Foix–Chavany–Marie syndrome

Operculum

Foix–Chavany–Marie syndrome (FCMS), also known as bilateral anterior opercular syndrome, is a rare neurological disorder characterized by the loss of voluntary control over the muscles of the face, tongue, pharynx, and masticatory muscles, while involuntary and reflexive movements remain intact. This condition is named after the French neurologists Charles Foix, Jean Alfred Émile Chavany, and Julien Marie, who first described it in 1926.

Clinical Features

Patients with Foix–Chavany–Marie syndrome typically present with:

• Dysarthria: Difficulty in articulating words due to muscle weakness.
• Dysphagia: Difficulty in swallowing.
• Facial diplegia: Paralysis of the facial muscles on both sides.
• Preservation of reflexive movements such as blinking and emotional expressions.

Pathophysiology

The syndrome results from bilateral lesions in the anterior operculum, which is part of the cerebral cortex involved in motor control of the face and mouth. These lesions can be caused by various factors, including:

• Stroke
• Traumatic brain injury
• Infections such as encephalitis
• Tumors
• Neurodegenerative diseases

Diagnosis

Diagnosis of FCMS is primarily clinical, supported by imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans that reveal bilateral lesions in the anterior operculum. Electromyography (EMG) and nerve conduction studies may also be used to assess the extent of muscle involvement.

Treatment

There is no specific treatment for Foix–Chavany–Marie syndrome. Management focuses on addressing the underlying cause and providing supportive care, which may include:

• Speech therapy
• Physical therapy
• Nutritional support to manage dysphagia
• Medications to control symptoms such as muscle spasticity

Prognosis

The prognosis of FCMS varies depending on the underlying cause and the extent of the brain lesions. Some patients may experience partial recovery of function, while others may have persistent deficits.

Related Pages

• Stroke
• Dysarthria
• Dysphagia
• Cerebral cortex
• Traumatic brain injury
• Encephalitis
• Neurodegenerative diseases