Schizencephaly, popularly called split brain, is an absurd situation in which brain breaks into grooves. General building of brain in the first 210 days prior to pregnancy takes a wrong route; culminating in it. Precise precondition of the disease is unknown however.
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 clefts in schizencephaly (a developmental disorder), may resemble the cysts that exist in porencephaly (disorder due to secondary fetal brain injury). One can be distinguished from the other by MRI testing to determine the type of brain tissue that lines the cleft.
In split brain disorder, the cleft is lined with brain tissue. In porencephaly, scar tissue and white matter are apparent.
Causes:
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?
Symptoms for this condition are dependent upon the the size of the clefts. Symptoms may include:
- 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?
Examination of genes is not presently possible. The disease comes to light only post MRI or CT scan of the brain.
Remedy:
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.