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).

DTI-based tractography of the arcuate fasciculus in patients with polymicrogyria and language disorders

“…In some cases, there is no direct correlation of the extension of the visible PMG lesions and the involvement of the AF, and so DTI gives additional information that may not be appreciated solely with conventional MR images. Our results are in accordance with other few case reports that showed severe disruption of the AF in patients with congenital PMG [11][12][13].…”

“…Bernal et al first reported two PMG patients with delayed speech development, poor prosody and impaired articulation who showed bilateral agenetic or rudimentary AF [11]. Another group has retrospectively reviewed DTI examinations in a small group of PMG patients that were evaluated with distinct scanners and protocols.…”

The Arcuate Fasciculus and Language Development in a Cohort of Pediatric Patients with Malformations of Cortical Development

“…Although it has not been well-studied to date, the idea that cortico-cortical connections may be abnormal in patients with MCD is consistent with a report by Munakata et al, 35 in which a case of unilateral left-sided polymicrogyria was associated with the absence of the left arcuate fasciculus. Similarly, Bernal et al 36 reported the absence of the arcuate fasciculus in 2 cases of bilateral perisylvian polymicrogyria. More recently, Saporta et al 24 suggested the functional relevance of such a finding after failing to identify the arcuate in 3 patients with congenital perisylvian syndrome and severe language dysfunction.…”

Independent contribution of individual white matter pathways to language function in pediatric epilepsy patients

“…At the population level, abnormal microstructural properties of association pathways in the left hemisphere have been previously reported both in patients with localization related epilepsy (McDonald et al, 2008; Kucukboyaci et al, 2012; Govindan et al, 2008; Kim et al, 2011) and in patients with malformations of cortical development (Bernal et al, 2010; Munakata et al, 2006). Studies regarding the relevance of these abnormalities to language in the epilepsy population are relatively rare; however, in a small series of patients with congenital bilateral perisylvian syndrome, Saporta et al observed a more severe language phenotype in patients with no identifiable arcuate fasciculus (Saporta et al, 2011).…”