Hemihydranencephaly

Hemihydranencephaly is a rare neurological condition characterized by the partial or complete absence of the cerebral cortex on one side of the brain. It is a type of Hydranencephaly, which is a more general term for conditions where the brain's cerebral hemispheres are absent to some degree and replaced by sacs filled with cerebrospinal fluid.

Symptoms

The symptoms of Hemihydranencephaly can vary greatly depending on the extent of the brain's malformation. Common symptoms may include:

• Microcephaly (abnormally small head)
• Seizures
• Developmental delay
• Intellectual disability
• Spasticity (stiff or rigid muscles)
• Feeding difficulties

Causes

The exact cause of Hemihydranencephaly is unknown. It is thought to occur due to a disruption in the blood supply to the developing brain during pregnancy. This could be caused by a variety of factors, including infection, trauma, or exposure to harmful substances.

Diagnosis

Diagnosis of Hemihydranencephaly is typically made through imaging studies such as MRI or CT scans. These can reveal the absence of brain tissue and the presence of fluid-filled sacs in its place.

Treatment

There is no cure for Hemihydranencephaly. Treatment is supportive and aims to manage symptoms. This may include medications to control seizures, physical therapy to manage spasticity, and nutritional support for feeding difficulties.

Prognosis

The prognosis for individuals with Hemihydranencephaly is generally poor, with many not surviving past infancy. However, some individuals may survive into adolescence or adulthood with appropriate supportive care.

See also

• Hydranencephaly
• Holoprosencephaly
• Porencephaly
• Schizencephaly

Hemihydranencephaly Resources
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