Developmental studies of postural regulations have contributed to a field of developmental neurology, and one of the most efficient approaches may be a measurement of plantar contact pressure patterns. In the present study, a photoelasticity technique which can visualize the loading force as isochromatic circle patterns was used as a “sensor” part forquantification of plantar pressure. Children with Down syndrome (DS) (n=18) known to have marked muscular hypotonicity were examined as a clinical model for functional evaluation of plantar arches in respect to body-weight-loading distribution. The results showed that the first fringe number in DS is significantly increased and Cv value for the local force supporting the body weight of DS children is significantly decreased. This type of analytical method proves to be useful for objective evaluation of musculo-skeletal functions of feet under the condition of standing upright atrest.

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With a photoelastic method, contact pressure patterns between foot and the floor surface were recorded, occasionally for one minute. The aim of this study is to evaluate the development or maturity of postural control. As a clinical model to be tested, Down syndrome characterized by marked muscular hypotonicity was chosen for quantitative analysis. The following results were obtained ;(1) the center of contact pressure is shifted significantly forward in Down syndrome, (2) the position tends to move forward on the left side in both normal and Down syndrome groups, and (3) there was no distinctive laterality of weight loading on either side. Flaccidity of ankle joint movements such as abnormal eversion was indicated by a special parameter, which was obtained for the first time by this method.

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The recent development of a new radiopharmaceutical 123 I-isopropyl-p-iodoamphetamine (IMP), which is taken up by the brain from the blood flow, has offered a possibility of constructing scintigraphy maps of regional cerebral blood flow (rCBF) using single photon emission CT. We evaluated the clinical utility of this method in moyamoya disease.
Five patients with moyamoya disease, who were examined by IMP-SPECT before and after bypass operations, were selected. On the IMP images before operations, all patients showed focal or global decrease of rCBF. There was a good correlation between the area and degree of rCBF abnormalities and severity of clinical symptoms and stage on angiography. The area of rCBF decrease coincided with the dominant area of rebuild-up phenomena on EEG. On the IMP images just after operations, the rCBF changed for the worse transiently in three patients, and the crossed cerebellar diaschisis appeared in two patients. On the IMP images 3 months after operations, the rCBF increased in company with the improvement of clinical symptoms. The increased rCBF on the point of operation was recognized in two patients with good prognosis.
These results suggest that IMP-SPECT is a noninvasive and useful method of assessing the effects of therapy as well as the characteristic hemodynamic abnormalities in moyamoya disease.

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Rats with huge porencephaly occupying the left cerebral hemisphere were used for this study. Porencephaly was caused experimentaly by the ligation of the left common carotid artery and subsequent hypoxic exposure at the age of 7 days. A large band of houseradish peroxidase (HRP) positive fibers was found crossing at the pyramidal decussation to reach the contra-lateral corticospinal tract and the deeper part of the funiculus, in the cervical cord. However, a small band of HRP positive fibers was noticed to reach the ipsilateral corticospinal tract without crossing at the pyramidal decussation. This result indicates that the developing pyramidal neurons in the right hemisphere carry the plastisity even after suffering from considerable hypoxia.

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Early single photon emission computed tomography (SPECT) using N-isopropyl-p-[¹²³I] iodoamphetamine (123 I-IMP) was performed in seventy-one patients with epilepsy admitted to Kanagawa Rehabilitation Hospital from July 1987 to February 1989 epilepsy and epileptic syndrome 27, encephalitis/encephalopathy 10, clinical Reye syndrome 3, cerebro-vascular disorders 8, sequelae of head trauma 4, cerebral palsy 5, brain anomaly 4, others 10.
We classified these cases according to the 1989 criteria of the International League Against Epilepsy, and compared with the findings of the SPECT studies with the EEG and CT. In idiopathic epilepsy, the SPECT findings were within normal limits. Abnormal foci on EEG were not correlated with low uptake areas on SPECT. In the symptomatic epilepsy, especially in the West and Lennox syndromes, SPECT showed a decrease in diffuse cerebral cortical blood flow during ACTH therapy. This was also seen in some cases with normal CT imaging. The results suggest that the hemodynamic pathophysiology of the brain and the indications of therapeutic effectiveness in epilepsy are shown more accurately by SPECT than CT.

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We studied the values of the Pourcelot's index of resistance in the anterior cerebral artery (RI-ACA) and basilar artery (RI-BA) in very low birth weight infants weighing less than 1, 500 g at birth. At the time of measurements, their postconceptional ages were 32, 34, 36, 38, 40 and 42 weeks. The mean value of RI-ACA (0.744±0.026, ±SD) was significantly lower than that of RI-BA (0.766±0.026) (p<0.001), and they were higher than those in normal term infants. However, the mean values of RI-ACA and RI-BA, averaged from the measurements of postconceptional ages at 38, 40 and 42 weeks, showed no significant difference from those of normal term infants. In contrast, the mean value of RI ratio (=RI-ACA/RI-BA) in very low birth weight infants showed no significant difference from that of normal term infants.

