Polymicrogyria is a common malformation of cortical development that is grossly characterized by abnormal cortical lamination and an excessive number of small gyri. The syndrome is manifested by global developmental delay of at least moderate severity, epilepsy, dysconjugate gaze, and bilateral pyramidal and cerebellar signs. Polymicrogyria can be generalized or focal, unilateral or bilateral. Among the rare, bilateral symmetrical forms several syndromes, such as bilateral frontal, frontoparietal, perisylvian, and parasagittal parieto-occipital polymicrogyria have been recognized. Severity of polymicrogyria is dependant on the location and size of the affected area.
The etiology of polymicrogyria is heterogeneous. The topographic arrangement of the lesions, the frequency of bilateral symmetry, and historical data may suggest a transient intrauterine perfusion failure in many patients. However, abnormal hemodynamic data in the fetal cerebral arteries and infectious or toxic theories fail to explain completely the distribution of all polymicrogyric lesions.