Many children with severe neurological disorders suffer from dysphagia. An attempt was made to improve tube feeding technique by using a Nelaton catheter.
Twenty-two boys and 21 girls ranging from 9 month-to 15 year-old who were diagnosed as cerebral palsy, sequelae of encephalitis, infantile spasms, and others were studied. Most of them demonstrated branchial aspiration radiographically. A Nelaton catheter was passed orally into the stomach 3 to 6 times daily and liquidized feeds were administered through it.
Sixteen children had had an indwelling nasogastric tube, some of whom had been throat carriers of Pseudomonas aeruginosa before the study. These throat carriers showed no bacterial growth in their throat swab cultures at the end of the study. Moreover, branchial aspiration was decreased with successful oral feeding in 8 among 16. Twelve children who had been orally fed with frequent aspiration gained weight and their nutritional condition improved. These 28 children showed a marked reduction in stridor and mucus secretion with fewer febrile episodes. To 15 children who had aspirated only in swallowing liquids, the oral catheter method wasused only for liquids with a noticeable improvement in their general condition. This method could not be used to 3 subjects due to persistent gagging on attempting to introduce the tube.
It is thought that stimulation of the pharynx by the passage of the tube facillitates swallowing and reduces aspiration. This method appears to be beneficial for children with severe dysphagia.

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Diagnosis of ataxia-telangiectasia (AT) has largely been dependent on the clinical findings such as cerebellar ataxia, telangiectasia, and immunological deficiency. However, diagnosis of AT by these ordinary criteria is sometimes not sufficient because of the lack of immunological abnormalities.
We examined three cases of AT by ordinary clinical criteria and also by X-ray sensitivity of cultured skin fibroblasts. Case 1, a 9-year-old boy, revealed typical clinical features of AT. However, he had no abnormality in serum IgA or IgE. Case 2, a 10-year-old boy, showed decreased serum IgA level. Case 3, a 19-year-old female, had typical clinical features of AT with normal serum IgA, and developed papillary adenocarcinoma of thyroid which was surgically removed.
Fibroblast strains derived from these three cases of AT and from the parents of Case 3 were examined with regard to X-ray sensitivity. Three fibroblast strains derived from AT patients (AT homozygotes) showed remarkable hypersensitivity to X-ray. Fibroblast strains derived from the parents (AT heterozygotes) of Case 3, however, showed normal X-ray sensitivity.
Recently, AT fibroblasts have been known to show hypersensitivity also to some mutagen like neocarzinostazin as reported by Shiloh et al. Fibroblasts from Case 3 revealed hypersensitivity to nocarzinostazin. However, the sensitivity of the strains from AT heterozygotes (the parents of Case 3) showed no apparent difference from that of control cells. The assay system for mutagen is quite unstable and proper conditioning of the seeding cell number is important for the carrier detection. However, the diagnosis of AT homozygotes was definitely established by X-ray irradiation to cultured fibroblasts from patients.

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We investigated short latency somatosensory evoked potential (S·ESEP) following median nerve stimulation in 15 children with congenital central nervous system disorders. Somatosensory stimuli were delivered through a disc electrode placed over the median nerve at the wrist joint. The uniform recording sites used were the central region of the scalp and Erb's point. Reference electrode was placed on the hand contralateral to the median nerve stimulated.
1) S·ESEP were normal in the patients with primary microcephaly, megalencephaly, Smith-Lemli-Opitz syndrome, 2 with hydrocephalus and 3 of 5 with Down's syndrome.
2) Lissencephaly syndrome: The components below N1 were absent and P3 was low in voltage.
3) Down's syndrome: P4 was absent in one patient and P1-P3 interpeak latency per one meter of height was prolonged in another patient.
4) Adrenoleukodystrophy: The components below weve absent at 10 years of age, and P3 was low in voltage at 12 years of age.
5) Menkes'kinky hair disease: The wave pattern of S·ESEP was normal but low in voltage.
6) Krabbe's disease: P4 was absent at 10 months and all components were distinctly detected at 1 year of age. P1 peak latency per one meter of his height was prolonged.
It was concluded that S·ESEP might be useful for the diagnosis of neurological disorders in somatosensoryconduction pathway and the investigation of progressive central nervous system diseases

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A follow up study was performed on 1007 epileptic children with respect to natural history, prognosti factors, and remission and relapse rates in the Matsudo Clinic. They were first diagnosed at ages less than 15 years and were followed up for 13 years on an average. Anticonvulsants were tapered off after the patients had had no seizures for at least three years and no seizure discharges in EEG for one to two years.
Fifty-one patients (5%) died during the period of this study, and 407 (43%) out of 956 living patients were medication-free without relapse for one to 15 years (mean: 5 years). There were 285 patients (30%) who had been seizure-free but dependent on medication or reducing the dosage of medication, and 264 patients (27%) were seizure-free for less than 5 years.
The remission rate was 71% for the five-year period. The relapse rate was 8% after discontinuation of drugs.
Factors for favorable outcome were as follows (a) grand mal, absence seizures and simple partial seizures, (b) no neurologic complication, (c) normal mentality, (d) less than 5 years between the first and the last seizures, (e) seizure control by the age of 10 years, (f) disappearance of epileptic discharges in EEG by the age of 15 years. On the contrary, factors of poor outcome were as follows:(a) Lennox-Gastaut syndrome, myoclonic seizures and complex partial seizures, (b) some neurologic deficits, (c) mental retardation, (d) the onset of seizures after 10 years, (e) severe EEG abnormalities.

