Prosencephalon in the context of Development of the nervous system

In the human brain, the diencephalon (or interbrain) is a division of the forebrain (embryonic prosencephalon). It is situated between the telencephalon and the midbrain (embryonic mesencephalon). The diencephalon has also been known as the tweenbrain in older literature. It consists of structures that are on either side of the third ventricle, including the thalamus, the hypothalamus, the epithalamus and the subthalamus.

The diencephalon is one of the main vesicles of the brain formed during embryonic development. During the third week of development a neural tube is created from the ectoderm, one of the three primary germ layers, and forms three main vesicles: the prosencephalon, the mesencephalon and the rhombencephalon. The prosencephalon gradually divides into the telencephalon (the cerebrum) and the diencephalon.

Holoprosencephaly (HPE) is a cephalic disorder in which the prosencephalon (the forebrain of the embryo) fails to develop into two hemispheres, typically occurring between the 18th and 28th day of gestation. Normally, the forebrain is formed and the face begins to develop in the fifth and sixth weeks of human pregnancy. The condition also occurs in other species.

Holoprosencephaly is estimated to occur in approximately 1 in every 250 conceptions; most cases are not compatible with life and result in fetal death in utero due to deformities to the skull and brain. However, holoprosencephaly is still estimated to occur in approximately 1 in every 8,000 live births.