Perceptual sound lateralization is determined from differences in time and loudness of signals arriving separately at bilateral ears, with the time difference being the more important one. The author examined the development of sound lateralization using a self-recording device developed by Sato T, which measures the sensitivity of sound lateralization on the basis of time and loudness differences separately. Subjects were 59 children aged 2 to 18 years and 12 adults who were audiologically and neurologically normal. Sound stimulus was 500 Hz continuous band noise. (1) The testable rate was increased in children older than the age of 4, and all children over 7 years of age could be tested. (2) Children over 4 years of age could lateralize sound on the basis of time difference. Sensitivity to time differences improved rapidly, showing a steep curve, in children from the ages of 4 to 6, and then increased slowly until adulthood. (3) Sensitivity to loudness differences increased and the normal range distribution decreased with age. There is a possibility that children aged 4 can discriminate loudness difference almost as well as adults.

View full abstract

We investigated the incidence rates of three neurological diseases in childhood in the City of Higashiyamato in suburban Tokyo with a total population of about 70, 000. The number of liveborn babies during 1985-1989 was 3, 958. The number of patients with cerebral palsy (CP) was 9 and the incidence rate was 2.2/1, 000, which was equal to the reports from several countries. Four out of 9 patients with CP had mild motor handicaps and were expected to “outgrow” their handicaps. The number of patients with “severe mental and motor retardation”(SMMR) was 4 and the incidence rate was 1.0/1, 000. Prenatal brain damage played a major role in the pathogenesis of CP and SMMR. The number of patients with Down syndrome (DS) was 7 and the incidence rate was 1.8/1, 000. Incidence rates of CP, SMMR, and DS still remain high and further strategy to prevent pediatric neurological diseases is necessary.

View full abstract

Development of the cerebellar granular layer, the external granular layer (EGL) and the internal granular layer (IGL), was studied morphologically to make complete serial sections of the brain from human fetuses ranging 12 to 40 weeks' gestation (WG). To examine the chronological changes and the regional differences, we measured the thickness of the layer microscopically among five different parts of the cerebellum: anterior lobe/hemisphere (AH), anterior lobe/vermis (AV), posterior lobe/hemisphere (PH), posterior lobe/vermis (PV) and flocculus (FL).
EGL was the most superficial layer composed of densely packed undifferentiated cells. Its thickness showed little changes during the fetal period of 12-40 WG for all parts except FL where EGL was thicker than those in other parts and made a gradual attenuation with development. We noticed at least three stages in the fetal development of IGL: 1) the primary or undifferentiated stage (before 18 WG) when IGL was hardly distinguishable from the layer of immature Purkinje cells (PCL); 2) the secondary or intermediate stage (18 to 30 or 35 WG) when it was clearly visible and almost stable in thickness for all parts ; 3) the tertiary or developing stage (30 or 35 to 40 WG) when it showed a dramatic increase in thickness as the formation of cerebellar folia was proceeding. During the intermediate stage the Lamina dissecans was observed between PCL and IGL typically in PH. Regional differences were detected in a period of transition from the intermediate to the developing stage among each part: the developing stage appeared earliest in AV and FL and latest in PH.
We presume, on the basis of microscopic observations on the serial sections of the brain, that a part of premature neuroblasts in the cerebellar medulla might migrate to the internal granular layer and differentiate into mature granule cells.

View full abstract

Single photon emission computed tomography (SPECT) with N-isopropyl-p-[¹²³I]-iodoamphetamine was performed twice at different times in 18 children suffering from partial seizures to evaluate the time-course changes of the hemodynamics in the focal region. Comparison of the 2 SPECT images revealed that a decreased regional cerebral blood flow (CBF) in the first was normalized in 4 cases (22.2%) and contracted in 7 cases (38.9%). The region of decreased blood flow in the second was contralateral to that in the first in 1 case (5.6%) and unchanged in 6 cases (33.3 %). Most of the normalized and contracted cases were those in which clinical seizures disappeared and the EEG findings were normalized. In these cases which responded to treatment, the decreased regional CBF was also found to be improved. Repeated SPECT appears to be useful for evaluating the therapeutic efficacy. On the other hand, most of the unchanged cases were those in which clinical seizures were frequent or uncontrolled, or which persistently showed the abnormalities in their EEG. SPECT was considered to reflect well the conditions of the epileptic foci. These results indicate that repeated SPECT for observing the time-course changes of the regional CBF represents a useful technique with can be applied in evaluating the therapeutic efficacy or deciding the time to discontinue treatment and for pathogenetic elucidation of the epileptic foci.

