Hemihydranencephaly is a severe brain condition characterized by complete or almost complete absence of cerebral cortex

Cerebral cortex

The cerebral cortex is a sheet of neural tissue that is outermost to the cerebrum of the mammalian brain. It plays a key role in memory, attention, perceptual awareness, thought, language, and consciousness. It is constituted of up to six horizontal layers, each of which has a different...

 with preservation of meninges

Meninges

The meninges is the system of membranes which envelopes the central nervous system. The meninges consist of three layers: the dura mater, the arachnoid mater, and the pia mater. The primary function of the meninges and of the cerebrospinal fluid is to protect the central nervous system.-Dura...

, basal ganglia

Basal ganglia

The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...

, pons

Pons

The pons is a structure located on the brain stem, named after the Latin word for "bridge" or the 16th-century Italian anatomist and surgeon Costanzo Varolio . It is superior to the medulla oblongata, inferior to the midbrain, and ventral to the cerebellum. In humans and other bipeds this means it...

, medulla

Medulla

Medulla refers to the middle of something and derives from the Latin word for marrow. Its anatomical uses include:* Medulla oblongata, a part of the brain stem* Renal medulla, a part of the kidney* Adrenal medulla, a part of the adrenal gland...

, cerebellum

Cerebellum

The cerebellum is a region of the brain that plays an important role in motor control. It may also be involved in some cognitive functions such as attention and language, and in regulating fear and pleasure responses, but its movement-related functions are the most solidly established...

, and falx

Falx

Falx is a Latin word originally meaning sickle, but was later used to mean any of a number of tools that had a curved blade that was sharp on the inside edge such as a scythe...

. It is a special type of Hydranencephaly

Hydranencephaly

Hydranencephaly, synonym hydroanencephaly, is a type of cephalic disorder.These disorders are congenital conditions that derive from either damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero...

It is a very rare disease. As it stands, only 7 cases have been reported.

References:

Greco F, Finocchiaro M, Pavone P, Trifiletti RR, Parano E. Hemihydranencephaly: case report and literature review. J Child Neurol. 2001;16 :218 –221