Epidemiological surveys on epilepsy have been carried out repeatedly in Japan. However, at present, a population-based survey according to the International Classification for Epileptic Syndromes is considered to be especially important. We therefore carried out a population-based survey on children with epilepsy under thirteen years of age, who resided in Okayama Prefecture on December 31, 1999 as the prevalence day.
The population of children in Okayama Prefecture under thirteen years of age on the prevalence day was 250, 997. The lists of children with epilepsy were collected from the medical records of 45 hospitals, 36 clinics and 3 institutions in and around Okayama Prefecture.
1) 2, 222 cases with active epilepsy were identified. The prevalence rate was 8.9 per 1, 000.
2) This prevalence rate was almost equal to the previous result (8.2 per 1, 000) given by another survey undertaken in 1975 for children under ten years of age in Okayama Prefecture.
3) If the cases with single seizure and/or the cases with seizures induced by fever are excluded, the prevalence rate decreased to 5.5 per 1, 000.
4) 2, 026 (91.2%) of the 2, 222 cases were classified into three major categories of the International Classification of Epileptic Syndromes. They consisted of 1, 557 cases (76.8%) with localization-related epilepsy, 449 cases (22.2%) with generalized epilepsy and 20 cases (1.0%) with undetermined epilepsy.
5) 303 (15.0%) of the 2, 026 cases were classified into the epileptic syndromes. However, the majority of other cases consisted of nonspecific types of epilepsy, and could not be classified in detail.
6) The guideline for informed consent in epidemiological study published in 2000 by the Committee of the Japanese Ministry of Public Welfare will be useful for neuroepidemiological studies in the future.

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For children with developmental disabilities in Japan, various social policies including education, medical care, pension and welfare have rapidly developed and prevailed during the last 50 years. The concept of living together with the disabled has been widely accepted. A high standard of social welfare has been established by virtue of high respect for human rights, progress in medicine and economic growth. Historically, the International Year for the Disabled played an important role in meeting the expectation of the disabled and their families. Amendment of the legal system has realized normalization and social independence. Based on this historical background, there are in the 21st century new trends of development in the education, welfare and social life of the disabled, aiming at a better society for all the people.

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Three types of researches have been carried out on brain-mind relationships: 1. researches on anatomical correlates of special talents (for example, perfect pitch) or deficits (for example, dyslexia), 2. researches to examine the relationship between a given cognitive syndrome and the site of brain damage, 3. researches to localize human cognitive function in the brain in vivo using functional magnetic resonance imaging (fMRI) and positron emission tomography (PET). fMRI is a particularly important because it is noninvasive. A tutorial covering basic aspects of this methodology is presented, along with a survey of recent fMRI data related to clinical application. Future investigations of the three types enumerated above are expected to further clarify brain-mind relationship

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Survival rates of extremely and very low birth weight infants have been improved dramatically. However, some of the survivors suffer from neurological sequelae. A recent report showed that about 25% of the children with birth weight of less than 1, 000 g had some handicaps, including cerebral palsy, mental retardation, and visual disturbance. Therefore, it is very important for pediatric neurologists to assess the neurological symptoms properly in the neonatal period. This symposium was organized to present recent progress in the methods for neurological assessment and to discuss how we should support the development of high risk infants.

