Congenital bilateral perisylvian syndrome as a rare clinicoradiological entity: a case report

Abstract

Abstract Congenital bilateral perisylvian syndrome (CBPS) is a recently described syndrome that includes developmental delay, variable cognitive deficits, prominent cortical pseudobulbar symptoms, and variable pyramidal signs. Seizures are common, and imaging studies are characteristic examinations. The underlying pathology is polymicrogyria. Polymicrogyria may have a focal or regional distribution or involve the whole cortical mantle. Females are affected more often than males. The sylvian fissures often extend more vertically at their posterior extent into the parietal lobes. The abnormality is usually symmetric. In this paper, we present a case of CBPS, and discuss the clinical and radiologic characteristics of this rare condition. Keywords: Perisylvian polymicrogyria, developmental abnormalities, magnetic resonance imaging Ozet Konjenital bilateral perisilviyan sendrom (KBPS), gelisme geriligi, degisik bilissel bozukluklar, belirgin kortikal psodobulber semptomlar ve piramidal bulgular ile karakterize, son yillarda tanimlanmis bir durumdur. Nobet sik gorulen bir bulgu olup goruntuleme calismalari karakteristiktir. Altta yatan patoloji, polimikrogiridir. Polimikrogiri, fokal veya bolgesel dagilim gosterebilir veya tum kortikal mantoyu etkileyebilir. Kadinlar, erkeklerden daha sik etkilenmektedir. Silviyan fissurler daha vertikal seyirli olarak pariyetal loblara dogru uzanmaktadir. Anomali siklikla simetriktir. Bu yazida, KBPS’li bir olguyu klinik ve radyolojik ozelliklerini tartisarak ortaya koyuyoruz. Anahtar sozcukler: Perisilviyan polimikrogiri, gelisimsel anomaliler, manyetik rezonans goruntuleme