Schizencephaly - Reasons, Effect and Cure

Published: 02nd June 2010
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Schizencephaly, also known as split brain, is a rare condition characterized by clefts, or splits in the brain tissue. It is due to poor anatomical development of the fetal brain sometime during the first seven months of pregnancy. The exact cause of this disease is unknown.

Two Forms:

The clefts can appear on one or both sides of the brain and advance from the brain's surface to the ventricles, which are filled with fluid. There are two forms of this disease:

Open Lipped: The walls of the cleft are separated
Closed Lipped: No separation of cleft walls

The fissure in schizencephaly (an evolutionary disturbance), may simulate the boils that occur in porencephaly (problem due to superficial embryonic brain damage). They can be differentiated through MRI examination and the exact nature of brain tissue can be diagnosed.

In Schizencephaly disease, split is layered with brain tissue. Meanwhile scar tissue and white matter are evident in porencephaly.


Reasons of the disease are unknown. Embryonic brain damage through the period of 4-6 months pregnancy is believed to be a cause. Damage may occur because of:

- Infection
- Reduced blood flow resulting in cerebrovascular accident (CVA, stroke)
- EMX2 gene mutation

While the clefts may be lined with gray matter, they can be also be surrounded by dense abnormal tissue with an unusually large amount of folding, called polymicrogyria. In addition to this, there may also be heterotopias (aberrant nerve clusters) present in various areas of the brain. Polymicrogyra and heterotopias are believed to be the result of abnormal neuronal migration during the gestational period.

What are the symptoms?

The disease may manifest itself differently according to the width of split. The manifestations comprise of:

- Delay in development Small heads (microencephaly)
- Accumulation of fluid on the brain (hydeocephalus)
- Limb paralysis Hypotonia (reduced muscle tone)
- Spasticity (increased muscle tone)
- Mental retardation Seizures

How is it diagnosed?

Genetic testing is currently unavailable. Diagnosis is determined by performing an MRI or CT scan of the brain.


Presently, there is no cure, but the goal of treatment is to manage the symptoms. Treatment may include:

- Anticonvulsants
- Surgical shunt in the brain to drain the fluid
- Surgical removal of the offending brain tissue that surrounds the cleft

The grave extent of split brain may be ascertained by the number of fissures and damage to the brain. Some sufferers may undergo fits and no other manifestations. Thanks to the unknown stature of the malaise, serious patients need total external assistance till death.


Jared Wright is the webmaster of - the
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