NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 17, Issue 6
Displaying 1-18 of 18 articles from this issue
  • [in Japanese]
    1985 Volume 17 Issue 6 Pages 484
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • Takashi Mimaki, Yasuhiro Suzuki, Tetsuzo Tagawa, Junko Tanaka, Haruo T ...
    1985 Volume 17 Issue 6 Pages 485-490
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Serum protein binding of 14C-diazepam in normal serum and the effect of anticonvulsants were investigated with the centrifugation technique. 14C-diazepam was bound by 98.1 per cent in normal human serum.
    At concentrations of 500, 750 and 1, 000μmol/l sodium valproate, the amount of the unbound 14C-diazepam was increased from 1.75±0.42%(mean±SD, n=6) to 3.51±0.71%(P<0.001), 3.96±0.19%(P<0.001) and to 4.05±0.75%(P<0.001), respectively. Further studies indicated that sodium valproate inhibited competitively serum protein binding of 14C-diazepam. At therapeutic concentrations, phenytoin, phenobarbital and carbamazepine had no effects on serum protein binding of 14C-diazepam.
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  • A Neuropsychological and Electroencephalographic Study
    Akiko Matsumoto, Tatsuo Takeuchi, Toshiyuki Kumagai, Shuji Miyazaki, K ...
    1985 Volume 17 Issue 6 Pages 491-499
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A neuropsychological and electroencephalographic study was done in a case of epileptic encephalopathy with marked dysphasia. The main lingustic disturbance was marked decrease of expressive language and impairement of auditory comprehension of colloquial language. The feature of the dysphasia resembled that of “global aphasia”. There was a significant correlation between the diffuse sharp and wave bursts in EEG and the degree of dysphasia. However, reduction of paroxysmal discharges preceded improvement of the language disorder. Ictal EEG with atypical absence was recorded, and an all-night EEG showed “electrical status epilepticus during sleep (ESES)”. It was postulated, from these data, that pathophysiology in this case involved both cortical and subcortical area, and the linguistic symptom was not merely “aphasia” nor “agnosia”, but was the result of more diffuse epileptic encephalopathy.
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  • Yoko Sugie, Michiko Sugama, Seiichi Sugama, Shinobu Kosaka, Tomio Shib ...
    1985 Volume 17 Issue 6 Pages 500-506
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Two cases (Cases 1 and 2) of hereditary motor and sensory neuropathy (HMSN) type I and two cases (Cases 3 and 4) of HMSN type II were described. All cases were male and aged from 3y5m to 11y11m with the onset in early childhood. Cases 1 and 2 were siblings whose father was also similarly affected, suggesting autosomal dominant inheritance. However, Case 2, the younger brother of Case 1, was still in the subclinical state.
    Muscle weakness and wasting were noticed below the knee, especially in the peroneal muscle group, in Cases 1, 3 and 4. Distal portions of upper extremities were also affected and sensory disturbance was noticed in Case 4. Motor conduction velocities were considerably decreased in Cases 1 and 2, mildly decreased in Case 3, and normal in Case 4. Sensory conduction velocities were mildly decreased in Cases 1, 2 and 3, and normal in Case 4. Neurogenic potentials were detected in all cases with EMG. Pathological studies including light and electron microscopic observations, fiber teasing and morphometry of sural nerve biopsy material from Cases 1, 2 and 3 were performed. A remarkable decrease of myelinated fiber density with a unimodal type of histogram and segmental de/remyelination were found in both Cases 1 and 2. The small axons surrounded by excessively thick myelin sheath as the main pathalogical change, and myelinated fiber diameter analysis revealed a unimodal pattern with loss of large myelinated fibers. Ouvrier et al. (1981) reported 11 cases of HMSN which did not fall into Dyck's classification because of the rapidly progressive clinical course. In our series, Cases 3 and espescially 4 had the characteristics similar to those in cases reported by Ouvrier. Careful follow-up observations of those two cases would be necessary for further evaluation.
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  • Manabu Kanayama, Naruji Sugiyama, Hideko Morishita, Tatsuya Ishikawa, ...
