Atypical Presentations of Foix-Chavany-Marie Syndrome (FCMS) in Stroke

Foix-Chavany-Marie syndrome (FCMS) presents with anarthria and bilateral (B/L) central facio-linguo-velo-pharyngo-masticatory paralysis with "autonomic voluntary dissociation." The most common cause of FCMS is cerebrovascular disease, while rarer causes include central nervous system infection, developmental disorders, epilepsy, and neurodegenerative disorders. Even though this syndrome is also referred to as (B/L) anterior operculum syndrome, patients with lesion in sites other than (B/L) opercular regions also can develop the syndrome. In this article we describe two such atypical cases. Case 1: A 66-year-old man with diabetes and hypertension who is a smoker had right-sided hemiplegia one year back developed the syndrome acutely two days before admission. CT brain showed left perisylvian infarct and right internal capsule anterior limb infarct. Case 2: A 48-year-old gentleman, who is a diabetic and hypertensive had right-sided hemiplegia one year back and developed the syndrome acutely two days before admission. CT brain showed (B/L) infarcts in the posterior limb of the internal capsule. Both patients had bifacial, lingual, and pharyngolaryngeal palsy thereby confirming the diagnosis of FCMS. None of them had the classical (B/L) opercular lesions on imaging and one patient did not even have a unilateral opercular lesion. Contrary to the common teaching, (B/L) opercular lesions are not always necessary to produce FCMS and can occur even without opercular lesions at all.