NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 24, Issue 6
Displaying 1-18 of 18 articles from this issue
  • [in Japanese]
    1992 Volume 24 Issue 6 Pages 524
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • Tadashi Kawakami, Hitoshi Yoda, Yosio Shima, Hiroshi Akamatsu
    1992 Volume 24 Issue 6 Pages 525-529
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We report the incidence of neonatal seizures in the last 10 years. Between 1981-1990, 28, 925 infants were born in the Japanese Red Cross Medical Center. Very low-birth-weight infants (<1, 500g) were excluded in this study. Seizures were identified in 67 infants (0.23%) during the first 7 days of life.
    Seizures occurred more frequently in low-birth-weight infants (1, 500-2, 499 g) than in normal-birth-weight infants (>2, 499 g). Fifty-one infants (0.19%) of 27, 276 normal-birth-weight infants and 16 (0.97%) of 1, 649 low-birth-weight infants developed seizures. The frequency of seizures was 0.25 % during the first 5 years and 0.20% during the second 5 years. In low-birth-weight group, the frequency of seizures decreased form 1.49% to 0.48% during the 5-year periods, and seizures due to post asphyxial hypoxic-ischemic encephalopathy also decreased from 0.14 % to 0.06%.
    The decreased incidence in neonatal seizures during the last 10 years was probably achieved by the progress in intervention of low-birth-weight infants in NICU and by the obstetrical prevention of intrapartum asphyxia.
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  • Hitoshi Yamamoto, Bunsei Egawa, Kumiko Horiguchi, Akiko Kaku, Kaneo Ya ...
    1992 Volume 24 Issue 6 Pages 530-535
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Cerebrospinal fluid (CSF) from 6 cases of asymptomatic infantile spasms (IS) (mean age, 6.1 months) was collected before and after treatment with adrenocorticotropic hormone (ACTH). The concentration of CSF tryptophan (TRP) metabolites was analyzed using HPLC and compared to the metabolite concentration in CSF from 10 age-matched controls (mean age, 6.7 months). Levels of CSF serotonin (5-HT), 5-hydroxyindoleacetic acid (5-HIAA), and kynurenine (KYN) at pretreatment were significantly lower in IS patients compared to controls (p<0.05). In contrast, the levels of CSF 3- hydroxykynurenine (3-OHKY) before ACTH treatment were significantly higher in IS patients than in controls (p<0.05). After the treatment, significant increases in 5-HIAA and decreases in KYN and 3-OHKY levels (p<0.05) were observed in CSF of infants whose seizures were eliminated by ACTH. These findings suggested that the presence of seizures in IS was associated with a significant decrease in serotonergic activity, or that the turnover in the direction of 3-OHKY was altered. The possibility that elimination of seizures by ACTH might be related to decreased production of kynurenine metabolites was discussed.
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  • II. Speech Development and Intelligence after the Damage of Cerebral Hemisphere under 1 Year of Age
    Naofumi Ichiba, Hirotoshi Takigawa
    1992 Volume 24 Issue 6 Pages 536-541
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    To investigate the functional plasticity of the brain in childhood, the speech development, the intelligence test and dichotic listening test were performed on 27 patients who had suffered from hemiplegia under 1 year of age. Among 13 patients with right hemiplegia, 7 to 24 years old, 11 patients showed a left ear dominance suggesting the lateralization of language in the right hemisphere. All 14 patients with left hemiplegia, 5 to 37 years old, showed a right ear dominance suggesting the lateralization of language in the left hemisphere. All 27 patients acquired speech function enough to converse with other people during daily life. There were no differences in speech development or intelligence scores between both groups of hemiplegia. Although there was no correlation between the speech development and the age of onset of hemiplegia, there was a correlation between the speech development and the intelligence score in both groups of hemiplegia.
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  • Ritsuko Tawa, Hiromi Matsuda, Takehiko Morimoto, Hideo Nagao, Hiroshi ...
    1992 Volume 24 Issue 6 Pages 542-547
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A three year-old boy with congenital sensory neuropathy with anhidrosis (CSNA) was described. Sural nerve biopsy specimens revealed an almost complete absence of unmyelinated fibers and a marked decrease of the density of small myelinated fibers with preservation of the density of large myelinated fibers. No evidence of active degeneration of unmyelinated or myelinated fibers was found. Skin biopsy specimens revealed the absence of nerve terminals and fibers innervating sweat glands, although sweat glands seemed to be apparently normal in their morphological findings. Therefore, it was concluded that the absence of pain and temperature sensations with preservation of touch sensation in our patient was compatible with the morphometric findings of nerve fibers of the sural nerve described. Similarly anhidrosis was concluded to be well explained by the absence of the innervation of sweat glands and the vessels around them. On the other hand, electrophysiologic studies, such as motor and sensory nerve conduction, short latency somatosensory evoked potential and auditory brainstem response, in which the function of the large myelinated fibers is presumably tested, were all normal. Therefore, the structure and function of such large myelinated fibers were spared in this case. From clinical viewpoints, electrophysiologic studies described above are useful to differentiate CSNA from other types of congenital sensory neuropathies, in which large myelinated fibers are affected.