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Long-term serial examinations of EEG, visual evoked potentials (VEP) and CT findings were made on 7 patients with subacute sclerosing panencephalitis. The clinical stages were classified by Freeman's criteria. An amelioration was found in 4 cases after the stage IIIB.
The basic EEG pattern showed alpha rhythm at the stage IC and high voltage slow waves increased from the stage IIB, which were replaced by low voltage slow waves after the stage IIIB. In the clinically ameliorated cases, theta and alpha waves reappeared. Continuous frontal delta waves were found at the IC-IIA stages.
All the cases showed periodic bursts and burst-burst intervals shortened with the progress of clinical stages. In the remission period the bursts disappeared.
Epileptic discharges, which were mostly composed of diffuse slow spike-and-waves and irregular spikeand-waves, were observed frequently at the IIB-IIC stages. These were localized bifrontally after the stage IIIB. Fourteen c/s positive spikes were found at IIA-IIIB stages.
Sleep EEGs after the stage IIA were unclassifiable according to APSS's criteria. Sleep spindles disappeared at the IIA-IIB stages. The REM percentage and REM density were low.
VEP was examined in 5 cases. Prolonged latency of wave IV was recognized in two cases after the stage IIB.
IV-V peak-to-peak amplitude was reduced in 3 cases after the stage IIIB.
CT scans disclosed the presence of frontal dominant diffuse brain atrophy after the stage IIB, which was further aggravated with the progress of the disorder.
These findings not only demonstrate the significance of EEG for the diagnosis of SSPE but also indicate advantageousness of EEG for determination of clinical stages and prognostication, when combined with the findings of VEP and CT.
The detection of delta waves in the frontal area by EEG and frontal dominant brain atrophy by CT in earlier stages of SSPE suggests fronto-occipital progression of the brain lesion.

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Combination therapy of sodium valproate (VPA) and clonazepam (CZP) was instituted in 18 children suffering from intractable epilepsy; 13 with symptomatic West syndrome and 5 with Lennox syndrome. The required daily doses of VPA ranged from 20.0 to 59.7mg/kg (mean 32.8), and those of CZP ranged from 0.03 to 0.19mg/kg (mean 0.08). At the first month of this therapy, complete seizure control was achieved in 5 out of 13 patients with West syndrome (38.5%), and in 1 out of 5 patients with Lennox syndrome (20.0%). The frequency of seizures were reduced by more than 50% in 15 cases (83.3%). The maximum effect occurred from 2 to 28 days (mean 10.4 days) after starting the treatment.
After follow-up for 12-46 months (mean, 24.7 months), this combination therapy was still effective in 7 cases (46.7%), and 5 cases (33.3%) were seizure-free.
In our studies, therapeutic benefit was evident at serum levels (samples taken 3 hours after the morning dose) of 49.0 to 148.0μg/ml (mean 93.8) for VPA, and of 5.4 to 50.5 ng/ml (mean 19.2) for CZP. This combination therapy was effective against tonic spasms, tonic seizures and myoclonic seizures.
Twelve cases (66.7%) had symptomatic side effects. Drowsiness (50.0%), increased salivary secretion (50.0%), thrombocytopenia (22.2%), and minor seizure status (5.6%) were seen. These side effects were dose-related and reversible.
This combination therapy of VPA and CZP may be effective against symptomatic West and Lennoxsyndromes, when serum levels are maintained between 50 and 100μg/ml for VPA, 10 and 35ng/ml for CZP.

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Bioavailabilities of three different phenytoin (PHT) powders sold in Japan (companies S, D and F) were compared. One hundred and fifty children (aged 2-15 years) were assayed PHT plasma levels in steady state under the condition that PHT plasma level was 2.5-15.0μg/m/ and S/D was obtained (S plasma level of PHT μg/ml, D: dose of PHT mg/kg/day).
These children were divided into two groups by age (under 6 years old and over 7 years old) for each group. In the older age group, bioavailability was high in the order of S, D and F with a statistically significance. In the younger age group, S and D showed equal bioavailabilities but F showed a low bioavailability also statistically significant.
It was concluded that these differences may have been caused by the degree of smashing of PHT crystals.
Official organization should check the bioavailability of anticonvulsant produced by different drug companies and physicians should always pay attention to this problem.

