Lidocaine was administered intravenously as a substitute for diazepam, to 12 patients with status epilepticus or clustering seizures aged 26 days to 11 years. The medication was very effective in 3 cases with acute convulsions, which disappeared immediately after infusion of lidocaine without relapse. The medication was effective only temporarily in 4 patients; they experienced relapsing seizures during drip infusion of lidocaine intravenously for maintenance. All the relapsing seizures were secondarily generalized ones with diffuse ictal discharges. In 2 cases of localization-related epilepsy, complex partial seizures evolved to secondarily generalized seizures immediately after administration of lidocaine. It must be noticed that in a relatively large number of cases lidocaine is ineffective or even harmful.

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With the recent advances in medical techniques, many patients with spina bifida have survived to adulthood. We reported here on the present social life and complications in 43 spina bifida cases followed up in the Tochigi Rehabilitation Center.
Fourteen of 23 patients (61 %) above eighteen years of age were able to achieve an independent social life. Most of these patients could walk with or without crutches and did not have mental retardation. Nine of these fourteen patients (64%) were capable of natural or high pressure urination and only three patients (21%) required the clean intermittent catheterization technique.
In school-age patients, all the nine junior and senior high school students and three of the nine primary school students were attending a school for the handicapped.
Their independent social life was related not only to the degree of severity of their disability, but also to the aid afforded by the society.

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In Okinawa, there were ten cases (7 male and 3 female) of subacute sclerosing panencephalitis (SSPE) from 1970 to 1996. All cases had a history of measles and none had received measles vaccination. The incidence of SSPE in Okinawa was 0.31 per million in 1970-1996. Being higher than other districts in Japan. Two cases contracted measles in 1973 and other three in 1990, showing a tendency to cluster.

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A population based statistical analysis was performed of the incidence of developmental disabilities in Higashi-Osaka, a city in Osaka Prefecture with a population of about 500, 000. The number of live births during 1988-1992 was 24, 980, of whom those with cerebral palsy (CP), severe motor and intellectual disability syndrome (SMIDS), infantile autism, mental retardation (MR) and Down syndrome numbered 30, 21, 26, 268 and 25, respectively. And the respective incidence rates per 1, 000 were 1.20, 0.84, 1.04, 10.7 and 1.0.
For CP, the percentage of premature children was 63%, higher than in previous reports. Extremely lowbirth- weight premature children (<999g birth weight) were especially notable at 20% of the CP total. For SMIDS, it is more important to understand the significance of medical care to support sufferers' social lives. High functional autistic children could not be evaluated at our center, though autism accounted for 8.6% of mental retardation. The medical functions of community institutions enable them to perform continuous, population based study of the incidence and situation of developmental disabilities.

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We investigated the status of the school life of 72 children with acquired brain damage attending to different schools: special schools (30cases), special classes in an ordinary school (9cases), and ordinary schools (33cases).
In the special school group, the main disorder was the sequelae of encephalitis/encephalopathy; there were 26 cases unable to walk, 29 with severe mental retardation, and 25 with epilepsy.
In the ordinary school group, main problems were the sequelae of a cerebrovascular disorder or a traumatic brain injury. Impairment was mild in this group ; only 2 cases were unable to walk, and only 4 were mentally retarded. However, many patients had learning difficulty and costed the families much work to achieve school lives.
In all groups, the use of welfare equipments improved the activity of daily living.
We emphasize that welfare equipments and community suppot systems should be utilized for these children

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We report five cases of multiple sclerosis (MS) and three cases of allied demyelinative diseases starting during childhood.
Three of the MS patients presented with atypical initial symptoms, such as acute encephalitis or myelitis, making an early clinical diagnosis difficult.
Ophthalmologic symptoms were noted in four of MS children, and in two with allied demyelinative diseases. Therefore, if a child shows ophthalmologic symptoms (i. e. optic neuritis, ophthalmoplegia), brain magnetic resonance imaging (MRI) should be conducted for the differential diagnosis of MS and other demyelinative diseases.
Cerebrospinal fluid analysis is not useful for the initial diagnosis of MS, because pleocytosis and increase of oligoclonal IgG band in cerebrospinal fluid are seen in both MS and other demyelinative disorders. However, neuron specific enolase (NSE) is slightly higher in the latter than in the former.
T₂-weighted MRI of multiple sclerosis showed multiple high intensity areas in the white matter of the cerebrum and cerebellum, capsula interna, and crus cerebri etc. Most of these lesions were clinically silent, being characteristic of MS. In two MS cases, however, initial MRI revealed no abnormal findings. Thus, the diagnosis of MS can not be made by initial MRI only.

