Desirable system of epilepsy research and epilepsy medical care in Japan are discussed with reference to past changes in the field of epilepsy.
The concept of epilepsy has changed from primarily that of a psychiatric illness to a brain disease following the advancement of epilepsy research and treatment. Researchers concerned with epilepsy have expanded from originally just Psychiatrists to now include Pediatricians, Neurosurgeons, and basic scientists.
Based on these changes surrounding the situation of epilepsy, the following has been proposed for the future development of epilepsy science and medical care in Japan:
1. An epilepsy clinic should be created as a special department which is separate from Psychiatry, Pediatrics, and other existing Departments within the hospital and the medical school.
2. New Board certification systems should be established under the Japan Epilepsy Society for making "Primary Epilepsy Specialist" (certified epileptologist, in Fig. 4) and "Highly Qualified Epilepsy Specialist" (Epilepsy Specialist).
3. Co-operation with the Japan Epilepsy Society and the Japanese Epilepsy Association should be promoted for facilitation of epilepsy medical care.

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Purpose: We conducted a multi-center study to investigate the correlation between psychotic symptoms and handedness in patients with focal epilepsy.
Methods: Three groups of patients (32 focal epilepsy patients without psychosis, 32 focal epilepsy patients with psychosis, and 25 patients with schizophrenia) were enrolled. General characteristics, epilepsy-related factors, and psychosis-related factors were evaluated. We evaluated handedness with the Annette Hand Preference Questionnaire and intelligence with the Wechsler Adult Intelligence Scale-Revised. The distribution of handedness and clinical characteristics of each group were analyzed.
Results: The distribution of handedness did not differ among the three groups. There was no significant correlation between handedness and the lateralization of EEG abnormalities. Several left-handed and ambidextrous patients showed MRI abnormalities in the left hemisphere or the bilateral hemispheres. Although the group of epilepsy patients with psychosis showed lower intellectual functioning than the other groups, intellectual functioning was not significantly associated with the distribution of handedness.
Conclusion: The epilepsy patients with psychosis showed no particular pattern of handedness compared with the epilepsy patients without psychosis or the patients with schizophrenia.

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To evaluate the efficacy and safety of gabapentin in the treatment of childhood intractable epilepsy, we performed a prospective open label study on 45 children and adolescents (age 0.9-17.3 years, average±SD 8.9±4.4 years). They had intractable seizures ≥ once a month that persisted despite appropriate usage of ≥ two antiepileptic agents. The initial gabapentin dose was 16.1±6.9 mg/kg and the dosages were titrated to 25.1±10.3mg/kg (average±SD). The responder rate (the rate of patients with ≥50% reduction in seizure frequency) was 15/45 (33.3%) in total, and showed a higher ratio in those with partial epilepsy (13/32) than in those with generalized epilepsy (2/13), but there was no significant difference. Gabapentin was more effective in controlling complex partial seizures than simple partial seizures: the responder rates were 66.7% and 25.0%, respectively (p<0.05). Ten patients had adverse experiences: somnolence occurred in 6 patients, drooling with somnolence, loss of activity, increase of appetite and rash in one, respectively. These adverse effects were mild and disappeared without any other treatment except discontinuation. Gabapentin seems to be safe for children, and may be useful as adjunctive medication for childhood partial epilepsy, especially complex partial seizures.

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Epileptogenesis is induced by repeated seizures attributed to several kinds of causes following the initial exposure to exogenesis factors (i.e. brain trauma, etc.) and/or endogeneous factors (such as gene abnormalities). Under the establishment of epileptogenesis, epileptic seizures are induced by hypersynchronization of neuronal groups due to abnormal activities of voltage-dependent and/or receptor-activated ion channels. In the above-mentioned epileptogenesis, partial mutation of the AMPA/KA receptor-channel complex induces both Ca²⁺and Na⁺influxes, thereby producing epileptic seizures and/or neuronal death.
Currently available antiepileptic drugs act as inhibitors of the Na⁺or T/L/PQ type Ca²⁺channels, modulators of K⁺channels or GABA_A receptor agonists/enhancers. Recently, antagonists of the AMPA/KA receptor, such as topiramate and talampanel, have been found to display a novel antiepileptic mechanism. Unlike other conventional antiepileptics, topiramate elicits a dual functional characteristic that inhibits neuronal death while exhibiting an antiepileptic (anti-seizure) effect.

