Dilara İÇAĞASIOĞLU, Mehmet Haydar ATALAR

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

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.

Nadir bir klinikoradyolojik durum olarak konjenital bilateral perisilviyan sendrom: Olgu sunumu

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.

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1. Kuzniecky R, Andermann F, Tampieri D, Melanson D, Olivier A, Leppik I. Bilateral central macrogyria: epilepsy, pseudobulbar palsy and mental retardation-a recognizable neuronal migration disorder. Ann Neurol 1989; 25: 547–54.
2. Kuzniecky R, Andermann F, Guerrini R. Congenital bilateral perisylvian syndrome: study of 31 patients. Lancet 1993; 341: 608–12.
3. Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric Congenital bilateral perisylvian syndrome: Clinical and MRI features in 12 Patients. Neuropediatrics 1997; 28: 198–203.
4. Barkovich AJ, Kjos BO. Nonlissencephalic cortical dysplasias: correlation of imaging findings with clinical deficits. Am J Neuroradiol. 1992; 13: 95-103.
5. Guerrini R. Polymicrogyria and epilepsy. In: Spreafico R, Avanzini G, Andermann F, eds. Abnormal cortical development and epilepsy. London: John Libbey, 1999; p.191–201.
6. Hattori H, Higuchi Y, Maihara T, Jung EY, Furusho K, Asato R. Congenital bilateral perisylvian syndrome: first report in a Japanese patient. Jpn J Hum Genet 1996; 41: 1189–92.
7. Harding B, Copp AJ. Malformations. In: Graham DI, Lantos PL (eds). Greenfield’s Neuropathology 6th edn. London, Sidney, Auckland: Arnold, 1997: p.397–533.
8. Sztriha L, Nork M. Bilateral symmetrical frontoparietal polymicrogyria. Eur J Pediatr Neurol 2002; 6: 229–32.
9. Borgatti R, Triulzi F, Zucca C, Piccinelli P, Balottin U, Carrozzo R, Guerrini R. Bilateral perisylvian polymicrogyria in three generations. Neurol 1999; 52: 1910–3.
10. Humbertclaude V, Pedespan JM, Azais M, Fontan D, Echenne B. Schizencephaly and upper limb malformation. Arch Pediatr 1996; 3: 357–9.
11. Kuker W, Friese S, Riethmuller J, Krageloh-Mann I. Congenital bilateral perisylvian syndrome (CBPS): Do concomitant oesophageal malformations indicate a poor prognosis? Neuropediatrics 2000; 31: 310–3.
12. Yekeler E, Ozmen M, Genchellac H, Dursun M, Acunas G. Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis. Pediatr Radiol 2004; 34: 908–11.
13. Titelbaum DS, Hayward JC, Zimmerman RA. Pachygyric-like changes: topographic appearance at MR imaging and CT and correlation with neurologic status. Radiology
14. Barkovich AJ, Koch T, Carrol C. The spectrum of lissencephaly; report of ten cases analyzed by magnetic resonance imaging. Ann Neurol 1991; 30: 139–46.
15. Fischbein N, Dillon WP, Barkovich J. Teaching atlas of brain imaging (1st ed). New York: Thieme, 2000.
16. Worster-Drought C. Suprabulbar paresis. Congenital suprabulbar paresis and its differential diagnosis, with special reference to acquired suprabulbar paresis. Dev Med Clin Neurol 1974; 30: 1–30.
17. Mariani C, Spinnler H, Sterzi R, Vallar G. Bilateral perisylvian softenings: bilateral anterior opercular syndrome (Foix-Chavany-Marie syndrome). J Neurol 1980; 223: 269–84.