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KMID : 0357919820160020262
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Korean Journal of Pathology
1982 Volume.16 No. 2 p.262 ~ p.265
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Holotelencephaly, Special Variant (an autopsy case)
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임인숙/In Sook Lim
이정희/고일향/지제근/Jung Hee Lee/Il Hyang Ko/Je Keun Ji
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Abstract
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Holotelencephaly is a congenital malformation of the telencephalon which is
characterized by developmental failure of the cerebral hemispheres. It is frequently
associated with midline facial defect. There are rare cases of holotelencephaly not
associated with midline facial defect.
We have experienced an autopsy case of atypical form of holotelencephaly
characterized by partial fusion of cerebral hemispheres at the parietal region with
developmental failure of the corresponding lateral ventricle. On the other band,
interhemispheric fissure of the frontal and occipital regions was well developed as well
as lateral ventricle of the corresponding regions. No facial anomaly ft, as present in this
case.
The above findings do not seem to coincide with reported cases of holotelencephaly. It
is postulated that this is an unusual Variant of holotelencephaly because of the
developmental correlation of the cerebral hemisphere and lateral ventricle.
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