View full abstract

A rat model produced by intraaortic injection of sodium iodoacetate was analyzed biochemically. Hind leg muscles obtained from iodoacetate-injected site showed a selective inhibition of the glyceraldehy de-3-phosphate dehydrogenase (G3PD) activity less than 10% of control mean. No loss of mitochondrial enzyme activity was observed. Electrical stimulation at the sciatic nerve revealed muscle contractures and this muscle showed a mild degeneration in histochemically.
This study indicates that this model will be useful to study the pathophysiology of human muscle glycogen storage diseases.

View full abstract

We reported cervical myelopathy due to atlanto-axial subluxation of athetoid quadriplegia of cerebral palsy. This lesion was present from upper cervix to lower medulla oblongata, and appeared on MRI. The changes of sSEP was useful to evaluate his spinal function. We think that pediatricians need to understand this pathophysiology, particularly for multihandicapped children.

View full abstract

We report a patient with the Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus, which was slowly progressive and an Ommaya's reservoir was set up. However, the hydrocephalus remained stable even when the shunt was removed due to infection. Generalized tonic clonic convulsions had appeared from six months after birth and were treated with valproic acid. The electroencephalogram showed hypsarrhythmia. He died at eight months of age. Autopsy revealed extensive micropolygyria of the bilateral cerebral hemispheres and hydrocephalus.
To our knowledge, it is rare for the Klippel-Trenaunay-Weber syndrome to be accompanied by congenital hydrocephalus, and there has been no previous report of its occurrence with micropolygyria.

View full abstract

A 13- year- old girl with Fahr disease (infantile form) was reported. Her parents were consangineous. Her elder sister had mental retardation and spasticity of the lower limbs, and died at 23 years of age. The patient suffered from infantile spasms at 3 month. She was bed-ridden, nonverbal, microcephalic and blind. Cranial CT revealed massive calcifications in the basal ganglia, periventricular white matter, dentate nucleus and cerebellar white matter. EEG showed a suppression- burst pattern. At 13 years, she died of pneumonia and hyperammonemia.
Microscopic examination of brain showed perivascular nonarteriosclerotic ferrocalcinosis. The periventricular granules are 1-4 μ or 12 μ in diameter. This pathological change was observed only in the central nervous system above midbrain. No calcifications were found in the pituitary and the vessels of pia mater. Also a reduced ornithine transcarbamylase activity was found in the liver, which was probably not related with cerebral calcifications.
Infantile form of Fahr disease is rare and may be heterogeneous in etiology. However, clinical manifestations and pathological findings were similar to those in previous reports of Fahr disease in childhood. It is one of the disorders causing infantile spasms.

