Agenesis of the Arcuate Fasciculi in Congenital Bilateral Perisylvian Syndrome

Abstract

To describe the absence of the arcuate fasciculi in 2 cases of congenital bilateral perisylvian syndrome (CBPS). Case series. Pediatric referral hospital-based study. Two patients with CBPS, referred to our institution as candidates for surgical treatment of epilepsy. Intervention Diffusion tensor imaging (1.5-T scanner; 15 encoding directions; b = 800 s/mm(2)) and deterministic tractography of the main projection and association tracts. Neuropsychology evaluation; fractional anisotropy, apparent diffusion coefficients, and anatomical aspect of the tracts. Absence of the arcuate fasciculus was observed in both subjects. Ancillary findings were complete absence of the superior longitudinal fasciculi in 1 case and underdevelopment in the other. Low fractional anisotropy of the left inferior occipitofrontal fasciculus was found in both cases. The same tract was maloriented in 1 of the cases. Agenesis of the arcuate fasciculus may accompany CBPS.