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A 9-year-old girl who suffered from intractable daily seizures from 4 years of age was studied. She had negative myoclonus (NM) in the left arm during sustained extension of the arms. Her MRI showed shrinkage in the medial surface of the right frontal region. During the NM, EEG showed spike and wave around the right central region and the surface EMG demonstrated silent periods (SP) in the left triceps brachii muscle. Jerk-locked back averaging, time-locked to the onset of the SP, showed a negative spike around the right central region preceding SP by 38msec. Although SPECT images obtained 3 hours after administration of ¹²³I-Iomazenil showed abnormal hypo-accumulation in the right basal and mesial surfaces of the frontal lobe on the visual inspection, the statistical assessment of the images, using three-dimensional stereotactic surface projection (3D-SSP), indicated significant decreased particular in the right pre-supplementary motor area (pre-SMA). Due to these clinical and laboratory findings, it was assumed that the origin of NM in the current patient might be in pre-SMA.

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Epilepsy in childhood sometimes involves behavioral problems, which are refractory to medical treatment. We describe a 7-year-old boy with frontal lobe epilepsy and severe behavioral problem, which dramatically improved following epilepsy surgeries. He had a severe attention deficit described as "acting vigorously as if driven by a motor", as well as severe mental retardation since 3 years old. He underwent anterior 2/3 callosotomy followed by two-time resections of left frontal lobe, and achieved not only complete seizure freedom but dramatic behavioral improvement. SNAP (Swanson, Norlan and Pelham) rating scale adjusting for him showed marked improvement in the component of his hyperactivity and impulsiveness.
This case highlighted strong relation between frontal lobe epilepsy and behavioral impairment in childhood from the surgical perspective. The improvement of the behavior as well as epileptic seizures following the left frontal lobe resection in the present patient indicated that epileptic focus in the left frontal lobe was also responsible for the behavioral disorder.

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Cerebral blood flow SPECT (single photon emission computed tomography) findings of cortical dysplasia in adults often show hypoperfusion. On the other hand, there have only been occasional reports of hyperperfusion. A few studies have reported cerebral blood flow at the site of cortical dysplasia in children. In this study, we performed SPECT scanning of the brain to assess cerebral blood flow, and EEG (electroencephalography) at the site of cortical dysplasia in 4 children, to assess changes over time. Although hyperperfusion was observed at the site of cortical dysplasia during early infancy in all cases, it changed to hypoperfusion over time in most cases or to isoperfusion in some cases. These observations suggested that the assessment needed extra attention taking into account age in months. In addition, cerebral hyperperfusion during early infancy may indicate cortical dysplasia at the hyperperfusion site. While cortical dysplasia cannot be detected by MRI in some cases because of insufficient brain myelinization during early infancy, this study suggested that the SPECT study of cerebral blood flow could contribute to an early diagnosis.

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Erratum: an addition to English Abstract
Note: erroneous description in the abstract
For two years we investigated the applicability of and problematic issues with the Diagnostic Scheme for Patients with Epliptic Seizures and Epilepsy proposed by the ILAE in 2001, by studying the diagnoses and classifications of patients with epilepsy who first visited our pediatric department. The subjects were 102 cases in 2005 and 136 in 2006 diagnosed based on the 5 axes. While seizure types (2 axes) could be classified using conventional methods, the diagnosis rate of disorder types (3 axes) was 52%, thus failing to reach a satisfactory diagnosis rate. Although the 5 axes are useful for understanding the details of individual cases, this scheme is an inadequate alternative to conventional methods of classification for disorder types and many issues remain to be resolved regarding its clinical use as a common language for diagnosing epilepsy cases.

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