The clinical application and future prospect of neuroendoscopic surgery are described. In the historical trend, it is emphasized that the neuroendoscopic surgery has become one of the major or leading procedures in “minimally- invasive approaches” to the intracranial lesions. The presently-available instrument includeprocedures using an endoscope of three different types: rigid-rod, steerable/ flexible-fiber-rod and semirigid-rod. To use a rigid-rod or fiber-rod scope, it is available to apply forceps, laser, a microballoon and other fine instruments. The indication for neuroendoscopic surgery has been expanded to almost all neurosurgical procedures, as “endoscope-assisted” procedures are applied more to micro neurosurgical procedures (endoscope-assisted microsurgery). The realistic indication for “pure neuroendoscopic surgery” includes reconstruction of cerebrospinal fluid (CSF) pathway such as third ventriculostomy, septostomy, foramen reconstruction, fenestration of septation; removal or biopsy of tumor/cystic lesion, mainly in the intraventricular regions; and removal of hematoma involving the cerebral parenchyma (intracerebral hematoma), ventricles/(intraventricular hematoma) or subdural space (subdural hematoma). The intraparechymal or deepseated intracisternal lesions can be the realistic indication for “pure neuroendoscopic surgery” when the instrument are further developed in future. Neuroendoscopic surgery will play a promising and major role in the “minimally-invasive neurological procedure” in the 21st century.

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The developmental change of spontaneous hand movements were investigated longitudinally in 5 lowrisk pre-term infants. The observation period was divided into three: 33-40, 41-52 and 53-60 weeks in order of gestational age. Both hand-mouth contacts (H-M-C) and hand-face contacts (H-F-C) were frequent in the 33-40-week period, and decreased drastically in the 41-48 week period. In the 53-60- week period, the number of H-F-C remained at a low level, while H-M-C increased again to the level of the first period. Furthermore, the mouth was open as the hand came into contact with it as frequently in the 33-40-week period as in the 53-60-week period. These findings suggest that H-M-C are neurologically distinct from HF-C, and that the H-M-C before 41 weeks and after 50 weeks share some common components of this neurological mechanism.

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We report here three infants with frequent convulsions in the post-eruptive stage of exanthem subitum (ES) due to human herpesvirus 6 (HHV-6) infection. Postictal electroencephalogram (EEG) showed in all the patients abnormal epileptic discharges, which disappeared in the following by three to eighteen months. In one case, brain magnetic resonance imaging (MRI) revealed focal gliosis. SPECT demonstrated hypoperfusion of the lesion. In the cerebro spinal fluid (CSF) of all the patients, HHV-6 DNA was negative on polymerase chain reaction (PCR) and HHV-6 antibodies were not significantly increased. Although encephalitis has been reported to complicate primary HHV-6 infection, our patients were not diagnosed as having encephalitis because of the clinical, CSF, EEG and CT findings. However, they had frequent convulsions. Not only virus invasion but also a secondary reaction including vasculitis may cause the central nervous system complications of HHV-6 infection. Frequent convulsions may occur in the post-eruptic stage after HHV-6 infection.

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Of the 75 patients with infection-related acute encephalopathy or encephalitis treated by us in the last 10years, 28 had acute onset encephalitis. The results of clinical studies on these 28 patients were as follows:(1) The number of cases who exhibited CNS manifestations during (A) and after pyrexia (B) were 15 and 13, and the ages of predilection were infancy and school age, respectively. (2) MRI studies in cases of A revealed multifocal CNS lesions in 1 case, localized lesions in 7 and normal findings in 7. In B, there were 3 cases with multifocal lesions, 8 of focal lesions and 2 of normal findings. (3) The 7 cases of localized lesions in A were divided into 5 of herpes encephalitis and 2 of suspected vasculitis. Vasculitis was suspected in 3 of 8 cases of localized lesions in B. Thus, vasculitis is considered to be an important cause of encephalitis. (4) Brain lesions in the 5 cases of herpes encephalitis were occipital dominant in 4. Only one case had a temporal lesion. (5) All cases with focal MRI lesions and CSF pleocytosis, having evidence of direct viral invasion, were herpes encephalitis. Direct viral invasion was not proven in any other cases. (6) Although the term encephalitis is often used in clinical practice, the process by which the CNS lesions occur in acute viral infection is still unknown. Therefore it is not easy to establish the diagnosis. The diagnostic criteria of encephalitis should be reconsidered. New specific methods to analyze the cause of CNS lesions are necessary.

