Foix–Chavany–Marie syndrome

Foix-Chavany-Marie Syndrome (FCMS), also known as Bilateral Opercular Syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach. Foix-Chavany-Marie Syndrome (FCMS), also known as Bilateral Opercular Syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach. There are two forms (also referred to as 'classifications') of FCMS; bilateral and unilateral. The bilateral form is most common (also referred to as the 'classical form') and is caused by the formation of lesions on both sides of the anterior or posterior region of the operculum. In contrast, the unilateral form is rare and is caused by the formation of lesions on one side of the anterior or posterior region of the operculum. Lesions located in the anterior regions of the operculum are associated with motor deficits and anarthria, a total absence of the ability to form speech or language. Lesions located in the posterior regions of the operculum are associated with parietal opercular functions. The two classifications of FCMS were established based on the location of the lesion, stroke, and trauma affecting the brain. Classifying FCMS based solely upon lesions yields five specific subtypes of FCMS currently known to fall into the bilateral and unilateral categories: The bilateral form of FCMS (also known as facio-labio-pharyngo-glosso-laryngo-brachial paralysis) is consistent with the classic presentation of bilateral corticobulbar involvement. It is characterized by well-preserved automatic and reflex movements. It is caused by lesions in the cortical or subcortical region of the anterior opercular area surrounding the insula forming the gyri of the frontal, temporal, and parietal lobes. The unilateral operculum syndrome is a very rare form of FCMS caused by the formation of unilateral lesions. In this form of FCMS, the unaffected hemisphere of the brain compensates for the unilateral lesion. Usually, this occurs when the unaffected region is the individual's dominant hemisphere. An individual affected with FCMS develops disabilities associated with voluntary movements using the facial, lingual, pharyngeal, and masticatory muscles. However, the reflexive and autonomic functions of these muscles groups are usually intact. Common symptoms include drooling, an inability to elevate and depress the mandible, difficulty chewing, inability of protruding tongue, swallowing, and loss of speech.