İbrahim ÖZTOPRAK, Suat TOPAKTAŞ, Özlem Kayın YILDIZ, Şeyda ÇEVİK, Ertuğrul BOLAYIR

Foix-Chavany-Marie syndrome due to a unilateral opercular infarction

Fasiyal, farengeal, lingual ve mastikatör kasların otomatik-volanter disosiasyon ile birlikte bilateral paralizisi Foix-Chavany-Marie sendromunu (FCMS) karakterize eder. Sendrom, tipik olarak bilateral operküler bölgeleri etkileyen ardışık serebrovasküler olaylara ikincil gelişir. 75 yaşında kadın hasta akut başlangıçlı konuşma ve yutma güçlüğü nedeniyle başvurdu. Nörolojik muayenede refleks ve otomatik hareketlerin korunması ile birlikte alt kraniyal motor sinirlerin bilateral paralizisi saptandı. Beyin manyetik rezonans görüntüleme, sağ frontal operküler bölgede akut enfarkt alanı ile birlikte bilateral hemisferlerde eski subkortikal laküner enfarktlar olduğuna işaret etti. FCMS’nun bilateral operküler lezyonlara bağlı oluşmasına karşın, nadiren kontrlateral subkortikal laküner enfarktlarla birlikte unilateral operküler bir lezyon da sendromun tipik klinik özelliklerine neden olabilir.

Unilateral operküler enfarkt sonucu gelişen Foix-Chavany Marie sendromu

Bilateral paralysis of facial, pharyngeal, lingual, and masticatory muscles with automatic-voluntary dissociation characterizes Foix-Chavany-Marie syndrome (FCMS). The syndrome is a rare neurological disorder which usually develops secondary to cerebrovascular accidents affecting bilateral opercular regions, typically as a result of subsequent strokes. A 75-year-old woman presented with acute onset of inability to speak and swallow. Neurological examination demonstrated bilateral palsy of lower motor cranial nerves with preservation of reflex and automatic movements. Magnetic resonance images of the brain showed an acute right frontal opercular infarction and old subcortical lacunar infarctions in the bilateral hemispheres. Although FCMS occurs due to bilateral opercular lesions, rarely unilateral opercular lesion accompanied by contralateral subcortical lesions may cause the typical clinical features of the syndrome.

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