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In 19 cases with newly diagnosed symptomatic West syndrome, we assessed interictal regional cerebral blood flow (rCBF) before ACTH therapy with single photon emission computed tomography (SPECT) Based on the SPECT findings, we divided these cases into 3 groups: normal rCBF (Group A, 7 cases), abnormal rCBF corresponding to cerebral lesions on MRI and CT (Group B, 6 cases), and abnormal rCBF in areas different from lesions on MRI and CT (Group C, 6 cases). We compared clinical features, response to initial treatment, and short-term outcome among these 3 groups. No significant differences were found in clinical characteristics (sex, age of onset, prior seizures before onset of spasms, EEG findings). Four cases inGroup B (67%) and 5 in Group C (83%) showed complete cessation of spasms after initial treatment (high dose vitamin B6→zonisamide→ACTH therapy), while in Group A only 2 patients (29%, p>0.05;compared to Group B or Group C) responded. Although not statistically significant, short-term prognosis (both seizures and development) after a mean follow-up of 2 years and 8 months was also worst in Group A. Our results suggest that normal SPECT findings may be predictive of unfavorable prognosis in infants with symptomatic West syndrome.

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Two hundred and two cases of CP in Shiga Prefecture, born between April 1977 and March 1987, were classified at 6 years of age into 5 groups: 69 with spastic diplegia (34%), 62 with tetraplegia (31%), 33 with hemiplegia (16%), 23 with the dyskinetic type (11%) and 15 with the ataxic type (7%). The rate of preterm birth was 32%, being highest in the spastic diplegia group. The etiological and risk factors were analysed in these cases.
In term infants with spastic diplegia, the presumptive causes were unknown in 55%, prenatal in 28%, and perinatal in only 17%. In preterm infants with spastic diplegia, periventricular leukomalacia was the most important. In term infants with tetraplegia, brain anomalies and hypoxic-ischemic encephalopathy were the two main causes. In preterm infants with tetraplegia, the presumptive causes were perinatal in 67%. In most of the patients with hemiplegia, there were unilateral lesions such as middle cerebral artery infarction and cerebral hemiatrophy. Most of the dyskinetic cases had perinatal causes such as bilirubin encephalopathy in 9 patients. In 40% of the ataxic cases, there were brain anomalies.

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The severity of the disability and complications was evaluated at 6 years of age in 202 cases of cerebral palsy (CP) in Shiga Prefecture (69 with spastic diplegia, 62 with tetraplegia, 33 with hemiplegia, 23 with the dyskinetic type and 15 with the ataxic type) born between April 1977 and March 1987. The degree of gross motor disability differed among the clinical types, being mild in 45%, moderate in 17%, and severe in 39%. Gross motor disability was generally correlated with mental retardation, with some exceptions. Some nonambulatory cases exhibited a normal or subnormal mentality, and most of such cases had been preterm infants with spastic diplegia. Most cases with mild gross motor disability and severe or moderate mental retardation had been term infants. Forty-eight percent suffered from epilepsy (25% in spastic diplegia, 86% in tetraplegia, 45% in hemiplegia, 39% in the dyskinetic type and 13% in the ataxic type). Microcephaly was noted in 35%(66% in tetraplegia and about 20% in other types).

