Foix–Chavany–Marie syndrome

Overview

Foix–Chavany–Marie Syndrome (FCMS), also known as opercular syndrome, is a rare neurological disorder characterized by bilateral paralysis of the facial, lingual, pharyngeal, and masticatory muscles. This condition leads to loss of voluntary movement in these muscles while automatic and emotional movements (such as spontaneous smiling or crying) remain intact—a phenomenon known as autonomic-voluntary dissociation. FCMS typically results from bilateral lesions in the opercular regions of the brain, which control motor functions of the lower cranial nerves.

Causes

Foix–Chavany–Marie Syndrome is caused by bilateral lesions in the opercular cortex (specifically the frontal and parietal opercula), affecting the corticobulbar tracts responsible for voluntary control of facial and oropharyngeal muscles. Common causes include:

• Bilateral strokes: Especially in the middle cerebral artery territory.

• Brain infections: Such as encephalitis or abscesses that affect both opercular areas.

• Head trauma: Resulting in symmetrical brain injury.

• Demyelinating diseases: Like multiple sclerosis in rare cases.

• Neurodegenerative disorders: Progressive forms may mimic or evolve into opercular syndromes.

Symptoms

The hallmark symptoms of Foix–Chavany–Marie Syndrome are related to orofacial and bulbar muscle paralysis. Common clinical features include:

• Loss of voluntary facial movement: Inability to smile, frown, or show other intentional facial expressions.

• Anarthria: Complete loss of the ability to articulate speech despite preserved language comprehension.

• Dysphagia: Difficulty swallowing due to pharyngeal muscle paralysis.

• Inability to chew: Due to masticatory muscle involvement.

• Preserved involuntary movements: Patients may still laugh, cry, or yawn spontaneously (autonomic-voluntary dissociation).

• Drooling: Due to poor control over oral muscles.

Diagnosis

Diagnosis of FCMS is based on clinical evaluation, supported by neuroimaging and exclusion of other neurological conditions. Diagnostic steps include:

• Neurological examination: Reveals bilateral lower cranial nerve dysfunction with preserved automatic movements.

• Brain MRI or CT scan: Identifies bilateral opercular lesions, most commonly due to ischemic strokes.

• Speech and swallowing assessments: To evaluate the extent of functional impairment.

• Electromyography (EMG): In selected cases to assess muscle involvement and rule out peripheral causes.

Treatment

There is no specific cure for Foix–Chavany–Marie Syndrome; treatment focuses on supportive care and rehabilitation. Management strategies include:

• Speech and language therapy: To aid communication using alternative or augmentative methods.

• Swallowing therapy and dietary modifications: To prevent aspiration and ensure nutritional intake.

• Physical and occupational therapy: To improve mobility and daily function.

• Management of underlying cause: For example, antiplatelet or anticoagulant therapy for stroke, or antibiotics for infections.

• Nutritional support: In severe cases, gastrostomy may be required for feeding.

Prognosis

The prognosis of Foix–Chavany–Marie Syndrome varies depending on the underlying cause and the extent of brain damage. In cases due to stroke, some patients may show partial recovery with intensive rehabilitation, while others may remain severely disabled. Prognosis is generally poorer when the lesions are extensive or due to progressive neurological diseases. Early diagnosis, prompt management of the cause, and sustained rehabilitation efforts are key to optimizing functional outcomes.