In this article, the studies on EL mouse as an model of epilepsy carried out in our laboratory since the preceding review by the author was published in 2003 are described. Experimental animals were EL and DDY (or few ddN) mice. Using the microelectrodes the activities of the parietal cortex (PCX) neurons were found extremely larval at the resting state but explosively active during the seizure state. In the PCX and Hippocampus remarkably abnormal activities of GABA system and DNA and/or other substances were found at the molecular level. A new therapeutic direction of seizures and abnormal behaviors of epilepsy were suggested by transplantation of cultured neural stem cells. The hereditary mode of the EL mouse is the autosomal dominant, but its seizure itself may provoke an epigenetic phenomenon.

Purpose: The self-completed NEO-PI-R questionnaire evaluates character with a five-domain facets (Neuroticism, Extraversion, Openness, Agreeableness, Conscientiousness) and 30 sub-facets. We assessed subjective personality traits of patients with epilepsy (PWE) using NEO-PI-R and examined relations between NEO-PI-R scores and BDI-II score.

Methods: The Japanese NEO-PI-R was completed by 143 outpatients. Scales were scored with the T-score (≥55, "high"; <45, "low") normalized by gender and age group in a general population. The last 40 patients simultaneously completed the BDI-II.

Results: We found the "high" trait in domain facet N and sub-facets N1 (Anxiety), N3 (Depression), and N6 (Vulnerability) and the "low" trait in C1 (Competence). BDI-II score correlated highly positively with N, N1, N2 (Angry Hostility), N3, N4 (Self-Consciousness), N5 (Impulsiveness), N6, and O1 (Fantasy) and highly negatively with A and C1 (¦r¦>0.4, p<0.01). There were no significant differences in the five domain facets between patients with temporal lobe epilepsy (TLE) and extra TLE nor between those with left and right TLE.

Conclusion: Neither a consistent personality trait in PWE nor a clear difference in personality traits by focus localization or lateralization could be determined. Comorbid depression is highly prevalent in PWE and may affect assessment of their personality traits.

To clarify the current situation of regional epilepsy care and its dissociation from the regional cooperative model for comprehensive epilepsy care, we retrospectively investigated the consecutive sample survey of epilepsy clinic of 1 certified epileptologist at Kyoto university hospital by means of inter-hospital network system from April 2013 to March 2016. Total 313 patients were introduced and the diagnoses were as follows; partial epilepsy: 115, generalized epilepsy: 67, isolated seizure: 53, psychogenic non epileptic seizure: 17, syncope: 34 etc. Referring purposes were different between epileptologists and non-epileptologists. The main referring purposes from epileptologists were related to surgical treatment, whereas those from non-epileptologists were related to diagnosis, medication and driving license and so on. As for transition, there were few consultations from pediatric neurologists. Based on these findings, it is necessary to prepare document and medical guidance in daily life for patients with epilepsy according to the referring purposes. Additionally, it is also important to provide information and educational opportunity of comprehensive epilepsy care for non-epileptologists in collaboration with other departments and conferences in order to promote transition in the future.

Perampanel (PER) is a novel class of anti-epileptic drug and a noncompetitive inhibitor of the AMPA receptor. Some reports have described the efficacy and side-effects of PER in Japan. We prescribed PER to 33 refractory epilepsy patients, including some with intellectual disabilities and/or an age under 12 years. A "Good response" was defined as more than 50% seizure reduction, and we investigated the responder rates for focal seizure (Fs) and generalized tonic clonic seizure (GTCS). The effective rate for Fs and GTCS were both 50%, and the overall seizure rate was 52%. The efficacy in patients <12 years of age was similar to those ≥12 years of age. Although there were no significant differences in the responder rates among the concomitant antiepileptic drugs (AEDs), two patients who received KBr combination treatment showed a good response. The responder rates with CYP3A4-inducing AEDs such as CBZ and PHT tended to be low (30% and 18%, respectively). Adverse events occurred in 55% of patients, including emotional and behavioral abnormalities in 30%, somnolence in 18%, and dizziness in 15%. We should therefore closely monitor young patients and those with intellectual disabilities, as emotional and behavioral abnormalities tend to occur with the administration of PER.

MOSES (Modular service package epilepsy) is a psychosocial-educational program for patients with epilepsy. It is designed to improve the patients' knowledge about epilepsy, coping skills and motivation to manage their disease. Literatures showed MOSES was effective for patients with epilepsy not only to gain knowledge about epilepsy and coping skills, but also to improve seizure frequency and the side effects of medications. We investigated quality of life (QOL), knowledge about epilepsy, mood and subjective evaluations of daily life in 55 Japanese patients with epilepsy before and after performing MOSES program. In this investigation, patients with epilepsy showed the improvement of scores in knowledge scale of epilepsy, overall QOL in Quality of life in epilepsy-31-P (QOLIE-31-P) and adaptation to epilepsy in Performances, sociodemographic aspects, subjective evaluation (PESOS) after MOSES program. MOSES is effective for patients with epilepsy not only to gain knowledge of epilepsy, but also to go through psychological changes positively.

We report a 2-year-old female patient with anoxic-epileptic seizures. Since 6 months of ages, she has had generalized tonic convulsion after crying too hard and holding her breath. It sometimes lasted for one hour. She was brought to a nearby clinic, but she had been followed up with no medication under the diagnosis of breath-holding spells. At 2 years and one month, she was referred to our hospital with increased ictal events in which crying, breath-holding, atonia, asymmetrical tonic convulsion and generalized clonic convulsion occurred in a sequence. Ictal EEG showed a low-voltage pattern followed by rhythmical activity with the right frontal predominance and subsequent intermittent polyspike-and-wave. Treatment with Kanbaku-Taisou-Tou (an herbal medicine) and iron had no effect, but carbamazepine decreased the frequency of occurrence and continuance length of convulsive phases. Combination therapy of carbamazepine and levetiracetam completely suppressed the emergence of convulsive phases. In case of breath-holding spells with long convulsive phase, ictal EEG should be considered. If anoxic-epileptic seizures are demonstrated, anti-epileptic agent(s) should be administrated.

Previous studies have estimated that one medial temporal lobe epilepsy (MTLE) patient per 100,000 population is a good candidate for surgery, but these patients are easily overlooked if the attendant physician and/or the patient's guardian do not recognize the specific seizure symptoms.

Over a period of 9 years in the western Iburi district of Hokkaido prefecture (population 205,000), we have encountered three surgical cases of MTLE patients who have become seizure free. All three patients had been diagnosed initially as having drug-resistant unclassifiable symptomatic localization-related epilepsy.

All three patients were eventually diagnosed as having MTLE because of an awareness of the characteristic clinical symptoms of MTLE and results of neuroimaging and electrophysiological studies; however, it took 5-11 years (mean 7.3 years) from the onset of the epilepsy attacks until a diagnosis of MTLE was made and 6-11 years (mean 8.3 years) until the surgeries were performed.

Because the initial seizures in MTLE generally occur in patients around 5-10 years of age, pediatricians are the attendant physicians for the initial diagnosis and treatment. Thus, it is important that general pediatricians are aware of the clinical features of MTLE to ensure a favorable prognosis for their patients.