    1985 Volume 17 Issue 6 Pages 507-513
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Reye-like syndrome and fatal hepatic damage were recently reported in epileptic patients treated with valproate (VPA). VPA (dipropylacetate) is an organic acid, and we postulated that VPA might affect mitochondrial function in the patients taking VPA. Therefore, we studied blood levels of ammonia, carnitine, lactate, pyruvate, amino acids, 3-hydroxybutyric acid and free fatty acids in epileptic patients on VPA, whose mean age was 9.7 years (range 2 to 17 years).
    The subjects were divided into 3 groups; 9 patients taking VPA alone (group A), 11 patients on VPA and other antiepileptic drugs (group B) and 18 patients on antiepileptic drugs other than VPA (controls). In spite of the low therapeutic doses of VPA in group A (16.2±3.8 mg/kg/day) and in group B (19.7±6.5mg/kg/day), the levels of lactate, pyruvate and alanine were significantly elevated in both groups A and B. Additionally, a decrease in carnitine concentration and an increase in ammonia concentration were also significant in group B. These results suggested that VPA had possible adverse effects on mitochondrial function, especially in cases of polypharmacy.
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  • I. The Distribution of Acute Brain Damage
    Shigeru Iino
    1985 Volume 17 Issue 6 Pages 514-524
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    One-day-old mice, Jcl: ICR strain, were exposed to 5% oxygen for hours, 34% of animals survived and 59% of the surviving mice had intracranial hemorrhage. Intracranial hemorrhage could be detected without sacrifice of the animals: it could be seen through the thin skin and skull of the newborn mice. Surviving mice were sacrificed 6 hours after the exposure to hypoxia and their brains were examined pathologically.
    Cortical hemorrhage usually appeared symmetrically in the bilateral parietal regions. In more affected cases it extended into the frontal and occipital regions. The distribution of such hemorrhagic lesions was similar to that of infarcted lesions of parasagittal cerebral injury in human neonates with hypoxic-ischemic encephalopathy. Hemorrhage and neuronal alternation were also found in deeper structures of the brain and the extent of cortical hemorrhage corresponded considerably to the severity of the whole brain damage. The pathogenesis of the cortical lesions was discussed.
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  • II. Long-term Prognosis of Partially Asphyxiated Mouse
    Shigeru Iino
    1985 Volume 17 Issue 6 Pages 525-533
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Long-term prognosis of the mice exposed to 5% O2 for 8 hours in the neonatal period was investigated. The mortaliy rate increased in proportion to the extent of intracerebral hemorrhage in the neonatal period. The mice with extensive intracerebral hemorrhage showed marked growth failure and the weight of the cerebrum examined at 90 days was significantly reduced as compared with that in controls. The brains of those mice were microcephalic, and some of them exhibited porencephaly or multicystic encephalomalacia.
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  • I. An Autoradiographic Study
    Akira Yoshida
    1985 Volume 17 Issue 6 Pages 534-547
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The brain of M1-5, M8-12, M1-10, M11-20, M1-20 and control mice were removed at 20 and 60 days and weighed. At 10 and 14 days the proliferation kinetics of the cerebellar granular cells was investigated in M1-10, M1-20 and control mice with 3H-thymidine autoradiography (the labeled mitoses method). On midsagittal sections of 30-day-old mouse cerebelli, in which granule cell production had already been ceased, the area of the granular layer was measured and granule cell were counted in unit areas and multiplied by the total area. In M1-20 the increase in brain weight was poor throughout the course. The generation time of the external granular cells was prolonged both at 10 and 14 days, and the ultimate number of the granule cells generated was significantly reduced (77%). In M1-5 the brain weight in adulthood and the granule cell number were almost the same as in controls, although the increase in brain weight was temporarily stunted (79%). In M1-10 the brain weight and the granule cell number were not completely restored, though the generation time of the external granular cells, which had been prolonged at 10 days, reverted to normal at 14 days. When the brain weight was compared between M1-5 and M8-12, and between M1-10 and M11-20 at 60 days, it was less in M8-12 than in M1-5, and likewise less in MM11-20 than These data suggest that early malnutrition may result in deficiency in brain growth in spite of the plasticity of immature brain and that even in the suckling period the time as well as the duration of the malnutrition is important factor for the subsequent brain growth.
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  • II. Golgi-Cox Study
    Akira Yoshida
    1985 Volume 17 Issue 6 Pages 548-557
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    This study was undertaken to investigate the effects of early malnutrition and subsequent rehabilitation on dendritic development of the mouse cerebral cortex with the quantitative Golgi-Cox method.
    At the 60 days of life the number of dendrite crossings of pyramidal cells was reduced in M1-20 group (mice nursed in litters of 18 from day 1 to 20). Dendritic development in M1-10 and M11-20 groups was better than in M1-20, but it was still insufficient. While the dendritic development in M1-5 group was almostthe same as that in controls, dendritic development in M8-12 group was not sufficient.
    The number of spines on apical dendrites of the pyramidal cells up to 75μm was reduced in M1-20 group at 20 days but recovered to normal at 60 days of life.
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  • I. Brain Damages Caused by Two Different Types of Total Asphyxia
    Katsuhiko Fujiwara
    1985 Volume 17 Issue 6 Pages 558-564
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Effects on mortality, morphological changes, proliferation kinetics of cerebellar neurons and ECG were compared between two types of total asphyxia in newborn mice which was produced by either CO2 or N2 breathing.
    Mortality was significantly higher in the CO2 group than in the N2 group. The neuropathological study of N2-exposed mice showed neuronal degenerations in the cerebral cortex, striatum and colliculus posterior. In contrast to the prolongation of the generation time in CO2-exposed mice, the cell-cycle study using 3H-thymidine autoradiography revealed no change in cell proliferation kinetics in the cerebellum of N2-exposed mice. ECG changes were severer in the CO2 group than in the N2 group.
    These results may suggest that brain damage is more serious in the CO2 group than in the N2 group and also that changes in cell proliferation seen in the CO2 group are caused by metabolic changes rather than hypoxemia per se.
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  • Toshihide Ishibashi, Masutomo Miyao, Mariko Momoi, Shigehiko Kamoshita ...
    1985 Volume 17 Issue 6 Pages 565-570
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A case of a 2-year-old girl with nemaline myopathy presenting severe respiratory failure was reported. The patient was the product of 38-week-uncomplicated pregnancy with a birth weight of 3, 560 g. Tube feeding was necessary due to poor feeding and transient cyanosis for the first 8 weeks. Her developmental milestones were slightly delayed including sittng at 7 months and walking alone at 20 months. Signs of respiratory failure were first noticed as an episode of lethargy and marked muscle weakness which developed within a few days before admission at the age of 2 years and 6 months.
    The physical examination revealed generalized hypotonia, myopathic face, high-arched palate and pectus carinatum. Generalized muscle atrophy was present, predominantly in thoracic muscles, and deep tendon reflexes were not detectable. Electrocardiogram showed probable right atrial and left ventricular hypertrophy. Her respiration was weak, and blood gas analysis showed severe hypoxia and hypercapnia. All other biochemical data on the serum were unremarkable including normal levels of creatine kinase and aldolase. Biopsy of the brachial biceps muscle was performed, and many nemaline rods were found in muscle fibers. Type 1 fibers were predominant and selectively atrophic. She required mechanical ventilation because of her severe respiratory failure.
    Nemaline myopathy has been considered to be a relatively benign congenital myopathy. However, the development of severe respiratory failure in early childhood as seen in our case suggests the presence of a rapidly progressive form which is distinct from the non-progressive type.
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  • Shinichi Hirabayashi, Hitoshi Kanda, Takahisa Tsuno, Taro Akabane, Kim ...
    1985 Volume 17 Issue 6 Pages 571-576
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We report a 16-year-old boy with Wilson disease who relapsed suddenly with generalized dystonia, which was improved by a high dosage of trihexyphenidyl. He was diagnosed as having Wilson disease when he developed a hemolytic crisis at age 6. When he was 14, gait disturbance, dysgraphia and dysarthria appeared. These were improved by an increase in D-penicillamine dosage only. He was well until he deteriorated acutely soon after suffering acute respiratory infection.
