NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 29, Issue 4
Displaying 1-15 of 15 articles from this issue
  • [in Japanese]
    1997 Volume 29 Issue 4 Pages 272
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • Comparison between Institutionalized Persons and Persons Living at Home
    Nobuhiko Takano, Masaki Hasui, Kazuya Honke, Seizo Kyoutani, Ritsuko H ...
    1997 Volume 29 Issue 4 Pages 273-277
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The seroprevalence of an anti-Helicobacter pylori antibody (ELISA) was investigated in institutionalized persons with severe motor and intellectual disabilities (SMID). The rate of seropositivity was significantly higher in persons with SMID under 29 years of age than in age-matched controls and in institutionalized patients with muscular dystrophy. No difference in seropositivity among SMID patients was found between those in our and other institutions. Therefore, it is speculated that Helicobacter pylori infection occurs soon after the institutionalization of SMID patients. Persons with SMID living at home, in their teens and twenties, had lower seropositivity than that of institutionalized patients but higher than controls. These data implicate the life style of persons with SMID and long-term institutionalization as one of important risk factors of Helicobacter pylori infection.
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  • Shinjiro Shimazaki, Keiko Kuremoto, Shoichi Oyama
    1997 Volume 29 Issue 4 Pages 278-284
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We evaluated the efficacy of diazepam and chloral hydrate given rectally for the prophylaxis of recur-rence of febrile seizure. The dose were 0.4 mg/ kg for diazepam and 250 mg (for children younger than 3 years old) or 500 mg (for over 3 years old) for chloral hydrate. Another dose was given after an interval of 8 hours if body temperature continued to exceed 38.0°C. Among the 452 patients with febrile seizures who visited our hospital from Jan. 1993 to Jun. 1995, 113 were studied who had at least one febrile episode in the follow-up period that extended over 6 months.
    These patients were divided into two groups: Group D (72 patients given diazepam) and Group C (41 patients given chloral hydrate). In group D and C, the numbers of febrile episodes were 238 and 167, and those of recurrent seizures 8 (3.8%) and 29 (20.4%), respectively. The recurrences rate was significantly higher in the latter group (p<0.005). There was no statistical difference as to the mean dosage of diazepam or chloral hydrate between the patients with and without recurrence. The numbers of patients with seizure recurrence were 8 (11.1%) in group D and 12 (29.3%) in group C, being significantly larger in the latter (p<0.005). Diazepam produced more adverse effects than chloral hydrate did.
    Thus diazepam suppositories for the prevention of recurrence of febrile seizures were more effective than chloral hydrate suppositories.
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  • Akira Onuma, Yasuko Kobayashi, Kazuie Iinuma
    1997 Volume 29 Issue 4 Pages 285-290
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Among the variable manifestating conditions of neuronal migration disorders, mental retardation, motor disturbance and epilepsy are the main features of developmental disabilities. We analyzed the relationship between clinical symptoms and magnetic resonance (MR) images, including surface anatomy scan (SAS). Thirty nine patients (23 males, 16 females; mean age 6.1 years) with neuronal migration disorders were studied. The diagnoses were cerebral palsy in 23 cases, mental retardation in 4, West syndrome in 4, Fukuyama type congenital muscular dystrophy (FCMD) in 6, Walker- Warburg syndrome in 1 and Dubowitz syndrome in 1. Cortical dysplasias were classified into the following 7 groups, mainly based on the SAS findings: complete agyria (AG 1), mixture of agyria and pachygyria (AG 2), bilateral complete pachygyria (BP 1), diffuse pachygyria with marked widening of the bilateral superior frontal gyrus (BP 2), unilateral pachygyria with hemispheric atrophy or hemimegalencephaly (UP), focal cortical dysplasia (FP) and other findings such as solitary schizencephaly (Others). Most cases of AG 1 and AG 2 showed spastic quadriplegia (6/7) and symptomatic generalized epilepsy (5/7), whereas cases of BP1 showed spasticity only in 1/8 and epilepsy in 7/8. Hemiplegia was observed in 6/7 of UP, 2/8 of FP and 2/4 of Others. Partial epilepsy was observed in 2/7 of UP and 1/8 of FP. Intellectual level was variable in BP 1, UP, FP, and Others, but all cases showed severe mental retardation in AG 1, AG 2 and BP 2. BP 2 was observed in all cases of typical FCMD (5/5). The birth weight was less than 2, 500 g in 6/7 of UP. The structural findings well correlated with clinical symptoms and epileptic seizure types. The surface anatomy scan was a very useful technique for detecting cortical dysplasias.
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  • Masaru Takayanagi, Katsuya Yamamoto, Hiroshi Nakagawa, Kazuie Iinuma
    1997 Volume 29 Issue 4 Pages 291-297
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Treatment of bacterial meningitis depends on its severity. The signs, symptoms, and laboratory values of 51 patients with bacterial meningitis admitting to the Department of Pediatrics at Sendai City Hospital from January 1985 to December 1994 were analyzed in order to evaluate their prognostic value. The overall mortality rate was 3.9%. The incidence of neurological deficit on discharge was 31.4%. According to their prognoses, patients were divided into two groups those who recovered with no detectable disabilities (good prognosis) and those who died or were left with neurological deficits (poor prognosis).
    An analysis of these groups using Fisher's exact probability test revealed that the following risk factors were associated with poor prognosis: 1) duration of fever (including the periods of relapse) for more than 10 days; 2) abnormal findings on brain imaging, such as cerebral infarction, cerebral hemorrhage, cerebral abscess and subdural effusion; 3) initial serum CRP value above 16 mg/dl; 4) initial CSF glucose value below 12 mg/dl; and 5) initial CSF LDH value above 220 IU/l. Streptococcus pneumoniae infection carried the worst prognosis; the causal organism of both the two fetal cases was S. pneumoniae. The incidence of poor prognosis was also high in S. pneumoniae meningitis (60.0%), compared to those by Hemophilus influenzae (46.7%) and group B streptococcus (25.0%). In the cases in which causal agents were not detected, this incidence was as low as 10 percent, showing significant difference from cases in which causal agents were identified.
    In order to improve the prognosis of bacterial meningitis, factors associated with poor prognosis should be recognized at early stages of the illness.
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  • Katsunori Fujii, Junichi Takanashi, Yoshinori Saito, Kazuto Tamai, Iku ...
    1997 Volume 29 Issue 4 Pages 298-302
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We described a family of dentatorubral-pallidoluysian atrophy (DRPLA). The mother presented with cerebellar ataxia at 35 years of age and thereafter her neurological symptoms became exacerbated. Her daughter had mental retardation during the preschool period and epilepsy at 10 years. Her son presented with epilepsy at 14 years. Their clinical phenotypes demonstrated maternal anticipation in this family. Genetical analysis of their DNA revealed CAG repeat expansion of the DRPLA gene, the number of which was 61 (mother), 65 (her daughter), and 63 (her son). MR imaging showed disappearance of T2 shortening of the red nucleus in the mother and her daughter in contrast to the normal appearance in her son. MR imaging was effective in evaluating neuropathological changes in the DRPLA patients.
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  • Hiroshi Ozawa, Machiko Takeda, Masayuki Sasaki, Kenji Sugai, Toshiaki ...
    1997 Volume 29 Issue 4 Pages 303-309
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We reported a 13-year-old boy with juvenile Huntington disease diagnosed by DNA analysis. Symptoms started with dysarthria at 6 years of age, which was followed by progressive dysgraphia and gait disturbance due to dystonia from 7 years, and by epileptic seizures from 12 years. Magnetic resonance imaging revealed atrophy of the bilateral caudate nuclei and T2- and proton- weighted high intensity area in both putamina. The CAG (cytosine-adenine-guanine) trinucleotide repeat on chromosome 4 p16 was markedly expanded to 81. For a child with dystonia with mental deterioration, juvenile Huntington disease should be considered in the differential diagnosis.
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  • Yosuke Mori, Sono Kaneko, Toyoaki Nakayama, Masatune Date, Natsue Naka ...
    1997 Volume 29 Issue 4 Pages 310-314
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We describe two cousins with severe infantile form of myotubular myopathy. In Japan this disease has previously been reported in only three families.
    Case I. The propositus, a 2-year-5-month-old boy, had been on a respirator since birth. He had a history of severe neonatal asphyxia and sequential hypotonia with dyspnea. Findings diagnostic of congenital myotubular myopathy, such as central nuclei and peripheral halo of muscle fibers, were demonstrated in his biopsied muscle.
    Case 2. A male the cousin of case I had congenital myopathy and died at 3 months of age due to respiratory failure. His muscle biopsy disclosed the identical findings as had been seen in case 1.
    These two cases were born to twin mothers, suggesting X-linked recessive inheritance. Early diagnosis and proper treatment of myotubular myopathy are important, because this condition may be erroneouslyinterpreted as the sequelae of neonatal asphyxia.
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  • Akira Uno, Makiko Kaga, Masumi Inagaki, Masaru Mimura, Motoichiro Kato
    1997 Volume 29 Issue 4 Pages 315-320
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A learning disabled (LD) child with disorder of verbal semantic comprehension was reported. This case showed normal ability of non-verbal semantic comprehension. He was not able to understand the meaning of what he read aloud and/or repeated, which he did well. The focus of his brain dysfunction in the left temporal lobe was revealed by SPECT in spite of MRI findings of no particular lesions. Neuropsychological and cognitive-psychological findings were similar to those reported of adult sensory aphasias with localized lesions.
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  • Noriko Haruhara, Akira Uno, Satoru Hirano, Masumi Inagaki, Makiko Kaga
    1997 Volume 29 Issue 4 Pages 321-325
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We report a girl with memory disorders. Her memory remained disturbed regardless of input or output modalities. She could recognize items and recall them randomly, but she could not recall them in the correct order. She showed memory impairment with regard to temporal context. We could clearly demonstrate this by cognitive psychological and neuropsychological tests and by analyzing the results of ordinary intelligence tests by input and output modalities. These results suggest that subjects who show memory disorders should undergo further testing to clarify the qualitative aspects of these disorders and the possibility that learning disabilities caused by memory disorders should be considered a specific type of learning disability.
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  • Masahisa Satoh, Toru Watanabe
    1997 Volume 29 Issue 4 Pages 326-330
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    We reported a case of partial seizures following aseptic meningoencephalitis.
    A 2-year-10-month-old boy was admitted to our hospital because of generalized seizures with fever. He had no history of previous seizures and had been well until 8 days before admission. He had been given a 4th DPT (diphtheria, tetanus toxoid and pertussis) vaccine 9 days before admission, and had developed fever and exanthema on the trunk the following day.
    Both fever and exanthema recurred repeatedly thereafter. After admission, he suffered from generalized seizures without fever and many kinds of partial seizures with psychiatric symptom.
    Despite administration of several antiepileptic drugs, these seizures persisted for one and half months, occurring 5 to 18 times a day. Thirty- six days after admission, MRI showed multiple dark areas on T1-weighted images and bright areas on T2-weighted images in the bilateral frontal area.
    We considered these to be due to cerebral vasculitis associated with aseptic meningitis. The patient's seizures were finally controlled by zonisamide administration. At the same time, fever went down. He has since shown normal development without seizures for 18 months.
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  • [in Japanese], [in Japanese], [in Japanese]
    1997 Volume 29 Issue 4 Pages 331-333
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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  • [in Japanese], [in Japanese]
    1997 Volume 29 Issue 4 Pages 334-336
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Download PDF (2756K)
  • [in Japanese]
    1997 Volume 29 Issue 4 Pages 337-340
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Download PDF (461K)
  • [in Japanese], [in Japanese]
    1997 Volume 29 Issue 4 Pages 345-349
    Published: July 01, 1997
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
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