To survey the current status of clinics with certified epilepsy specialists in Japan, we administered a questionnaire to epilepsy specialists certified by the Japan Epilepsy Society who worked in clinics. Of the 82 eligible epilepsy specialists, 71% of them responded the questionnaire. After excluding 26 respondents who indicated that their clinic was not an epilepsy clinic, 32 participants remained for further analysis. All participants were aged over 40 years, and 81% had experience in high-level epilepsy training facilities. In total, 84% reported their clinics had electroencephalographs, and 53% indicated their clinics were allowed to calculate higher electroencephalogram reading fees than usual. In addition, 53% of them provided care for over 400 patients with epilepsy, 41% were involved ongoing research. 94% had experience referring multiple patients to epilepsy facilities with high-level medical functions. The main reasons for choosing referral facilities were "providing medical care suitable for the purpose" and "connection of people such as doctors." This report clarified that the medical care for patients with epilepsy provided by epilepsy specialists at clinics in Japan is highly specialized.

Purpose; We have to understand some differences of current clinical circumstances and social welfare works between metropolitan and local areas in Japan, when we discuss to establish a medical cooperation system for epilepsy. The Japan epilepsy society (JES) surveyed current situations and problems on cooperative relationship using a questionnaire from medical doctors who have treated patients with epilepsy at metropolitan areas. The purpose of this work is to make plan for practical clinical network on epilepsy. Methods; Target areas on this survey were Tokyo 23 district, Kanagawa, Aichi, Osaka, and Fukuoka prefectures. The JES obtained some answers from chiefs doctors whose facility had at least a full-time doctor in neurology, neurosurgery, pediatrics, psychiatry, and emergency departments, and was possible to examine MRI and EEG. The JES also asked some questions to boarded epileptologists working at the treatment and welfare center, and private clinic on epilepsy. Results; We obtained 214 answers from 1,312 doctors in five areas, and response rate was 16.3%. A characteristic of respondents in this study was accounting for 47.9% of pediatrician. Possible examinations at responders facilities were standard EEG (97.2%), therapeutic drug monitoring (96.7%), MRI (92.4%), neuropsychological test (69.7%), and video EEG monitoring (24.2%). All aged patients were acceptable by 66.7% of hospitals. Acceptance ratio of candidates for epilepsy surgery and of patients with psychiatric symptoms were 11.7% and 17.9%, respectively. The most frequent reason of unacceptance was having severe psychiatric symptom by 79.1%. Making circumstances that physicians easily ask some questions to epileptologists (59.3%) and offering some educational programs on epilepsy (57.9%) were important to make cooperation medical system for epilepsy. Discussion; The number of epilepsy center in metropolitan area is still few for population. To increase number of epilepsy center and establish a medical cooperation system for epilepsy based on educational programs are required. Make some regional medical cooperation systems within the metropolitan areas is needed for patients and family with epilepsy.

The nation-wide status of epilepsy care in the rural areas of Japan has not been studied and is not known. To obtain the basic information, we conducted a survey targeting the alumni and alumnae of Jichi Medical University, who are obliged to serve as primary care physicians for rural areas for a predetermined period. The questionnaire items of Web-based surveillance included diagnosis and treatment, social welfare, driver's license and support from local epilepsy centers, covering many aspects of clinical epilepsy care. While clinical guidelines are widely used for standardized practice among rural physicians, level of examinations and available medications varied among districts. Their awareness of local epilepsy centers is still limited. The support system by local epilepsy specialists is to be improved for epilepsy care in the rural districts.

There are few epilepsy specialists in Akita, and non-specialists manage and treat most of the epilepsy patients. This study surveyed the actual state of the management and treatment for epilepsy in Akita. We conducted a questionnaire survey of 97 medical doctors in four different departments at 35 institutions. The questionnaire contained 24 items regarding the number of patients, type of the tests and treatments conducted, and issues regarding the management and treatment for epilepsy. Completed surveys were obtained from 47 medical doctors. Symptomatic localization-related epilepsy was common. Cerebrovascular disease was the most frequent cause. Evaluation of electroencephalography and magnetic resonance imaging performed in more than 90% of patients contributed to diagnosis of epilepsy. Seizures were well controlled by antiepileptic drugs in 90% of patients. Levetiracetam was selected most often for localization-related epilepsy, followed by carbamazepine and lamotrigine. Valproic acid was selected most often for generalized epilepsy, followed by levetiracetam and lamotrigine. Issues in the management and treatment for epilepsy included difficulties in the interpretation of electroencephalography and the lack of local epilepsy specialists.

Among subjects with transient loss of consciousness (TLOC), 90% of cases consist of syncope, epilepsy, or psychogenic non-epileptic seizure (PNES). However it is not easy to distinguish these causes of TLOC. We investigated clinical information from patients with TLOC as assessed by both epileptologists and cardiologists. Subjects included 65 patients older than 15 years. Syncope occurred in 31 cases, epilepsy occurred in 13 cases, and PNES occurred in 4 cases; 17 cases were of unknown etiology. Many cases TLOC experienced convulsions. We divided cases into a minor seizure group and a generalized seizure group and examined both groups. Syncope was more common in the minor seizure group, and epilepsy was more common in the generalized seizure group, with significant differences between groups. The present study suggests that a differential diagnosis between syncope and epilepsy may be possible based on whether convulsions do or do not occur with TLOC.

