Foix-Chavany-Marie syndrome

Classification according to ICD-10
G12.2	Motor neuron disease
ICD-10 online (WHO version 2019)

The Foix-Chavany-Marie syndrome , also known by the synonyms bilateral anterior operculum syndrome (AOS) and faciopharyngoglossomasticatory diplegia , is a rare syndrome that is based on bilateral damage to the cerebral cortex in the central region and that leads to a dissociation of voluntary motor skills and automatic (emotional) motor skills in the facial, chewing and swallowing muscles. The patients are not able to perform voluntary movements in the muscles mentioned, while, for example, involuntary emotional and reflex movements of the same muscles are intact.

The syndrome is named after Charles Foix , Jean Alfred Émile Chavany and Julien Marie, who first described it.

Mark

The detection of cerebral cortex damage, which consists of a bilateral (bilateral) lesion of the frontoparietal opercula, is diagnostically relevant . People with Foix-Chavany-Marie syndrome can not move the facial , jaw , tongue , and pharyngeal muscles at will. As a result, they are unable to move the facial muscles (facial muscles) willingly (e.g. smile ), while involuntary muscle movements, such as those that occur when coughing or yawning , remain possible. Emotional facial expressions (e.g. when crying ) are retained and the eyes are closed while sleeping .

diagnosis

The diagnosis can be made using magnetic resonance imaging . There is a bilateral lesion of the frontoparietal opercula.

literature

• C. Foix, JAE Chavany, J. Marie: Diplégie facio-linguo-masticatrice d'origine sous-corticale sans paralysie des membres (contribution à l'étude de la localization des centres de la face du membre supérieur). In: Revue neurologique. Paris, 1926, 33, pp. 214-219. (Initial description)
• M. Weller: Anterior opercular cortex lesions cause dissociated lower cranial nerve palsies and anarthria but no aphasia: Foix-Chavany-Marie syndrome and "automatic voluntary dissociation" revisited. In: J Neurol. 1993; 240 (4), pp. 199-208. PMID 7684439

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !