Methods of computer technology (intelligent technology, IT), such as multimedia and virtual reality, are utilized more and more in all medical fields including child neurology. Advances in the digitalization of individual medical data and multi-media technology have enabled patients to be able to obtain their own medical data by small media and to receive medical treatment at any hospitals even if they are located in distance place. Changes from a doctor oriented to patients oriented medicine is anticipated.
It is necessary to store medical data from birth to adulthood and to accumulate epidemiological data of rare diseases such as metabolic diseases or degenerative diseases especially in child neurology, which highly require tele medicine and telecare at home. Moreover, IT may improve in the QOL of patients with neurological diseases and of their families. Cooperation of medicine and engineering is therefore necessary.
Results of our experiments on telemedicine, telecare and virtual reality are described.

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The management of refractory status epilepticus (RSE) is crucial in preventing neurologic impairment.Although a variety of treatments for RSE including continuous infusion of midazolam (MDL) or pentobarbital (PTB) have been carried out, they are not always effective. Intravenous very-high-dose phenobarbital (PB) has been recommended as having many advantages in the United States, but is not available in Japan.
We treated 3 patients suffering from long term RSE with non-intravenous high-dose PB (NIHDPB). T h e i r seizures were not controlled by continuous infusion of MDL and/or PTB. PB was initially given intramuscularly or rectally and then orally. Within a few or ten days, seizures were completely controlled, and consiousness level g r a dually improved in all cases. The serum levels of PB at seizure control ranged from 50 to 58μg/ml. The epilepti form activities on EEG nearly disappeared in the absence of the burst suppression pattern. Hypotention and respiratory depression did not develop during NIHDPB. Elevated gamma-GTP levels with normal hepatic transaminases were seen in all cases, but it was not necessary to discontinue NIHDPB. NIHDPB may be one of the most effective and safe treatments in Japan for status epileptics refractory to continuous infusion of MDL or PTB.

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To obtain a model system, useful in brain research, we have developed a new perfused brain model in rats. In this model the cerebral circulation is separated from the systemic circulation, while the connection between the central and peripheral nervous system is preserved. After the external carotid and vertebral arteries were ligated, bilateral common carotid arteries and external jugular veins were cannulated. Rinsed human O-typed red blood cells and modified Ringer's solution were injected using two infusion pumps, and the venous blood was drained. The infusion rate was adjusted to achieve normal cerebral blood flow (CBF) of rats (the control state).
Normal electrocortical activities were observed on electroencephalograms (EEGs) for more than 1 hour in the control state. Somatosensory evoked potentials were also recorded. Infusion of pentylenetetrazol (PTZ) induced epileptic discharges on the EEG and caused an increase in blood pressure. Measurements of cerebral oxygenation with near-infrared spectroscopy (NIRS) showed increase in total and oxygenated hemoglobin accompanied by a decrease in deoxygenated hemoglobin. Since the flow rate was not changed, the changes in the NIRS parameters may have reflected dilatation of arterioles.
Using this new model, we can change CBF and/or cerebral metabolism without affecting the systemic circulation. Unlike previous isolated perfused brain models, the present model maintains the connection between the central and peripheral nervous system. Thus, our model should provide a new approach to brain research.

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In order to investigate the effect of selenium (Se) on electrocardiograms (ECG), we studied the serum Selevels and ECG in 25 patients with severe motor and intellectual disabilities. Serum Se levels in patients receiving tube feeding with enteral formulae were lower than those in orally fed patients. ECG abnormalities including an inverted T wave and depression of the ST segment were more common in patients under tube feeding. During tube feeding with enteral formulae, it is necessary to examine serum Se levels and ECG. Serum Se levels should be kept at 5 to 6μg/dl to prevent cardiac dysfunction. To prevent selenium deficiency, either addition of ordinary foods, replacement by selenium-rich formula, or Se supplementation is recommended.

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We reported a 41-day-old girl starting with partial status convulsivus. She had suffered from infantile epileptic encephalopathy manifesting with recurrent focal motor status convulsivus, eventually requiring general anesthesia for the treatment. She had had never myoclonic seizures nor tonic spasms. The examinations including brain MRI scan and metabolic screening tests were all negative. The ictal EEGs showed the onset of the seizures from the left frontal region. Interictal EEG demonstrated multifocal spike discharges as well as discontinuous abnormal EEG pattern resembling suppression-burst during sleep. The seizures were resistant to the multiple antiepileptic drugs, but zonisamide achieved full seizure control as well as improvement of the EEG. Her psychomotor development was severely retarded at 3 years of age.

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We report here a 14-year-old boy suffering from intractable epilepsy since the age of 2. Neuroimaging showed a lesion in the left temporal lobe. He underwent resection of the left temporal lobe and multiple subpial transection of the left frontal lobe at the age of 8. Histopathological findings of surgical specimens were similar to those of tubers of tuberous sclerosis (TSC), although he had no other TSC stigmata. To discriminate from cortical dysplasia grade III, we examined the Immunohistochemical expression of hamartin and tuberin, the TSC1 and TSC2 gene products. Based on results, we diagnosed this case as having TSC. He has been seizure free since the operation. Although lower than preoperatively, his intelligence quotient has not been declining progressively

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We reported an 11-year-old girl with left thalamic infarction causing aphasia and dysgraphia. The lesion corresponded to the perfusion area of the tuberothalamic and paramedian arteries. Confrontation naming and word finding were impaired, but phonological cuing was very helpful despite the absence of amnesia. Dysgraphia was observed only in Kanji (morphogram) writing, and was also improved by visual cuings. These findings suggested difficulty in memory retrieval, in which the left thalamus might have some role.

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A 14-year-old girl was diagnosed as having herpes simplex encephalitis by a polymerase chain reaction examination of the cerebrospinal fluid. MRI showed diffuse high signal areas at the bilateral temporal lobes and right frontal lobe.
She was treated with a total of 350 mg/kg of acyclovir for 23 days, and discharged without abnormal neurological findings.
On the 90th day after the onset, however, she became excitable and aggressive to her family. She excessively seeked relations with several boy friends. MRI revealed dilation of the bilateral ventricles and atrophy of the temporal lobes. These pathologic changes including the atrophy of hippocampus may be responsible for the character changes in this case.

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Two pediatric cases of hemophagocytic syndrome (HPS) with the central nervous system (CNS) involvement are reported. Case 1, a two-month-old girl with primary hemophagocytic lymphohistiocytosis, showed high fever, skin rash and hemorrhagic diathesis followed by convulsion, impairment of consciousness and involuntary movements. Case 2, an eight-year-old boy, complained of visual hallucination in the course of measles complicated with interstitial pneumonitis. MRI of the two cases disclosed multiple T2-hyperintense areas in the CNS. Both clinical symptoms and MRI findings of these cases significantly improved in response to cyclosporin A (CsA) combined with methylprednisolone (mPSL) pulse therapy. Although the pathogenesis of CNS lesions in HPS remains to be elucidated, the etiologic role of hypercytokininemia is obviously important. Because CsA and mPSL have a potent inhibitory effect on the action of cytokines, it is rational and worthwhile to use these agents in the treatment of HPS with CNS involvement.

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We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spikewave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.

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