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Volume 26, Issue 6
Displaying 1-12 of 12 articles from this issue
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[in Japanese]1994 Volume 26 Issue 6 Pages 474
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (114K) -
Akihisa Okumura, Fumio Hayakawa, Kuniyoshi Kuno, Jun Natsume, Kazuyosh ...1994 Volume 26 Issue 6 Pages 475-479
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSBrain CT scan was performed at 40 weeks of conceptional age in 17 preterm infants with periventricular leukomalacia (PVL). The finding of periventricular low density was not useful in differentiating patients with PVL from normal infants, because this finding was seen in 40% of normal infants. The following findings were characteristic of PVL:(1) a marked low density area in centrum semiovale, (2) an irregular outline of ventricular wall, and (3) low density spots in periventricular white matter. The findings of ventricular dilation with irregular wall and marked low density area in centrum semiovale were correlated with a finding of volume loss on MRI during late infancy and the severity of neurological impairment, especially in severely affected patients. Marked low density area in centrum semiovale was characteristic of severe PVL demonstrated on brain CT scan.View full abstractDownload PDF (4758K) -
Toshiaki Hashimoto, Masanobu Tayama, Kazuyoshi Murakawa, Masahito Miya ...1994 Volume 26 Issue 6 Pages 480-485
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSStudies of magnetic resonance images have revealed morphological disorders of the brainstem and cerebellum in autistic children and adults. When we studied development of the brainstem and cerebellum in autistic patients, these structures were significantly smaller in autistic patients than in controls. Although the brainstem and cerebellum significantly increased in size with age in both groups, the development of the pons, cerebellar vermis I - V and cerebellar vermis VI - VII was significantly more rapid in autistic patients than in controls. However, the development of the other brain structures in the posterior fossa did not differ between autistic patients and controls. The regression intercepts of the brainstem and cerebellum as well as those of their components were significantly smaller in autistic patients than in controls. These results suggested that significant anatomical changes took place in the posterior fossa brain structures in the prenatal period in autistic children, but were not progressive.View full abstractDownload PDF (2033K) -
Seiichi Sugama, Kiyomi Atsukawa, Kaoru Kusano, Akira Akatsuka, Yukikat ...1994 Volume 26 Issue 6 Pages 486-492
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSFew cases have been reported on the structural autosomal abnormality (SAA) focusing on epilepsy excluding those of Down syndrome and Klinefelter syndrome. We investigated patients who had SAA with special reference to epilepsy.
Various types of epilepsy were observed in its severity in our cases as well as previously reported cases. There was no correlation between the degree of mental retardation, motor dysfunction, brain damage on CT scan, and severity of epilepsy. Some cases had brain dysplasia, such as agenesis of corpus callosum, pachygyria, and mega cisterna magna. No correlation was found between these brain dysplasias and severity ofView full abstractDownload PDF (786K) -
Clinical Symptoms and DiagnosisTokiko Fukuda, Hideo Sugie, Yoko Sugie, Masataka Ito, Satoshi Tsurui, ...1994 Volume 26 Issue 6 Pages 493-497
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMuscle, erythrocyte and/or leukocyte phosphorylase b kinase (PBK) activities were measured in 5 patients and in 2 families. The relation between phenotype and enzyme deficiency in clinically variable samples based on measurement of erythrocytes, leukocytes or muscle was described. Three patients showed the enzyme deficiency in liver and the disease was transmitted as an X-linked recessive trait. In these cases, our results suggested that carrier detection was more reliable with erythrocytes than leukocytes. Two patients showed enzyme deficiency in muscle, and the mode of inheritance was not clear. In these cases, muscle biopsies were necessary for diagnosis.View full abstractDownload PDF (582K) -
Evaluations of Sensitivity and SpecificityKazutaka Yamada1994 Volume 26 Issue 6 Pages 498-503
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSFor the purpose of early detection and early habilitation for developmentally handicapped children, we started medical screening for pediatric neurological diseases among children in Sodegaura City, Chiba prefecture, since 1978.
We calculated sensitivity, the rate of judging abnormal children to be abnormal, specificity, the rate of judging normal children to be normal and positive predictive values, the rate of judging abnormal children who have abnormal sign, of sitting alone at nine months of age whether an infant is MR or not.
The sensitivity was 70.0%, the specificity was 98.3% and the positive predictive value was 26.5%, all of them were high rates.
Therefore we conclude that children who can not sit alone until nine months of age are possibly mentally retarded and the capability of sitting alone or not at nine months of age is an useful screening test for the purpose of early detection of mentally retarded children.View full abstractDownload PDF (722K) -
Who Were Born between 1977 and 1985 in Kyoto City;Mariko Yuge, Yuriko Yamori, Toyoko Kanda, Ruriko Andoh, Hiroshi Fukase ...1994 Volume 26 Issue 6 Pages 504-510
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSFirst, we longitudinaly analyzed 177 cerebral-palsied children (CP) who were born in Kyoto City between 1977 and 1985. They were all visited St. Joseph Hospital for Crippled Children in Kyoto during the period 1977 to 1991 in order to investigate the relationship between the prognosis of perinatal care and the changing panorama of CP in Kyoto. The average occurrence rate of CP per 1, 000 live birth during the 9 years in Kyoto was 1.12. 177 CP were divided into 3 groups according to their birth years: 1977-1979 (47 CP); 1980-1982 (62 CP); and 1983-1985 (68 CP). Then we compared the distribution of birthweights and gestational periods among the 3 groups. We found a significant increasing trend in the percentage of CP in babies with birthweight less than 2, 000 g.