    On admission, he could neither walk, speak nor swallow, and showed violent involuntary movement and right hemiplegia. Hepatic functions, however, were normal. Dystonia and anarthria were persistent, although involuntary movement and hemiplegia gradually improved. Surface EMG showed the characteristic dystonic pattern.
    A few weeks after treatment with trihexyphenidyl at 10 mg daily was started, he became able to stand, walk and write. When the medication was discontinued to ascertain its effects, he became obviously worse and could hardly walk or write. Psychiatric symptoms including irritability and emotional instability were noticed as adverse effects, but they were not severe enough to stop the medication. Seven months after treatment began, the dystonia tended to fluctuate, increasing in the morning and decreasing in the evening, with no corresponding diurnal changes in homovanillic acid and dopamine levels in the CSF.
    Fahn (1979) reported that the treatment of dystonia of various types with a high dosage of trihexyphenidyl was especially effective in childhood. In the present case, we too experienced a beneficial effect with trihexyphenidyl at the dosage of 10-12 mg daily without any severe adverse effects.
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  • Yoshiaki Hirata, Akimichi Ishikawa, Kyoichi Somiya
    1985 Volume 17 Issue 6 Pages 577-582
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A female infant started having palpebral myoclonia on each feeding time at 16 hours and a generalized clonic convulsion at 48 hours after birth. They were controlled by i. v. PB-Na following by oral maintenance of PB 5 mg/kg/day. But emprosthotonic spasms with series formation appeared at 42 days of age. They occurred both at sleep and waking states. Her head was asymmetric and linear pigmentation was seen on the right side of her neck and the center of her forehead. These findings were compatible with the Linear Nevus Sebaceous syndrome. A CT scan revealed left hemiatrophy of the brain. The EEG taken after the initiation of tonic spasms revealed a typical suppression-burst pattern. Treatment with clonazepam, acetazolamide, sodium valproate and a high dose of vitamin Bs were tried but without success.
    The two courses of ACTH-Z injection were tried, but the effectiveness was transient. The tonic spasms and tonic seizures have continued several times a day.
    The EEG taken at one and nine months of age showed 1.5 to 3 cps slow spike and wave bursts and rapid rhythms during stage 2 and 3 of sleep. It suggested the change to Lennox-Gastaut syndrome.
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  • Junichi Arai, Shota Miyake, Michiko Hayashi, Hiroko Iwamoto, Nobuko Mi ...
    1985 Volume 17 Issue 6 Pages 583-587
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The patient, a 15-year-old boy of normal intelligence, developed muscle stiffness in early childhood, with or without weakness, after physical exercises especially in the cold weather. This stiffness occurred in extremities, jaws, and the tongue, and was aggravated by physical exercises (paradoxical myotonia). Sometimes he experienced a generalized flaccid palsy immediately after violent exercises in the cold weather, but never at rest. The family history revealed 19 members with similar symptoms in 4 generations, and suggested the mode of autosomal dominant inheritance in this disease.
    General physical examinations were negative. He was no neurological abnormality except for marked grip myotonia and eyelid myotonia. Slight percussion myotonia was elicited in the tongue and the deltoid muscle.
    Laboratory examinations revealed elevated serum creatine phosphokinase (810 IU/I; normal range 0-150). Other laboratory data were all normal, including complete blood counts, blood chemistry, urinalysis, T3 and T4.
    Administration of KCl (5g) resulted in an increase of plasma potassium concentration (from 3.5 to 4.5 mEq/l), and aggravation of the muscle stiffness and weakness was observed. EMG showed myotonic discharges on needle insertion and normal motor unit potentials at room temperature, but the muscle became contracted and electrically silent after the hand had been immersed in the water for 12 minutes.
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  • [in Japanese], [in Japanese], [in Japanese]
    1985 Volume 17 Issue 6 Pages 588-590
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
  • [in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...
    1985 Volume 17 Issue 6 Pages 590-592
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
  • [in Japanese], [in Japanese], [in Japanese]
    1985 Volume 17 Issue 6 Pages 595-596
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • [in Japanese]
    1985 Volume 17 Issue 6 Pages 597-599
    Published: 1985
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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