Recent advance in experimental teratology has induced a rapid progress in clinical studies of the congenital malformations of central nervous system, enabling those diagnosis and prognosis to determine more accurately by using the various methods of neuroradiological investigation, such as pneumoencephalography, carotid angiography, together with brain scanning.
The author summarized clinical signs and symptoms and neuroradiological findings of three instructive cases. In each case, both clinical and anatomo-histological studies were made as carefully and extensively as possible.
Hans Kundrat was the first to define arhinencephaly. Yakovlev pointed out in his pathoarchitectonic studies of cerebral malformation that the term “arhinencephaly” is a misnomer and he proposed the term “holotelencephaly”. However more inclusive term “holoprosencephaly” has recently been suggested by DeMyer & Zeman. The author prefers the last mentioned term, considering his own results. The causation of holoprosencephaly is disccused.