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Partial motor seizures occur, followed after a variable interval by well-localized myoclonic jerks, in the patients with Kojewnikow syndrome, or epilepsia partialis continua. However, the epileptogenic focus remains to be established. We studied the epileptogenic focus of a 17-year-old boy with intractable Kojewnikow syndrome. Interictal EEG, ictal EEG, jerk-locked averaging, and ictal SPECT revealed a left central focus. A giant SEP indicated increased exitability of the primary sensory area. Magnetoencephalogram (MEG) and electrocorticogram identified the epileptogenic focus in the pre- and post- central cortex, indicating involvement of both the primary motor and sensory areas. Surgical treatment was successful. Thus, MEG proved to be the most accurate tool in the presurgical evaluation.

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We report here a 9-year-old girl with ophthalmoplegic migraine. At the age of 2 years and 6 months she first developed left ptosis and ophthalmoparesis that resolved gradually within 2 weeks. She experienced similar episodes repeatedly. After 5 years of age, left periorbital pulsatile pain preceded ptosis and ophthalmoparesis, and after 7 years, she showed permanent left third nerve paresis even between the attacks. On cranial MRI the left oculomotor nerve showed swelling and contrast enhancement, the latter being more prominent in the ictal than interictal images.
Ophthalmoplegic migraine should be considered in the differential diagnosis of opthalmoplegia in children even in the absense of headache. The diagnosis is strongly suspected when MRI demonstrates swelling and enhancement of the oculomotor nerve.

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We reported identical twin cases having convulsion associated with mild gastroenteritis almost simultaneously. The patients were 14- month-old boys. They suffered from vomiting from one day before the onset of convulsion. Convulsions occurred firstly in the first twin, and then in the second one immediately after the convulsion of the first one. The twins experienced 6 and 4 convulsions respectively, although they were treated with diazepam and phenobarbital. An ictal EEG of the second twin revealed a focal paroxysmal discharge in the right occipital area spreading rapidly to all areas. During the follow-up period, no recurrence of seizures was observed and psychomotor development was normal in both of them.

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We report a pair of 1-year-5-month-old female monozygotic twins with generalized loss of pain sensation, but without impairment of other sensory modalities and the diaphoretic function. Routine electrophysiological investigations revealed no abnormalities. Morphometric analysis of biopsied sural nerve showed that the number of small myelinated fibers was reduced and that of unmyelinated fibers was normal or mildly reduced. On the basis of these findings, we suspected a diagnosis of a rare disorder, HSAN type V, which has not previously been reported in Japan.

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A boy with ocular type myasthenia gravis was reported. The therapeutic effect of pyridostigmine bromide and corticosteroid was insufficient. However, clinical symptoms disappered rapidly after an influenza A virus infection. On a peripheral lymphocytes subsets analysis, the CD 3, CD 4 and CD 4/CD 45 RA positive lymphocytes increased with the therapy and decreased after the infection. By contrast, CD 19 positive lymphocytes decreased with the therapy and increased after the infection. These results suggested that influenza A may improve the clinical signs of myasthenia gravis, as is the improved case with measles.

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We report here two cases of recurrent bacterial meningitis following traumatic cerebrospinal fluid rhinorrhea.
Case-1: an 1-year-old girl had a penetrating injury to the nasal cavity with a chopstick. From 1 day after this accident, she had suffered from recurrent bacterial meningitis. She was diagnosed as having cerebrospinal fluid rhinorrhea, and underwent surgical repair of the bone defect.
Case-2: a 5-year-old girl had suffered from bacterial meningitis 4 times after head trauma.
A bone defect was demonstrated by 3-D CT and repaired surgically. We consider that 3-D CT is a useful tools to detect cerebrospinal fluid fistula.

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[in Japanese]

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