Syndromes of bilateral symmetrical polymicrogyria

A number of anatomicoclinical syndromes have been described in which bilateral symmetrical polymicrogyria is the underlying morphologic abnormality. We retrospectively reviewed the clinical, epileptic, and morphologic manifestations of bilateral symmetrical polymicrogyria in 21 patients to determine whether certain areas are at particular risk for these syndromes.

Clinical records and brain MR studies of 21 patients with bilateral symmetrical polymicrogyria were reviewed to confirm the presence and determine the location of polymicrogyria and to qualitatively correlate location with developmental, neurologic, and epileptic histories. The locations we found were compared with published reports of bilateral symmetrical polymicrogyria to determine whether these locations were random or whether predilections exist for certain areas.

Analysis revealed six patients with bilateral frontal polymicrogyria, nine with bilateral perisylvian polymicrogyria, one with bilateral parietal polymicrogyria, one with bilateral parasagittal parieto-occipital polymicrogyria, two with bilateral frontal polymicrogyria and bilateral perisylvian polymicrogyria, one with bilateral perisylvian and bilateral parasagittal parieto-occipital polymicrogyria, and one with bilateral perisylvian, bilateral parieto-occipital, and bilateral parasagittal parieto-occipital polymicrogyria. Symptom complexes were non-specific, but seemed additive according to the regions of brain involved.

Bilateral symmetrical polymicrogyria has a propensity to develop in specific regions of the cerebral cortex. When the regions are extensive, the areas involved often appear to be simple topological additions of those regions. These locations and the identification of several familial cases raise the possibility that genetic mechanisms influence the development of these malformations in some patients.