FETAL GLOBOID-CELL LEUKODYSTROPHY IN ONE OF TWINS

被引：19

作者：

OKEDA, R

SUZUKI, Y

HORIGUCHI, S

FUJII, T

机构：

[1] UNIV TOKYO,FAC MED,DEPT PEDIAT,TOKYO 113,JAPAN

[2] TOKYO METROPOLITAN KOMAGOME HOSP,DEPT PATHOL,TOKYO,JAPAN

[3] TOKYO METROPOLITAN TSUKJII MATERN HOSP,DEPT OBSTER & PEDIAT,TOKYO,JAPAN

来源：

ACTA NEUROPATHOLOGICA | 1979年 / 47卷 / 02期

关键词：

Fetal globoid cell leukodystrophy; Galactocerebrosidase; Globoid cells;

D O I：

10.1007/BF00717039

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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. © 1979 Springer-Verlag.

引用

页码：151 / 154

页数：4

共 9 条

[1]

CHRISTENSEN E, 1961, ACTA NEUROL SCAND, V37, P163

[2] FETAL GLOBOID-CELL LEUKODYSTROPHY (KRABBE DISEASE) - PATHOLOGICAL AND BIOCHEMICAL EXAMINATION [J].