脳梁欠損症および本症に合併する奇形

書誌事項

タイトル別名
  • Corpus callosum agenesis and associated malformations.

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抄録

We studied 10 patients with corpus callosum agenesis by magnetic resonance imaging (MRI) and examined the developmental quotient (DQ) or intelligence quotient (IQ) in each case. These patients were classified into two groups which were defined from the MRI findings as follows; group 1, two patients with the complete agenesis of the corpus callosum; group 2, eight patients with the partial dysgenesis of the corpus callosum on MRI. In the group 2, five cases had the defect of the body or the splenium (localized type), and three cases had the defect from the body to the splenium of the corpus callosum (unlocalized type). MRI precisely revealed the size and the shape of the corpus callosum, especially in the sagittal view, and at the same time, the associated abnormalities. The associated abnormalities were seen in 10 cases as follows; hydrocephalus (10 cases, 100%), myelomeningocele (7 cases, 70%), Arnold-Chiari malformation type 2 (8 cases, 80%), subdural effusion (1 case, 10%), holoprosencephaly (1 case, 10%), congenital atresia of the esophagus (1 case, 10%).<BR>DQ in two cases with the agenesis of the corpus callosum was 21, and 10, respectively. DQ and IQ were examined in 5 cases with the localized type and in 3 cases with unlocalized type. In the localized type, the mean scales of DQ and IQ were 69 and 75, respectively; in the unlocalized type, 65 and 80, respectively. The scales of DQ were very low in the patients with agenesis of the corpus callosum. In such cases, corpus callosum agenesis was accompanied with holoprosencephaly or anoxic brain.<BR>In conclusion, the nature and the degree of the associated malformations or lesions influenced the prognosis of the patients.

収録刊行物

  • 脳と発達

    脳と発達 21 (1), 14-18, 1989

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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