전종뇌의 신겸해부학적 검색
Neuroanatomic Study on Holoprosencephaly

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지제근; 김순배
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서울대학교 의과대학
Seoul J Med 1984;25(3):359-374
In 1882 Kundrat grouped a number of cerebral
malformation under the designation of arhinencephaly.
In 1959, Yakovlev carefully studied
10 cases and pointed out that the term arhinencephaly
was a misnomer because parts of rhinenceph
alon were present. Subsequently,
the term holoprosencephaly has come into use
for this brain deformity.
In the past years , a number of reports have
appeared, pointing out the relationship between
the facial and cerebral manifestations, (DeMyer
et aI. , 1963: DeMyer et aI., 1964) familial
occurrence, (DeMyer et al .. 1963: Hintz et aI. ,
1968) endocrine dysgenesia, (Edmonds, 1950;
Haworth et aI. , 1961; Hintz et aI., 1968)
chromosomal abnormalities, (Godin et aI. , 1968;
Lorch et aI., 1978) bony change, (Siebert et aI. ,
1981) radiologic, (Kulander et aI. , 1966) echoen
cephalographic (Patel et aI., 1980) and computerized
tomographic changes (Derakhsh et aI. ,
1980). In this report, we are concerned about
the relationship between the facial and cerebral
manifestations and the certainty of the anteriorposterior
gradient. We also laid emphasis on
the changes of the pituitary gland and cerebelllum. And on the basis of our observations, we
reconsidered the pathogenesis of the holoprose"
ncephaly which has so far been presented
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 25 No.3 (1984)
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