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Human fetuses and young infants have a repertoire of distinct patterns of spontaneous movements. A set of these movement patterns are known as general movements (GMs), which were defined by Prechtl as gross movements involving the whole body, and lasting from a few seconds to several minutes, or longer. GMs are characterized by the variable sequence of arm, leg, neck and trunk movements which begin gradually, wax and wane in intensity, force and speed, and end gradually. Extension and flexion movements of the arms and legs are mostly complex and variable because of superimposed rotations and frequent, slight changes in direction, which make the movements fluent and elegant. Initially GMs are complex, and then differentiate into single movements. GMs show no change during the fetal period, but change in early infancy into writhing, fidgety, oscillating, saccadic and swips & swaps. Hadders- Algra indicated that on EMG, burst duration of phasic activity shortens, burst amplitude attenuates and tonic background decreases with development. In contrast to normal GMs that are smaller and monotonous in trajectory, abnormal GMs include poor repertoire of GMs, cramped-synchronized GMs, chaotic GMs, absent fidgety, and abnormal fidgety, that are periodic and monotonous, showing no developmental change. For example, a normal twin showed elliptic or round trajectory of GMs that became smaller with age, whereas the other with periventricular leukomalacia and cerebral palsy had periodic and rectilinear trajectory showing no developmental change. GMs, especially fidgety, correlate with neurological prognosis of infants. Einspieler reported that GMs' sensitivity and specificity for neurological prognosis of high risk infants are 96% and 95%, respectively. Clinical significance of GMs (1) includes non-invasive, secure and easy observation, (2) high coincidence between trained observers, (3) high reliability 78-98%, mean 90% and, (4) correlation of abnomlal GMs with the presence and degree of brain damage.

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The infants' brain during the prenatal, perinatal and neonatal periods is susceptible to injury. Many problems in the perinatal period often result in bleeding, ischemia and other pathological changes in the infant brain. Which can subsequently cause cerebral palsy or developmental disorders. Unless they are discovered early and measures are taken, permanent brain damage may remain. Although neurological examinations at this stage is very difficult, it is very important to be familiar with neurological signs and assessment of extremely and very low birth weight infants and to discover early any abnormal findings of diseases such as neonatal asphyxia, intraventricular haemorrhage, periventricular leukomalacia, neonatal seizures and hydrocephalus.

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Flash visual evoked potential (Flash-VEP) is easily recorded in preterm infants. However, its clinical application has not been established due to its great variability in response. Our longitudinal studies on the two components of the N1 wave facilitated peak definition and established normal ranges that are clinically valuable. The N1a (early component of the N1) peak latency decreases at about 4.6 msec/week between 30 and 40 weeks postmenstrual age.
A flash-VEP study in the preterm period enables us to observe the neuronal development in the human visual system that normally proceeds in utero. Flash-VEP analyses on preterm infants demonstrated that the decrease in the N1a peak latency reflects the progress of myelination in the visual pathway according to the developmental program irrespective of preterm birth. The developmental changes of the N1 wave configuration reflect the maturation of the neuronal networks in the visual cortex, which is accelerated by extrauterine visual experience.
Using improved methodology and peak denomination that we proposed, flash-VEP can be applied to preterm infants safely, and should provide us with neuro-developmental information of the human cerebrum.

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Doppler sonography is convenient and useful for evaluating intracranial lesions and hemodynamics, especially in the neonate. Recently we used power flow Doppler imaging (PF) to show vessels with a low flow velocity and a small caliber. The pulsed Doppler system (PD) in combination with PF can selectively visualize small vessels and slow blood flow, such as the steady flow conditions of the lenticulostriate artery (LSA). As PF can reveal the vessel crossing at a right angle to the ultrasonic beam, the bilateral middle cerebral arteries can be observed through the anterior fontanel under the same conditions. We can also evaluate the laterality of intracranial hemodynamics using PF. Four-dimensional PF images, which are reconstructed from the several three-dimensional reconstruction images during one cardiac beat, can show the pulsation of intracranial arteries. This technique may provide a new quantitative and qualitative method for intracranial circulation.

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We reviewed the concept and history of early intervention (EI). We summarized the results of three randomized controlled trials for high-risk young children: Abecedarian, CARE, and the Infant Health Developmental Program in the USA. All of those interventions showed positive effects on child IQ during the first 3 years of life. A randomized EI trial for very-low-birth-weight (VLBW) infants at 8 institutions in Japan also showed enhancement of some aspects of development, including behavioral problems, circadian rhythm, and speech. The present problems of EI for VLBW infants in Japan include lack of financial support by the government, lack of evidence to determine the onset, intervals and duration of the service, and difficulties in objective evaluation of its short/long term efficacy. To enhance the maximum development of VLBW infants, pediatric neurologists should advocate the importance of EI to the government.

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[in Japanese]

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