptibility loci associated with the three diseases, respectively. This is the first phase of a two-part study on the genetic risk profile for fever-related seizure disorders.

View full abstract

Dramatic aggravation of partial seizures, unreported until now, was observed in association with gabapentin (GBP) overdose in an 18-year-old man with symptomatic localization-related epilepsy accompanying focal cortical dysplasia in the right occipital lobe. The patient compulsively took 13400 mg GBP in a single dose one evening. The next morning he was ataxic and had very frequent simple partial seizures with eye deviation to the left, which appeared similar to his habitual seizure. The patient's blood level of GBP was 17.62 μg/ml, and laboratory examinations detected no abnormalities. Ictal EEG, however, showed that the origin of the induced seizures was different from that of his habitual seizures. His ataxia and seizures subsided immediately after cessation of GBP and hydration, with no sequel. This case shows that occasional induction of seizures by GBP is not limited to myoclonus or absence seizures but may involve partial seizures in case of overdose.

View full abstract