Meige syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at January 6, 2017
StartDiseasesMeige syndrome

Meige syndrome is a rare condition that strikes women more frequently than men. It is a neurological disorder which is characterized by involuntary contractions and spasms of the facial muscles surrounding the eyes, the mouth, and other areas. Meige syndrome is also called Brueghel's syndrome, idiopathic blepharospasm-oromandibular dystonia syndrome, and segmental cranial dystonia.

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Definition & Facts

Meige syndrome is a condition known as a dystonia. A dystonia causes the muscles to contract involuntarily. In this case, the muscles involved are those around the eyes, mouth, tongue, jaw and neck. Some are affected in the arms or legs, and some contractions can be painful. It is an illness of older people, with most affected between 40 and 70 years of age, although some cases start earlier.

Up until the 20th century, the condition was considered to be psychiatric and the afflicted were put into insane asylums until Henry Meige, a French neurologist, determined it stemmed from the central nervous system. He described the disease in 1910.

Symptoms & Complaints

Symptoms and severity will vary from person to person. Blepharospasm, the contraction of the muscles causing forced and frequent blinking, can happen in response to a stimulus, such as wind and air pollution, stress, bright lights, fatigue and others, and may increase in severity over time.

The space between the eyelids may narrow and some patients may actually become blind because the spasm keeps the lids shut. This may affect one eye, but usually it affects both. Patients may also have dry eyes. The jaw and tongue are affected similarly. The muscle spasms make it difficult to open and shut the mouth, which affects eating.

Grinding the teeth, pursing the lips, clenching the jaw, and grimacing are all manifestations of this dystonia. Patients may also have spasms of the tongue causing it to protrude, and spasms of the throat making swallowing difficult.

Breathing may become affected if the respiratory system is involved, and others may have spasms in their necks. Over time, muscle tone may weaken (hypotonia). Patients may see their symptoms fade away, while others’ symptoms may become progressively worse.

Causes

The cause of Meige syndrome is unknown. However, it is believed that the origin of the spasms and contractions is in the basal ganglia of the brain. The basal ganglia, which is located at the bottom of the brain, is made up of nerve cells. It regulates motor functioning and learning. Other areas that may be involved are the midbrain and/or the brainstem. These three structures are where blinking is controlled and recent research suggests that this is where the defect lies.

There are three muscles involved in blinking. They are the orbicularis oculi muscle, located around the eyes; the corrugator supercilii muscle, which involves the frown; and the procerus muscle, located at the root of the nose which controls blinking.

The trigeminal nerve transmits nerve impulses to and from the major parts of the face to the basal ganglia, and if there is a defect it may result in a muscle spasm in response to a stimulus such as bright lights or eye irritation. Meige syndrome symptoms may also occur as a side effect of some medications, such as those used in treating Parkinson’s disease. Lowering the dose can help.

Diagnosis & Tests

There is no definitive test for Meige syndrome. Diagnosis must be based on patient medical history, observation of characteristic symptoms, and a thorough physical examination. The doctor will look for increased eye irritation, abnormal eye blinking, and spasms in other affected areas. It is often misdiagnosed by general practitioners because it is such a rare condition. A neurologist who treats motor disorders would be more likely to recognize it.

Treatment & Therapy

Treatments involve drug therapy and the injection of botulinum A toxin (Botox). The treatment aims at specific areas. The drugs used are:

All of these medications have side effects, some of which can be serious. Botulinum A toxin (Botox) has been approved by the Food and Drug Administration (FDA) for use with Meige syndrome, and it has met with some success. Small amounts are injected into the affected muscles which become spasm-free. It is particularly effective with helping swallowing and speaking. The treatment lasts for for a few months, then another injection must be administered. It works for both the eye muscles and those of the lower face, but not for everyone. Some results are only partially successful, others more complete.

Other therapies include performing special movements called sensory tricks. Examples are chewing gum, gently touching the lips, mouth and chin, and biting on a toothpick.

Deep brain stimulation also has had success. A low voltage electrode is placed in the brain and is attached to a pulse generator, similar to a cardiac pacemaker. The pulse is conducted through a very thin wire and is sent to the parts of the brain that are malfunctioning and causing spasms.

The physician can adjust the pulse generator in his office. Stereotactic radial surgery is used as a last resort for those who have not responded to other treatments. Speech therapy and swallowing therapy have also been shown to reduce the intensity of the condition.

Prevention & Prophylaxis

Prevention of Meige’s syndrome is impossible at this time since the cause is not known. Measures can be taken to avoid certain spasms. A person suffering from this condition should determine what may trigger the spasms and try to avoid it.

For example, if the eyes are sensitive to bright lights, one should avoid them or wear sunglasses, which will also protect against pollutants and particles blowing into the eyes. Stress or fatigue may also bring on the spasms, so getting adequate rest and reducing stress may help stave off symptoms. Counseling or psychotherapy may assist in reducing stress.