Computed tomograms (CT) from 509 epileptic children with possible intracranial pathology were studied. The infants of 6-24 months of age had apparently a slight enlargement of the frontal subarachnoid space as compared to the older children.
Pathological CT was found in 131 cases (25: 7%) of epileptic patients, 60.4% of infantile spasms patients, 52.4% of hemiconvulsion patients, 35.2 % of Lennox-Gastaut syndrome patients, and 16.2% of generalized tonic and/or clonic convulsion patients. Brain atrophy was the most frequent CT abnormality. Other pathological changes included brain tumor (2 cases), cerebral infarct (2 Cases), subdural hematoma (2 cases), tuberous sclerosis (2 cases), periventricular leucomalacia (1 case), agenesis of corpus callosum (1 case), normal pressure hydrocephalus (1 case), and periventricular calcification suggesing intrauterine cytomegalovirus infection (1 case). Two cases with status epilepticus showed hemispheric brain edema followed by an atrophy of the same hemisphere in the follow up CT pictures.
Patients with atrophy revealed by CT were found to have more pronounced mental and motor handicaps than patients without brain atrophy. Because of its non-invasive character, CT was a most suitable tool for patients with such a functional disease as epilepsy to find out an undetected morphological change.

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Six cases of brain tumor with seizures in children, 1-14 years old, two boys and four girls, were reported. These cases showed neither abnormal neurological findings nor symptoms of increased intracranial pressure, except two cases who showed the mental disturbances, such as emotional incotinence and disorientation in one case and menory disturbance and irritable character in the other cases. All cases manifested generalized tonic seizures, and one of these cases showed generalized tonic and clonic convulsions and another case had psychomotor seizures. Jacksonian or focal seizures were not observed in any cases. The interval from the initial seizures to the diagnosis ranged from three weeks to three years. Electroencephalogram showed focal slow bursts in all cases. In two cases, focal spikes were found. A high densisty area in computed tomography (CT) was found in all cases. The diagnosis of the brain tumor without any abnormal neuro logical findings in children should be indebted to CT examination. All cases were operated on, and the tumors were removed totally or subtotally. These brain tumors were found in the frontal lobe in two cases, in the temporal lobe in three cases and in the middle cranial fossa in one case. The tumor size was from 5 to 310 grams in weight, and it was not related to the duration of symptoms. Histological diagnosis was tuberous sclerosis, teratoid tumor, ependymoma, gemistocytic astrocytoma, malignant meningioma and angioblastic meningioma. The nature, incidence and localization of the brain tumor with seizures, the seizure type and the clinical examination were discussed. The seizure in brain tumor does not show a characteristic pattern in children. It will be necessary to perform the CT scan in the case with a focal slow burst on the electroencephalogram. Electroencephalography and CT scan seem to be the valuable diagnostic method for differentiating the symptomatic epilepsy.

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[in Japanese]

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Thirty-two epileptics with spike-and-slowwave complexes persisting over 8 years since the onset of convulsive seizures in infancy and childhood were selected among 7652 out-patients at the Department of Neuropsychiatry, Tokyo Medcal College. Thirteen cases exhibiting episodic symptoms were designated as the “episodic group” and 19 cases which exhibited no episodic symptoms were referred to as the “control group”.
The EEG was examined on the average of more than once a year and more than ten examinations were carried out in ten years. The duration and frequency of the spike-and-slowwave complex were determined only in the intervals of sizures. Changes in the frequency of more than 1 Hz and the duration of more than one second were recorded and examined.
The focus of the secondary seizures was determined on the basis of EEG findings and clinical symptoms persisting for 10 years or more.
Psychiatric symptoms were classified according to Landolt. Only the episodic symptoms were studied and chronic epileptic psychoses were excluded in this study.
The mean ages in which the onset of epileptic seizures began were 5.7 years in the episodic group and 8.4 years in the control group. The episodic symptoms on average appeared at 15.5 years of age.
In each group many refractory cases of tonicclonic seizures were included. They had convulsive seizures for several consecutive years and were seizure-free for the subsequent several years. This pattern of reapperance of tonic-clonic seizures was repeated. The clinical seizures reappeared again in some cases where the tonicclonic seizures had cased for more than 10 years since the onset of convulsions in infancy and childhood. Furthermore, the episodic group exhibited the maximum frequency of tonic-clonic seizures at the ages of two to three years of age and the intervals of seizures were shorter than those in the control group. The ratio of female patients to male patients in the episodic group was higher. There were 9 female and 4 male cases.
Episodic symptoms did not appear in the cases with primary tonic-clonic seizures but were found in the cases complicated secondary tonicclonic seizures with absece or psychomotor seizure. Most of these cases showed the temporal focus in EEG.
The frequency of spike-and-slowwave complex increased in seven cases and remained unchanged in six cases of the episodic group of patients, while the control group showed eight cases of patients with increase, eight without changes, two with fluctuations, and one with decrease in frequency. The duration of spikeand slow-wave complex was prolonged in two cases, unchanged in three cases, and shortened in eight cases in the episodic group, while in the control group there were eight prolonged cases, six unchanged cases, four shortened cases and one fluctuating, changeable case. In summary, there was a tendency of increase in the frequency of spike-and-slow-wave complex and of decrease in its duration. During the 15-20 year period, in the episodic group as compared with the control group.

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Two hundred and six patients with chronic headaches in childhood were studied. There were 127 patients with migraine (classic migraine 73, common migraine 54), 57 with unclassifiable headaches who complained of paroxysmal throbbing headache but did not satisfy Holguin's criteria of migraine, and 22 with muscle-contraction headache.
Activation procedures, including hyperventilation, photic stimulation and sleep, were used in all of the patients.
EEG was abnormal in 35% of migraineurs. In 24 cases (19%) the abnormality consisted of slow activity and in 32 cases (25%) of spike activity. In cases with classic migraine, the incidence of abnormality was not different from that of common migraine. The incidence of the abnormality was not related to age of onset of migraine, sex, length of history of migraine or frequency of attacks.
In cases with unclassifiable headaches 47% of EEGs were abnormal. In 10 cases (18%) the abnormality consisted of slow activity and in 21 cases (37%) of spike activity.
Muscle-contraction headache revealed an incidence of 41% EEG abnormality. In 2 cases (9%) the abnormality consisted of slow activity and in 7 cases (32%) of spike activity.
A high incidence of EEG abnormalities wasfound in all types of headaches. The incidence of spike activity in this study was higher than in many previous reports, probably because sleep records were obtained in all the patients.
From the fact that the incidence of EEG abnormality was not related to the type of migraine, age of onset of migraine, length of history of migraine or frequency of attacks, it was concluded that the changes found in EEG of migraineus children were not merely a secondary result of repeated attacks.

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Dystrophic axons were evaluted in the biopsied sural nerves in 4 cases of infantile neuroaxonal dystrophy. The mental deterioration and pyramidal signs were noted in all and the optic nerve atrophy in two cases. All showed a progressive mental and physical deteriorations from early infancy. Laboratory examination disclosed mild to moderate increase of serum alkaline phosphatase, LDH and GOT. Electroencephalograms showed fast waves or spikes. Motor nerve conduction velocities were normal. Fascicular nerve biopsy was performed at the age of 1 year 7 month in Case 1, 1 year 11 months in Case 2, 2 years 1 month in Case 3 and 3 years 10 months in Case 4. The abnormal axons were hardly seen at the light microscopic level except the large myelinated ones in thick sections. However, rather frequent abnormal unmyelinated axons could be observed under the electron microscope. These abnormalities were the stacked tubulomembranous or membranous structures, more dense than usual membrane, and the excessive amount of mitochondria and glycogen granules. A quantitative study disclosed that the number of adnormal axons was rather small, but its estimated incidence was more frequent in the unmyelinated fibers (0.89%) than in the myelinated ones (0.28 %). Although definite comparison was hardly possible because of the rather limited area studied, both myelinated and unmyelinated fibers were involved without any predominancy. The diameter histograms of both myelinated and unmyelinated fibers did not show evident abnormalities in all cases. From this result the pathogenetic mechanism in the peripheral nerve in this disorder seems to be different from usual neuropathies of both axonal degeneration type and segmental demyelination type, in which the predominant involvement was common in the large myelinated fibers.

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Three-year-seven-month-old boy who showed several cafe-au-lait spots on his back and macrocephalus was reported. His family history disclosed autosomal dominant heredity of von Recklinghausen disease. His younger brother showed a large head size (more than 98 percentile) at 6 months of age. The present case was borne with a normal head circumference (34.0 cm), but his head size was recognized to be rapidly increased since a month of age. His head circumference was 54.0 cm (more than 98 percentile) at 3 years and 7 months of age. His body length, weight and chest circumference were within normal limits. Neurologically, he showed no abnormalities but retarded by approximatelyone year of age by the psychosomatic assessment. Craniogram revealed a large calvariurn without any signs of increased intracranial pressure or dysostosis. The radiological measurement of his skull films by Haar's method proved that he had apparently macrocranium. CT scans revealed a normal ventricular size and no brain tumors.
Neurofibromatosis is a familial and congenital disease of neuroectodermal and mesenchymal origin where hyperplasia of these tissues is often present. We propose that macrocranium is an expression of von Recklinghausen disease.

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A 16-day-old girl was admitted to our hospital with a history of feeding difficuty, convulsions and cranial enlargement, and died at the age of 4 months. Autopsy disclosed hydranencephaly accompanied by subdural effusion and hematoma.
She was born at term but resuscitation was necessary. Shortly after birth, she developed feeding difficulty, convulsion, hypertonicity and hypothermia. On admission, she was apparently jaundiced and her head was enlarged with bulging anterior fontanel. Skull x-rays revealed widening of cranial sutures. CT scan at the age of 41 days disclosed only low density area in the supratentorial region. Transillumination of the head, cerebral angiography and electroencephalography agreed with the diagnosis of hydranencephaly. Her symptoms were alleviated after ventriculo-peritoneal shunting but she died of acute subdural hematoma complicated with systemic infection.
Postmortem examination revealed bilateral subdural effusion and hematoma. Both cerebral hemispheres were replaced by the fluid-filled membranous sac. The wall of the membranous sac has a continuity with the remnants of limbic and temporal lobes, basal ganglia, thalamus and midbrain. Aqueductal stenosis was present and the pyramids absent. Microscopically, the wall of the membranous sac was composed of leptomeninges and glial tissue. Multiple sections of the membranous sac disclosed the absence of ependymal lining on the inner surface of the sac. Both internal carotid arteries were very narrow and anterior, middle and posterior cerebral arteries were also hypolastic.
Pathogenesis of hydranencephaly and differential diagnosis from congenital hydrocephalus were discussed. Clinical implication of subdural effusion (hematoma) in the present case was also stated.

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