Epilepsy is considered relevant to various medical dimensions, including primary and secondary care for diagnosis and drug treatment, and higher-order medical facilities, i.e., epilepsy centers, for surgical treatment. Cross-organizational and medical cooperation are essential to support patients with different degrees of epilepsy. The Ministry of Health, Labour and Welfare launched a project of regional medical cooperation system and selected 8 local prefectural governments for the practice in 2015. Our medical center has been designated by the Hiroshima prefectural government to implement this project. In addition to ongoing educational and disease awareness activities, we organized a council comprising multiple professionals for community cooperation. We also established a sub-working group with committee members from 9 secondary care hospitals in the different secondary medical areas within Hiroshima prefecture. As a result of these activities, we constructed our own Hiroshima prefecture model of the medical care cooperation system, which has been already implemented. Herein we describe our efforts to increase epilepsy awareness, create a regional workshop for healthcare providers, and establish an epilepsy medical network for community cooperation.

Perampanel (PER) has a different mechanism of action compared with previous anti-epileptic drugs (AED) and is expected to be effective to treat refractory epilepsy patients. Usually, efficacy assessments include the percentage of patients with a ≥50% seizure reduction, while seizure resolution has a marked impact on patients. Here, four refractory epilepsy patients aged 19 to 41 years who achieved seizure freedom for longer than 3 months up to 1 year and 8 months with adjunctive PER treatment are described. In these patients, monthly partial-onset with secondarily generalizized seizures had continued for 11-36 years despite treatment with 4-5 AEDs. Recently, however, main seizure types of these patients were secondarily generalized seizures (sGTCS). Four patients had moderate or severe intellectual disabilities and their EEGs showed paroxysmal discharges in the frontal or temporal regions. The initial dose of PER was once-daily 1 mg, and 1-mg increments per 2 weeks or 2-3 months up to 4-6 mg were made until seizure freedom was achieved. Then, these doses were maintained until seizure relapse. Once-daily doses at the end of follow-up were 6-12 mg. These slower titration schedules were associated with less adverse side effects. As AMPA receptors are critical to epileptic synchronization and the generation and spread of epileptic discharges in human epilepsy, PER could reduce the propagation of seizures, thereby explaining its favourable efficacy outcomes for sGTCS. PER could achieve seizure resolution in some patients with intractable partial-onset seizures, especially with sGTCS.

Lacosamide (LCM) is a novel class of anti-epileptic drug that selectively promotes slow inactivation of the sodium channel. Case studies, including pediatric cases, have not been described in Japan. We examined the efficacy and side effects of LCM in Japanese epilepsy patients, including those under 16 years old. Responders were defined when seizures were reduced by more than 50%. The responder rate for overall seizure was 40%. There were no significant differences in efficacy by seizure types or age (<16 or ≥16 years old). Among epilepsy types, the efficacy in patients with focal epilepsy (17/39) was higher than in generalized epilepsy (0/8) (p=0.0045). Patients with ≤2 AEDs had significantly higher efficacy (16/27) than those with ≥3 AEDs (8/33) (p=0.0055). In patients with concomitant AEDs, LTG (2/16) and PER (0/10) were significantly less effective than other drugs (LTG: p=0.0055, PER: p=0.0007). Although adverse effects occurred in 23% of patients, including somnolence 20%, dizziness 5%, agitation 2%, there was no significant difference by age (<16 and ≥16 years). Our study suggests that LCM is effective focal-epilepsy patients especially those with ≤2 concomitant AEDs. Studies of more cases with long-term observation are needed to establish the role of LCM in childhood epilepsy treatment.

Given that a number of adverse side effects have been associated with the use of Zonisamide (ZNS), we aimed to investigate risk factors associated with the incidence of renal lithiasis in patients with epilepsy taking ZNS. Sixty patients diagnosed with epilepsy taking ZNS for at least 1 year were included. Risk factors for renal lithiasis were noted, including 1) age; 2) presence of an intellectual or motor disability; 3) treatment duration and dose and plasma levels of ZNS; and 4) use of polytherapy. Of 60 patients taking ZNS, eight patients (13.3%) were diagnosed with renal lithiasis, as determined by abdominal ultrasonography and computed tomography.

A Mann-Whitney U test revealed that patients diagnosed with renal lithiasis had a significantly longer duration of ZNS treatment relatively to patients who did not develop renal lithiasis (p=0.019).

Subjects were divided into two subgroups by dosing period. One group comprised subjects with a dosing period less than X years and the other group comprised subjects with a dosing period more than X years. Statistical analysis by Fisher's exact test showed a high incidence of renal lithiasis in those with a dosing period more X years than in those with a dosing period less than X years in the X=6 years (p=0.019), 10, and 11 years (p=0.024, p=0.018) groups. Additionally odds ratio was highest in the X=10 and 11 years groups.

Renal lithiasis-positive group showed a lower blood concentration of ZNS (10.6 μg/ml) than the renal lithiasis-negative group (17.4 μg/ml). However, the reason for this finding could not be ascertained. In addition, other risk factors of renal lithiasis were not significant.

Based on these findings, the author recommends that the dosing period for treatment with ZNS should be restricted to less than 10 years, and for patients who have received over 10 years of with ZNS, treatment with other anti-epileptic drugs should be considered.