View full abstract

We reported a case of an eight-year-old boy with CEOP. His parents and sibling were in good health. There was no family history of epileptic and neurological disease. He had his first attack of unconsciousness with fixation of eye movement for a few minutes at the age of 7 years. After six months, he experienced attacks of vomiting followed by loss of consciousness and elementary visual hallucinations consisting of red and blue colors. Sometimes he complained of contraction of visual field for 10 to 20 seconds, as if a curtain had fallen following the visual hallucination of a bright light spot. At the age of eight years, he was admitted to our hospital for evaluation and therapy. Investigations included neurological examination, IQ, CT findings were normal. Visual evoked potential revealed more reduced amplitude in the left side than in the right. The EEG findings during the waking state showed continuous bilateral 1-2 c/s spike and wave complex discharges in occipital and posterior temporal areas. These discharges were immediately suppressed by eyes-opening in an illuminated room, but not in a dark room. However, during the light sleep stage, diffuse irregular spike and wave discharges appeared frequently with left side dominance. From the clinico-electrophysiological findings we diagnosed him as CEOP.
In order to investigate the changes of the occipital spike and wave discharges by photic stimulation, we administered intermittent photic stimulation (IPS) for 10 seconds at each frequency between 1-30 flashes/sec (f/s) in a dark room. These discharges were suppressed immediately after stimulation with 9-13 f/s, and there was a delay of two seconds before suppression when stimulation with 6-8 f/s was given. They were suppressed by the photic stimulation from the frontal direction but not from the lateral direction.
From the EEG and clinical findings, we conclude that the suppressing mechanism induced by photic stimulation was related to the centroencephalic system.

View full abstract

A 5-year-old girl was admitted to our hospital because of fever, vomiting, diarrhea, convulsion and disturbance of consciousness. She was diagnosed as having acute encephalopathy, which is characterized by the symmetricalow density area in the thalami on CT. Serial MRI findings revealed the bleeding with edema in the thalami, and multifocal lesions with prolonged T 1 and T 2 relaxation time in the acute phase of the illness. Multifocal lesions were no longer found two months later. The sequential changes on the images about the bleeding in the thalami were not confirmed by simultaneous CT scans. The findings of the bleeding in the thalamic lesion are consistent with those of an autopsy report previously described and indicate the vascular involvement in the thalami in acute encephalopathy, which is characterized by the symmetrical thalamic lesions with the characteristic finding of low density on CT scans.

View full abstract

Magnetic resonance imaging (MRI) findings were evaluated in two cases with moyamoya disease. A twelve-year-old girl and a five-year-old boy were admitted to our hospital with the complaints of transient ischemic attack. They were diagnosed as having moyamoya disease by cerebral angiogram. MRI clearly presented cerebral infarction by T₂-weighted imaging and multiple small round or tortuous hyposignal areas around the basal ganglia by proton density weighted imaging. These findings were consistent with moyamoya vessels shown by cerebral angiogram. Moyamoya vessels were visualized more clearly on proton density weighted imaging than on T₁-weighted imaging.
MRI is less invasive than cerebral angiogram and repeated safely. It might play an important role in a follow-up study of morphological changes on moyamoya vessels.

View full abstract

A 12-month-old girl with ocular myasthenia gravis was reported. Electromyograms (EMG) of the orbicularis oculi muscle were studied during a period of 16 months. The degree of the waning phenomenon on EMG correlated well with that of her clinical symptoms. The study of post-tetanic exhaustion after repetitive electrical stimulation at 20 Hz for 4 seconds, was the most sensitive technique in the evaluation of the function of neuromuscular junction. EMG showed the waning phenomenon in the initial worsening after a high dose methylprednisolone therapy, and at the symptom-free stage without medication after the steroid therapy.
In our patient, orbicularis oculi EMG was useful in the estimation of the severity of ocular myasthenia gravis and the effect of its treatment.

View full abstract

We reported a clinical course and autopsy results of an 8-year-old severely handicapped girl with marked periventricular leukomalacia. She was well until 3 days prior to first admission in local hospital. Two days prior to admission, she began to vomit. Twelve hours later, she was noted to be lethargic and developed malaise with frequent vomitting. At physical examination on admission, she had frequent fits and her posture was decerebrate rigidity. Consciousness disturbance continued for two weeks. Thereafter, she became severely handicapped with spastic quadriplegia, mental retardation and intractable epilepsy. She was transeferred to our hospital one monthlater. We cared her totally and carefully with our rehabilitation staff, but during her course several rare happenings occurred; she suffered from subdural hemorrhage due to hypocupremia and received an operation for the release of contracture of her hips. She died of acute cardio-respiratory failure at 8 years and 5 months of age. Her autopsy findings were characteristic of the damage to an immature brain during development; cactus formation of cerebellar cortex and periventricular leukomalacia

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract