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Fetal globoid cell leukodystrophy in one of twins

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Summary

Twins with high-risk globoid cell leukodystrophy (GLD) at the 6th gestational month were studied morphologically and enzymatically. One of them was affected and the other was probably the carrier of this disease. Central nervous system and other viscera developed normally for the gestational age in both fetuses.

The lesions of GLD were found in the fetus with marked deficiency of galactocerebrosidase, but not in the probable carrier. The lesions were located only in the considerably myelinated areas viz., the brain stem and the spinal cord. Ultrastructurally, the globoid cells in the fetal GLD seemed to originate from glial cells, probably oligodendroglia.

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Author notes

  1. R. Okeda

    Present address: Department of Pathology, Medical Research Institute, Tokyo Medical and Dental University, No. 5-45, 1-chome, Yushima, Bunkyo-ku, Tokyo, Japan

Authors and Affiliations

  1. Department of Pathology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    R. Okeda

  2. Department of Pediatrics, Faculty of Medicine, the University of Tokyo, Tokyo, Japan

    Y. Suzuki

  3. Department of Obsterics and Pediatrics, Tokyo Metropolitan Tsukiji Maternity Hospital, Tokyo, Japan

    S. Horiguchi & T. Fujii

Authors
  1. R. Okeda
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  2. Y. Suzuki
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  3. S. Horiguchi
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  4. T. Fujii
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Okeda, R., Suzuki, Y., Horiguchi, S. et al. Fetal globoid cell leukodystrophy in one of twins. Acta Neuropathol 47, 151–154 (1979). https://doi.org/10.1007/BF00717039

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  • DOI: https://doi.org/10.1007/BF00717039

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