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The age at the first clinical referral or diagnosis of cerebral palsy (CP), the age at the onset of treatment, the route of referral, and the kind of school entered were investigated in 202 cases of CP in Shiga Prefecture (69 with spastic diplegia, 62 with tetraplegia, 33 with hemiplegia, 23 with the dyskinetic type and 15 with the ataxic type) born between April 1977 and March 1987.
In the hemiplegia, spastic diplegia and ataxic types, the age at the first clinical referral or diagnosis, was above 1 year in 42%, 39% and 33%, respectively. In the tetraplegia and the dyskinetic types, by contrast, such a delay occurred in only 9% and 4%, respectively. Cases of the former three types were referred from medical institutions less frequently (53% in the ataxic type, 52% in spastic diplegia and 40% in hemiplegia) than those of the latter two types (76% in tetraplegia and 61 % in the dyskinetic type). About 30% of the cases were referred from health centers (38% in spastic diplegia, 33% in hemiplegia, 30% in the dyskinetic type, 20% in the ataxic type and 16% in tetraplegia). A considerable number of cases visited a clinic without reference (27% in hemiplegia, 27% in the ataxic type, 10% in spastic diplegia, 9% in the dyskinetic type, and 8% in tetraplegia).
Fifty-three percent of the cases entered an elementary school (ordinary classes in 30% and special classes in 23%), 41% a special school, and 5% entered a protective institution.
The early diagnosis of hemiplegia, spastic diplegia and the ataxic type of CP was difficult in some cases Cases with suspected signs of CP should be referred to clinic early in the absence of definite diagnosis.

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The authors assessed the status of mental health of families caring for relatives with developmental disabilities and neurological diseases. They were caring for their children in good morale, however, “burnouts” or neurotic conditions were more frequent in these families than in the medical staffs, the latter being studied in our previous researches. Mentally healthy persons consulted their spouse about their children as well as other things, and received his/her assistance in daily care. Families helped by unrelated persons were in better mental state. Families who had utilized inpatient care before entering a school had higher expectations from their surroundings. Therefore, medical services should provide support to such families to help improve their mental health.

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We reported here a 5-year-old girl with Sotos syndrome who developed acute shock encephalopathy syndrome (ASES), and differentiated ASES from Reye syndrome (RS). Abrupt onset of shock and status epilepticus developed and these were followed by disseminated intervascular coagulation (DIC) and liver damage. Gradual elevation of hepatic enzymes, high serum bilirubin value, and normal serum ammonia value in acute phase were incompatible with typical RS. Liver histology showed severe, diffuse necrosis of hepatocytes consisting of granular and vacuolar degeneration, which were quite different from those of RS. Thus, the disease process of ASES was shown to be different from those of RS.

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The author reported a case of spastic cerebral palsy in a 4-year-old boy who underwent functional posterior rhizotomy and were followed up for more than one and a half years after surgery to evaluate the degree of spasticity. The patient's preoperative ADL was highly restricted due to severe spasticity. In the surgery, the bilateral rootlets from L2 to 51 were selectively cut if an abnormal reflex activity was demonstrated by neurophysiological methods. Spasticity markedly decreased postoperatively and alleviated the family's burden for daily care. During the period of follow up, residual spasticity has subsided and the effect in controlling spasticity was long-standing.
Functional posterior rhizotomy has been recognized as an established neurosurgical treatment for spastic cerebral palsy in childhood in the North America. However, the procedure is uncommon in Japan. The author outlined here the procedure and its history.
Functional posterior rhizotomy is a strong armament for treating spasticity in cerebral palsy. The procedure would greatly benefit patients with spastic cerebral palsy in combination with current treatments.

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I reported here girl with localization-related epilepsy who developed particular involuntary movements (IVMs) with a therapeutic dosis of carbamazepine. An epileptic seizure, loss of consciousness followed by a tonic-clonic seizure on the left, occurred at 9 and half years and carbamazepine was administrated. With the blood levels of 9.3μg/ml (4 hours after administration), oral dyskinesia, choreiform movements of fingers of both sides, and myoclonus of the left arm developed. These IVMs disappeared soon after the decrease of the dosis (5.8μg/ml, 6 hours after administration). As there was no other clinical and laboratory findingsresponsible for these IVMs, carbamazepine was thought to be the causative agent for them. The neurons and neural system related to the localization-related epilepsy may also have been involved.

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We studied the language comprehension deficit of a 11-year-old child with a semantic-pragmatic disorder. We used an original test battery using abstract nouns common to the tasks of repetition, reading aloud, auditory comprehension and comprehension of written words. Although he could repeat and read aloud words as good as normal controls, he could not choose correct pictures from semantically or phonemically resembling pictures by listening to or reading target words. This test demonstrated the dissociation between his phonemic and semantic processing abilities. An examination of the cerebral blood flow with SPECT suggested that the dysfunction of the left temporal lobe caused the deficit in language comprehension.

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[in Japanese]

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[in Japanese]

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