Neuroanatomic Study on Holoprosencephaly

Abstract

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 presente