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Magnetic resonance imaging (MRI) was performed in 270 patients with various neurologic complaints (1-15 Y) with a 0.5 tesla superconducting imaging system (MRT-50 A, Toshiba Co.) using a field echo sequence (TRITE: 300 ms/14 ms) and a spine echo sequence (TR/TE: 2, 000 ms/100 ms or 2, 000 ms/120 ms, and 2, 000 ms/30 ms). The slice thickness was 10 mm. Hyperintensity areas on T2-weighted images were noted at the occipital lobe in 33 patients (12.2%). Twenty-seven of them had hyperintensity within the deep white matter, which revealed iso- or hypointensity on Ti-weighted images. The diagnosis for the 27 patients included medulloblastoma after multidisciplinary therapy (1), congenital heart disease (1), neurofibromatosis (1), tuberous sclerosis (1), congenital muscular dystrophy (1), congenital myotonic dystrophy (2), febrile convulsion (2), autism (3), epilepsy (9) and unknown causes (6). Because the hyperintensity areas are age-dependent, they may result from delayed myelination in the central nervous system.

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In the series of our studies of positron emission tomography (PET), we had some cases whose cerebral blood flow was reduced in the cerebrum, cerebellum and brain stem, and was preserved only in the basal ganglia region. We studied their clinical features and electrophysiological findings of these cases. These 5 cases included neuronal ceroid lipofuscinosis, Krabbe disease, Tay-Sachs disease, progressive myoclonus epilepsy and subacute sclerosing panencephalitis. Clinically they showed symptoms associated with diffuse cerebral and brain stem involvements. Electrophysiological studies also revealed the involvements of cerebrum and brain stem. These 5 cases were classified to persistent vegetative state clinically. Vegetative state was considered to be heterogenous concerning about cerebral metabolism. There may be one group presenting a peculiar cerebral metabolic condition described here in vegetative states. And this condition may be specific to some neurodegenerative or metabolic disorders that involve cerebellum and brain stem as well as cerebrum.

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We investigated the incidence rates of several neurological diseases in childhood in a suburban Tokyo area with a total population of about 130, 000. The number of liveborn babies during 1985-1989 was 6, 772. The number of patients with cerebral palsy (CP) was 13 and the incidence rate was 1.9/1, 000, which is equal to the results of reports from several countries. Patients with severe mental and motor retardation (SMMR) was 7 and the incidence rate was 1.0/1, 000. Five out of the 7 children with SMMR always needed medical care and were always or very frequently hospitalized. Prenatal brain damage played a major role in the pathogenesis of CP and SMMR. The number of patients with Down syndrome (DS) was 9 and the incidence rate was 1.3/1, 000. The number of patients with mental retardation (MR) except DS was 64 and the incidence rate was 11.6/1, 000. We conclude that the incidence rates of CP, DS, SMMR, and MR still remain high and that we need further strategy to prevent pediatric neurological diseases.

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We experienced two cases of myocarditis occurring during ACTH therapy for infantile spasms. Myocarditis cases occurred during the reduction of ACTH doses, and viral infection was suspected. Steroid replacement therapy was effective in the both cases. It is important to consider that ACTH therapy for infantile spasms could sometimes lead to severe myocarditis.

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Two cases of ictal automatisms of frontal lobe origin are reported. Both two cases had automatisms characterized by abrupt onset and ending, short duration, preservation of consciousness and frequent attacks in cluster. The ictal manifestations included rocking of body and violent thrashing of limbs in prone position with loud screaming. No initial staring or postictal confusion was noted. Interictal EEGs showed spikes in the frontal area. Ictal EEGs began with attenuation of background activity and/or fast rhythmic waves dominant in the frontal area. Just after clinical seizures, high voltage slow waves in bilateral frontal areas and rhythmic alpha waves in the occipital areas were recorded. The EEG findings suggested that the paroxysmal activities were localized in bilateral frontal lobes, not spreading to the temporal lobe.

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A 10-year-old girl had an infarction in the left brain during an acute viral meningoencephalitis. She initially showed seizure, unconsciousness and fever, and right hemiplegia gradually developed. She died at the 48th day of disease from respiratory disturbance and renal failure. Cranial MRI during the acute phase of the disease, when there was no clinical sign of right hemiplegia, showed a high intensity lesion in the left parietal and occipital areas on T-2 weight image. Autopsy disclosed the findings suggesting viral encephalitis, including multiple focal necrosis, perivascular round cell infiltration, proliferation of glial cells and spongy degeneration with some intranuclear inclusion bodies, and infarction in the left hemisphere. These findings suggest that T-2 high intensity lesion on MRI reflected infarction.

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Three patients with hyponatremia were found at an residential home of mental developmental delay. Because pimozide had been administered to all of them, it was suggested that pimozide might have induced compulsive water drinking resulting in hyponatremia. To my knowledge, there has been no previous report that pimozide may induce hyponatremia.
As children with mental developmental delay and/or autism frequently develop epilepsy, hyponatremia should be included in the differential diagnosis of convulsive seizures. Particularly when antipsychotic drugs such as pimozide have been given, we should pay attention to polydipsia, polyuria and/or general malaise and prevent hyponatremia.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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