Visual sensory and ocular motor function in children with polymicrogyria: relationship to magnetic resonance imaging.

PURPOSE: To assess visual and ocular motor function in children with polymicrogyria (PMG).

METHODS

The medical records of 15 children (0.4-4 years of age) with PMG documented by magnetic resonance imaging (MRI) and with age-corrected visual acuity measured by Teller acuity cards were reviewed retrospectively. Cortical function was assessed by pattern visually evoked potentials (VEP). Ocular motor function was assessed by video-oculography or clinical assessment. Results were compared to age-matched controls.

RESULTS

Extent of PMG involvement varied from bilateral fronto-parietal to bilateral-diffuse. Nine children had involvement of the occipital lobe. Visual acuity at presentation was normal in 5 children (≥20/40 Snellen equivalent for age) and subnormal in 10 (average 20/200 equivalent). Visual acuity was similar in children with or without involvement of the occipital lobe (P = 0.4). Follow-up visual acuity was available for 9 children; 3 improved and 6 failed to improve (5 of whom had seizures). PMG involving the occipital lobe significantly reduced VEP amplitude and signal-to-noise ratios. Three infants without visually-guided behaviors had VEP responses. All 3 children with cytomegalovirus-related PMG without retinal disease had preserved visual function despite generalized MRI abnormalities.

CONCLUSIONS

All children with PMG had recordable visual function either by visual acuity or VEP testing, however the majority did not show longitudinal improvement in acuity. Seizures may impose limits on visual acuity development. Children with cytomegalovirus-related PMG, microcephaly, and developmental delay can have normal visual acuity. Children with a recordable VEP but without visually guided behaviors may have a defect in sensorimotor transformation.