Hemimegalencephaly

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What is hemimegalencephaly? What clinical manifestations are commonly associated with hemimegalencephaly? 

Hemimegalencephaly is a rare brain malformation associated with cortical overgrowth on one hemisphere.

It is commonly associated with intractable partial seizures, significant developmental delay, and progressive hemiparesis.

Several genetic causes have been discovered including PIK3CA, PIK3R2, AKT3, and MTOR. 

Surgical removal of the malformed hemisphere (hemispherectomy) may be necessary to achieve seizure control.

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