Second, we examined 162 CP; 15 born outside of Kyoto were excluded because of extraneous influences of perinatal care. 162 were divided into 3 groups according to their birth years: 1977-1979 (44); 1980-1982 (56); and 1983-1985 (62). We also compared the risk factors and prognosis according to the birthweights and gestational ages among the 3 groups. This study revealed a decreasing trend in the frequency of convulsions of full term CP, dyspnea and oxygen administration of premature CP in the neonatal period. At the same time, the motor development of CP without mental retardation had improved significantly in the later period. However, in the last 3 years the distribution of the severity of motor disturbance at 4 years of age among CP with birthweight of 1, 000-2, 000 g was either mild or severe.
This study suggests that monitoring of CP from an institution for handicapped children is useful in considering the effect of perinatal care in a restricted region.View full abstractDownload PDF (802K) -
Ryuhei Kodaka, Jiro Ono, Kenji Takai, Junko Tanaka, Toshisaburo Nagai, ...1994 Volume 26 Issue 6 Pages 511-517
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a surviving case of 6-year-old boy with pontine hematoma. He complained of headache as an initial symptom and developed progressively Foville's syndrome with impairments of the IX-XII cranial nerves. Although brainstem tumor was suspected initially using CT scan, MRI revealed the existence of hematoma in the ventromedial pons. During the first 4 months of his clinical course, Gd- DTPA did not demonstrate any enhancement in that lesion. However, hemangiomatous lesion was suspected by subsequent serial MRIs with positive Gd-DTPA enhancement. Using conservative treatment including oral corticosteroids, all the neurological deficits disappeared in several months and he did not show any recurrence of clinical signs for 3 years. It was suggested that MRI was very useful in the differential diagnosis and the followup of hematoma in the posterior fossa.View full abstractDownload PDF (6340K) -
Tetsuzo Tagawa, Kazumasa Otani, Yasuyuki Futagi, Hiroshi Arai, Soutaro ...1994 Volume 26 Issue 6 Pages 518-521
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA girl aged 1 year and 2 months with hypomelanosis of Ito was reported. She suffered from intractable epileptic seizures since the second day after birth. Charateristic bizarre hypopigmented skin lesions were seen on her left shoulder and extended in a linear fashion to the flexion side of the left upper extremity. Brain MRI revealed diffuse enlargement of the left hemisphere, dilatation of the left lateral ventricle, and neuronal migration anomalies, indicating hemimegalencephaly. The types of her seizures were secondarily generalized or unilateral initially, followed by infantile spasms at 1.5 month. Around 4 months of age, frequent partial seizures appeared. Her seizures were intractable despite vigorous anticonvulsant therapy. As patients with hypomelanosis of Ito are frequently associated with neurological abnormalities, brain MRI studies were thought to be essential in detecting central nervous system anomalies, particularly in the presence of early onset intractable seizures.View full abstractDownload PDF (4676K) -
Electrophysiological Findings with Clinical CourseYoshihiro Maegaki, Jun Tohyama, Kenzo Takeshita1994 Volume 26 Issue 6 Pages 522-527
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of early-onset and slowly progressive chronic inflammatory demyelinating polyneuropathy (CIDP) was reported. Her progressive gait disturbance began at six years of age and she developed pes cavus. At the age of 13 years, a diagnosis of CIDP was made, and oral corticosteroid therapy was started. This therapy was effective, but the disease relapsed soon. Muscular strength improved after supplementation of an immunosupressant with oral corticosteroid, following steroid pulse therapy.
On peripheral motor conduction study, M waves showed very low amplitudes and remarkably delayed onset-latencies. New units of M wave appeared and amplitudes increased soon afterinitiation of the corticosteroid therapy because of an improvement of conduction block, and durations of Mwave were prolonged. Then, in accordance with shortening of M wave latencies, some dispersed units were synchronized and durations reduced. At relapse, changes of M wave showed an inverse relationship to the changes of M wave with clinical improvement. The electrophysiological findings started improving 1 week after the therapy, while clinical improvement was detected several weeks later. We conclude that the change of M wave was a sensitive marker to evaluate the effect of the therapy in our CIDP case.View full abstractDownload PDF (675K) -
Yoshiko Tanaka, Shinichiro Hamano, Takahiro Nara, Youko Nakanishi1994 Volume 26 Issue 6 Pages 528-533
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of 1-month-old female infant with hemimegalencephaly was reported. This disorder is a rare malformation characterized by congenital hypertrophy of one hemisphere and ipsilateral ventriculomegaly. Clinical signs of the patient included a left sided macrocephaly, intractable seizures and delayed development. Ictal single photon emission computed tomography (SPECT) on the 49th day using 99mTc-HMPAO showed hyperperfusion in the left occipital and frontal lobes, when ictal EEG showed a sharp wave-burst appearing continuously in the left occipital lobe that spread to the left frontal lobe. Interictal SPECT on the 48th day demonstrated and increased tracer accumulation in the left hemisphere, especiallyin the left frontal lobe. In this case, the megalencephalic hemisphere might have an important role for epileptogenesis and its spread according to the ictal SPECT findings.View full abstractDownload PDF (7383K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1994 Volume 26 Issue 6 Pages 534-536
Published: November 01, 1994
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (3056K)
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