[Bilateral anterior operculum syndrome]

Abstract

Characteristically, patients with anterior operculum syndrome (AOS), also known as Marie-Foix-Chavany syndrome cannot perform voluntary movements of the face, jaw, tongue, and pharynx. As a result, they are unable to speak, swallow, grimace, smile, or carry out any other voluntary facial masticatory or linguopharyngeal movement. At the same time, involuntary movements of the aforementioned muscles are fully preserved. Laughing, yawning, and coughing, as well as mimicking movements accompanying emotions, eye closure during sleep, or the blink reflex are unaffected. The pathoanatomic substrate of this voluntary-involuntary dissociation is a bilateral lesion of the frontoparietal operculum usually caused by ischemic strokes. The prognosis is usually poor, especially concerning the ability to speak and eat. The name Marie-Foix-Chavany for this syndrome is fully established, although the characteristic voluntary-involuntary dissociation is not mentioned in the original French publication of 1926. We present a case and discuss clinical features, pathophysiology, and differential diagnosis of AOS. In a 7-year follow-up, we observed better functional outcome than is commonly described in the literature.