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
Özet
Konjenital bilateral perisilviyan sendrom (KBPS), gelişme geriliği, değişik bilişsel bozukluklar, belirgin kortikal psödobulber semptomlar ve piramidal bulgular ile karakterize, son yıllarda tanımlanmış bir durumdur. Nöbet sık görülen bir bulgu olup görüntüleme çalışmaları karakteristiktir. Altta yatan patoloji, polimikrogiridir. Polimikrogiri, fokal veya bölgesel dağılım gösterebilir veya tüm kortikal mantoyu etkileyebilir. Kadınlar, erkeklerden daha sık etkilenmektedir. Silviyan fissürler daha vertikal seyirli olarak pariyetal loblara doğru uzanmaktadır. Anomali sıklıkla simetriktir. Bu yazıda, KBPS’li bir olguyu klinik ve radyolojik özelliklerini tartışarak ortaya koyuyoruz.
Anahtar sözcükler: Perisilviyan polimikrogiri, gelişimsel anomaliler, manyetik rezonans görüntüleme
Keywords
References
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Details
Primary Language
English
Subjects
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Journal Section
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Publication Date
March 11, 2010
Submission Date
April 9, 2009
Acceptance Date
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Published in Issue
Year 2010 Volume: 32 Number: 1