/foix chavany Marie syndrome

foix chavany Marie syndrome

Rare Disease

MONDO:0023171

Also Known As

foix chavany Marie syndromeanterior opercular syndromebilateral anterior opercular syndromecongenital Foix-Chavany-Marie syndromecongenital Foix-Chavany-Marie syndrome (subtype)facio-Labio-pharyngo-Glosso-laryngo-brachial paralysisfacio-pharyngo-glossal diplegia with automatic-voluntary movement dissociationfacio-pharyngo-glosso-masticatory diplegiaopercular syndrome, anteriorpseudobulbar paralysis, cortical type

Definition

Foix-Chavany-Marie syndrome (FCMS) is a cortico-subcortical suprabulbar or pseudobulbar palsy of the lower cranial nerves, characterized by severe dysarthria and dysphagia associated with bilateral central facio-pharyngo-glosso-masticatory paralysis, with prominent automatic-voluntary dissociation in which involuntary movements of the affected muscles are preserved.