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  • Shigeaki Inoue
    1992 Volume 24 Issue 6 Pages 548-553
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Activities of neuron-specific enolase (NSE) in the cerebrospinal fluid (CSF) were measured in 104 neonates (24 cases with CNS diseases and 80 cases without CNS diseases) aged from 0 to 27 days and 50 children without CNS diseases aged 3 months to 15 years.
    They were determined by using the radioimmunoassay of the Eiken Kit.
    The enzyme activity in neonates without CNS diseases was significantly higher than that in children. No significant correlation was found between the level of NSE activity and gestational age, birth weight or the days after birth.
    The mean level of NSE activity in the CSF of neonates with CNS diseases was significantly higher than that of neonates without CNS diseases. Those activities gradually decreased with the clinical course.
    It was considered that the appropriate cut-off level of NSE activities in CSF for anticipating CNS damage and neurological sequelae was 20.0 ng/ml.
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  • Kazuhiro Kamuro, Masumi Inagaki, Yutaka Tomita
    1992 Volume 24 Issue 6 Pages 554-558
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We studied correlation between morphological abnormalities of Chiari malformation and evoked potentials (short-latency somatosensory evoked potential [SSEP] and auditory brainstem response [ABR]). On SSEP the inter-peak latency prolongation of P3-N1 was revealed in 6 out of 8 cases with Chiari malformations. The feature of positive wave between P3 and N1 was divided into 2 groups. The tendency of the positivity between P3 and N1 was more marked in cases of prolonged P3-N1 latency and correlated with the medullary kink. On ABR the prolongation of III-V inter-peak latency was revealed in one side in 3 patients Chiari malformations with malformed pons and tegmentum.
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  • Yoshito Hirayama, Hisaharu Suzuki, Masataka Arima
    1992 Volume 24 Issue 6 Pages 559-564
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    In a study of prenatal etiologic factors for 5, 901 severely multi-disabled children (jushoji as called in Japan), 159 patients were found to have been exposed to maternal febrile illnesses in utero. Among them, 57 patients did not have any causative factors other than maternal febrile illness. In addition to severe brain dysfunction, nonspecific anomalies were found in 46.2 % of 26 children exposed to maternal hyperthermia before 4 months of gestation. The recent findings of other investigators tend to support the concept of unusual hyperthermia being teratogenic in human. Our retrospective study suggested that maternal hyperthermiamight induce severe brain damage of embryo, but no definite malformation complex could be determined.
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  • Kazuo Ejiri, Osami Okubo, Masahiko Okuni
    1992 Volume 24 Issue 6 Pages 565-570
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We studied age related correlations of mismatch negativity (MMN) auditory event-related potentials (ERPs) using odd ball paradigm in 121 normal subjects (from 6 months to 43 years) and 33 mentally retarded subjects (from 2 to 18 years). The subject was instructed to read a book in order to ignore the stimulus during the experiment, or no specific task was imposed on young child. The MMN wave form was clearly obtained in normal 6 month old subjects. As the age advanced from 6-months, the latencies of MMN progressively shortened till about 7 years, when the latency reached adult level. One-third of mentally retarded patients had prolonged MMN than that normal age-matched subjects. Reliable recording of P300 requires subject's cooperation and attentiveness, which are difficult to achieve in young children or mentally retarded patients. MMN may be utilized in children to evaluate the development of the cognitive function.
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  • Junichi Takanashi, Katsuo Sugita, Haruko Horiguchi, Mizue Iai, Hiroo N ...
    1992 Volume 24 Issue 6 Pages 571-574
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We reported longitudinal cranial MRI studies of a neonate with tuberous sclerosis who presented convulsive seizures on the first day after birth. Cortical tubers were not ditected on MRI performed at the age of 1 month, but became evident at 18 months after birth. This finding might reflect the pathological difference between the amount of myelin around the cortical tuber and other white matter lesions, which increased with age. A heterotopic islet was shown as partially stratiform appearance on MRI. This result suggests that the structure of the heterotopic islet might be heterogeneous.
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  • Katsunori Shimizu, Hideo Sugie, Hiroshi Sato, Masataka Ito, Satoshi Ts ...
    1992 Volume 24 Issue 6 Pages 575-580
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A patient was reported, who was diagnosed as having tuberous sclerosis in neonatal period. He began to have complex partial seizures at 3 weeks of age. At 27 days of age, brain CT revealed a large high density lesion from the left temporal cortex to the left posterior horn, in addition to periventricular nodules. These lesions were not enhanced after injection of contrast medium. Brain MRI demonstrated more nodular lesions, the intensity pattern of which was different form those in previously reported cases. Ictal EEG showed secondary generalization fromleft temporal focus, which was compatible with complex partial seizures. In English literature, neonatal cases of tuberous sclerosis with large parenchymal lesions on brain CT were reported by only a few authors, and only two cases of neonatal MRI findings were documented. The neonate with tuberous sclerosis should be carefully evaluated because of the atypical appearance of MRI in rare cases.
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  • Kenji Mori, Ichiro Yano, Toshiaki Hashimoto
    1992 Volume 24 Issue 6 Pages 581-586
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A pedigree of benign familial neonatal convulsions (BFNC) was reported. Seven members of two generations experienced convulsions in the neonatal period and/or in early infancy. All of these members except one had a good prognosis. One member who had infantile spasms was uneventfully delivered at 37 weeks of gestation, with a birth weight of 2, 562 g and, without trouble during pregnancy. At the age of 20 days, she began to have adversive seizures. Later, she developed complex partial seizures and infantile spasms at 1 month and 10 days of age. Interictal EEG showed hypsarrhythmia. Biochemical investigations and MRI of the head revealed no abnormality. Treatment with sodium valproate and carbamazepine succeeded in stopping the seizures and she had no seizures after 3 months. But her psychomotor development was moderately delayed at 8 months. No case with severe epilepsy such as infantile spasms has been reported in the previous literature on BFNC. From our experience, early treatment and careful follow-up are considered to be important for BFNC.
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  • Akihisa Okumura, Norihide Maeda, Masao Kito, Yoshiko Haga, Kosaburo As ...
    1992 Volume 24 Issue 6 Pages 587-590
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We reported a girl with “prolonged”cerebellar ataxia for whom steroid was effective. At the age of 9 months, she developed gait disturbance, tremor and abnormal eye movements following exanthema subitum. Her symptoms were prolonged for more than 4 months and she was admitted to our hospital. The symptoms were successfully suppressed with repeated ACTH treatment but recurred in a few weeks after cessation of the therapy. Steroid was also effective but reduction of the dosage resulted in worsening of symptoms. Immunological mechanism was suspected for her disorder because of her response to steroid and ACTH.
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  • Hitoshi Osaka, Seiji Kimura, Atsuo Nezu
    1992 Volume 24 Issue 6 Pages 591-594
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Serial imaging studies in a 16-year-old female affected by Fahr's disease accompanied with persistent vitreous artery were performed. She suffered from chronic subdural hematoma, which was re-moved at the age of 5 months. Brain CT scan performed at the age of 4 revealed calcified lesions at the corticomedullary junctions in the frontal lobes of cerebrum. Yearly repeated CT scans revealed an increasing number of the calcified lesions, which were observed at the cortico-medullary junction of cerebrum, globus pallidus, thalami and dentate nuclei of the cerebellum. Calcifications have not increased in the last 4 years. Laboratory examinations of serum and urine failed to prove any abnormalities. She also had bilateral persistent vitreous arteries. The combination of these rare two diseases may have occurred coincidentaly, but there is a possibility that the capillary dysfunction played a role in the pathogenesis of two diseases.
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  • Hisamichi Tauchi, Nozomi Sano, Takehiko Morimoto, Hideo Nagao, Hiroshi ...
    1992 Volume 24 Issue 6 Pages 595-599
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We reported the first case of typical infantile-type neuronal ceroid-lipofuscinosis (INCL) in Japan. The patient was a 1-year-old girl presenting with rapidly progressive psychomotor deterioration and blindness. Muscular hypotonia, microcephaly and myoclonic jerks became marked with the progression of her disease.
    Diminution in amplitude of EEG, VEP and ERG was prominent in the initial stage, but ABR was normal. MRI showed progressive brain atrophy. Electron microscopic examination of the biopsied skin revealed granular matrix, the specific inclusion bodies, in the epithelial cell of sweet glands. Many sea-blue histiocytes were demonstrated in her bone marrow samples.
    INCL is a common progressive encephalopathy in the Scandinavian countries, but a typical case had not yet been fully reported in Japan. This prompted us to report our case. Future reports are need for the study of INCL in Japan.
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  • [in Japanese], [in Japanese], [in Japanese], [in Japanese]
    1992 Volume 24 Issue 6 Pages 600-602
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • [in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...
    1992 Volume 24 Issue 6 Pages 602-605
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
  • [in Japanese], [in Japanese], [in Japanese], [in Japanese]
    1992 Volume 24 Issue 6 Pages 605-606
    Published: November 01, 1992
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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