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Prenatal damage to the developing central nervous system causes permanent neurological deficits. Among children who have mental and/or phisical handicaps, microcephaly is a common phisical manifestation. In this experiment, the development of the microcephalic mouse brain has been investigated by neurochemical and histofluorescent techniques.
Administration of cytosine arabinoside (ara-C) to the pregnant mice (30mg/kg, I. P. injection on days 13.5 and 14.5 of gestation) led to a microcephaly in their offsprings, with an approximately 50% reduction in the brain wet weight. Contents of protein, RNA, and DNA in the cerebral hemisphere of 1-day-old treated mouse was also one half of the control values (P<0.001), and those reductions persisted through the period examined. The cellular density (DNA/gram wet weight) of ara-C treated cerebral hemisphere was, however, not different from that of the control, and the developmental pattern of the cerebral hemisphere was similar to that of the control.
The total noradrenaline and serotonin contents in the cerebral hemisphere of ara-C treated mouse increased at the same rate as those of the control, and there were no statistical differences between two groups through the period examined. Noradrenaline and serotonin concentrations in the cerebral hemisphere of ara-C treated mouse were about 2.0 times more than those of the control even at 1 day of age. This phenomenon also lasted through the period examined. Histofluorescent studies showed that catecholaminergic fibers in the cerebral cortex of ara-C treated mouse increased in its densities, thus comfirming the biochemical data. Noradrenaline fibers in the somatosensory cortex, moreover, produced numerous terminal arbors and varicosities.
Although the total GABA content in the cerebral hemisphere was about 50% less in the ara-C treated mouse, GABA concentration was not different from that of the control.
These findings indicate that fewer cortical neurons in the microcephalic mouse have been innervated moreby monoaminergic nerve terminals, thus causing abnormal cortical functions.

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A case of posthemorrhagic hydranencephaly in utero with congenital deficiency of factor MI (fibrin stabilizing factor) was reported. A 79-day-old boy was born of normal delivery and had umbilical bleeding on the 11th day after birth, which was treated by fresh blood transfusion. His sister died probably of intracranial hemorrhage, and his brother has been treated for congenital deficiency of factor XII. They also suffered from umbilical bleeding at their neonatal period. Therefore those cases may have had an autosomal recessive factor XIII deficiency. The neuropathology showed hydranencephaly and polymicrogyria with gray matter heterotopia and hemosiderin deposits in the cerebral white matter of residual cerebrum. Those findings suggested that an intracranial hemorrhage occured about 3 months of gestational age.

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The use of radiation therapy has significantly improved the prognosis of certain brain tumors. However, a few patients have been reported who developed cerebrovasculopathy accompanying transient ischemic attacks several months to several years after radiation therapy. The present report described cerebrovascular disorders after radiation therapy for brain tumors.
The first case was an 8-year-6-month-old boy treated with a total dose of 5, 200 rads after partial removal of a right periventricular astrocytoma extending into the thalamus. Two years and 7 months after completion of the radiation therapy, he showed transient ischemic attacks of numbness in the right upper limb and right hemiparesis. Arteriography revealed stenosis or occlusion of the anterior and middle cerebral arteries. Preoperative arteriography did not show occlusion nor narrowing of the cerebral arteries. The second case was a 2-year-8-month-old boy diagnosed as diencephalic syndrome, because of marked emaciation and a huge tumor mass expanding into the diencephalon and frontal lobe on the brain CT scan. He was irradiated with up to 5, 000 rads. Seven months after radiation therapy, he developed transient right hemiparesis. Arteriography revealed stenosis or occlusion of the middle sized cerebral arteries.
Although radiation therapy is acceptable in children with certain brain tumors, and very few patients develop postradiation vasculopathy, the risk of radiation therapy requires more careful consideration in the treatment of intracranial tumors.

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A five-year-old boy with Down syndrome who showed the karyotype, 46, XY, dic t (21; 21) (p11; p11) and moya moya disease was reported. CT scan and cerebral angiography were taken for his recurrent convulsive seizures followed by postictal paralysis. CT scan showed diffuse cerebral atrophy with low density lesions in the right frontoparietal lobes, and later showed more widespread changes in the both hemispheres. Angiography revealed the stenosis of the distal portions of bilateral internal carotid arteries and the occlusion of the circle of Willis with collateral networks which were typical of moya moya disease. Furthermore, the findings of tubular and focal stenosis of bilateral extracranial carotid arteries might suggest the presence of a systemic vascular disorder.

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We presented a 3-year-old boy complaining of hemichorea in the left upper extremity. Cranial CT scan performed 3 days after the onset revealed slight low density area in the right frontal region, and a low density area appeared in the head of the right caudate nucleus 12 days after the onset. This lesion was enhanced by the contrast material. A porencephalic change was detected in the same region 28 days after the onset and was not enhanced. The right CAG showed an occlusion of the right middle cerebral artery at the MC (M1) portion. On the 30th day of the disease, the hemichorea spontaneously disappeared. Six months later, he had neither neurological defects nor abnormal movement.
On the basis of these informations, we supposed that the hemichorea in this patient was related to the disturbance not only of the caudate nucleus but striatal circit (cortex-striatum-globus pallidus-thalamuscerebral cortex). In some reports on adult patients hemichorea were correlated with infarction of the caudate nucleus due to the cerebral artery occlusion disease. This may be the first case of the hemichorea which presented the caudate infarction shown by CT scan in children.

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[in Japanese]

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[in Japanese]

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