Meige syndrome is an organic neurological movement disorder that belongs to the group of focal dystonias. Already the French neurologist Henry Meige (1866 – 1940) dealt with this topic and described the clinical picture in detail in 1910. Meige syndrome is named after him.
What is Meige syndrome?
The contractions between the jaw and mouth muscles are called oromandibular dystonia. Meige syndrome (also known as blepharospasm-oromandibular dystonia, idiopathic orofacial dyskinesia, or Brueghel syndrome) illustrates a combination of oromandibular dystonia and essential blepharospasm. It is based on a dysfunction of certain brain structures that triggers an involuntary spasm of the eyelids.
Causes
Neurologic muscle disease is not a generalized disorder. Because it usually occurs in combination with lower facial dystonia, physicians are working to see the trigger or triggers in context. They are exploring the complexity of the various causes, considering numerous possibilities. Beginning with medications (such as neuroleptics), degenerative brain diseases (Parkinson’s disease), circulatory problems or brain damage in childhood, to accidental injuries to the head and brain. However, with respect to Meige syndrome in the single state, no cause is currently known.
Symptoms, complaints, and signs
The syndrome manifests as involuntary eyelid spasms that occur unilaterally or bilaterally around the eyes. This continuous contraction is caused by the orbicularis oculi muscle (sphincter of the eyelids). It is responsible for opening and closing the eyes. If this muscle contracts, the eyes close. This often occurs with frequent blinking in the form of forced blinking, sometimes persistent. Usually both eyes are affected, one of them always more than the other. The facial expressions appear very disfiguring due to the muscle spasms. As the muscle contraction progresses, the ability to see is impaired, which means that many affected persons can additionally only go out on the street with an escort. Symptoms of Meige’s syndrome also include spasms in the lower face, facial, jaw, and pharyngeal muscles. Sudden facial movements, grimacing, grinding teeth, sticking out the tongue, contracting the cheek, pulling the lower lip over the upper lip, and pursing the mouth are only a fraction of the problems that sufferers have to contend with in everyday life. Such phenomena are in no way associated with pain or comparable to a malignant disease. However, the helplessness caused by the sudden unpredictable occurrence of the spasms alone places those affected under enormous pressure of suffering. Because the conspicuous illness is not understood by other people, because they cannot assess it correctly or misinterpret it, those affected usually avoid the public. They withdraw, become contact-shy or, in the worst case, develop depression.
Diagnosis and course of the disease
In the initial diagnosis, the treating neurologist must proceed in the exclusion procedure. He thus clarifies whether the origin is symptomatic, idiopathic, or genetic. In most cases, the “idiopathic”, intangible form appears. A decisive indication for this clinical picture is the increased occurrence within the family. These facts must be communicated to the treating neurologist during the initial history. In order to establish an exact diagnosis, differential forms of eyelid closure and eyelid opening disorders have to be excluded. This concerns in particular inflammatory eye diseases or tics. Inferences of confusion with ocular myasthenia, a disturbed transmission of nerve impulses to muscle, also receive attention.
Complications
As a result of Meige syndrome, affected individuals suffer from severe spasms that occur in the eyelids. They occur spontaneously, so a general course of the disease usually cannot be predicted. Similarly, there may be discomfort when closing or opening the eyes, so that the everyday life of those affected by Meige’s syndrome is considerably limited. Furthermore, the discomfort of the eyes leads to a severe limitation of vision and, in the worst case, to blindness.Due to these limitations, many activities of daily living can no longer be carried out without further ado, so that patients are dependent on the help of other people. The quality of life is therefore significantly reduced by Meige’s syndrome. Furthermore, Meige’s syndrome can also cause various spasms in the patient’s face, so that the teeth can also be damaged. In everyday life, the patient suffers from pain, which not infrequently can also lead to depression and other psychological upsets. With the help of medication, the symptoms of Meige’s syndrome can be limited. However, those affected are dependent on a very protracted therapy. Whether this results in a reduction in life expectancy depends greatly on the severity of Meige syndrome and cannot usually be predicted in general terms.
When should you see a doctor?
If the affected person experiences problems opening and closing the eyes, a visit to the doctor should be made. If there are disturbances in voluntary movement, spasms in the face, or continuous contractions of the eyelid, a doctor should be consulted. If the complaints result in reduced vision, the affected person needs help. If the general risk of accidents and injuries increases, a doctor should be consulted. If chewing function is impaired, swallowing disorders or jaw problems occur, a doctor should be consulted. If teeth grinding occurs, a check-up visit to a doctor is recommended. If the affected person experiences that he/she cannot subject his/her facial features to voluntary regulation, he/she needs to see a doctor. Often people present describe that the sufferer makes grimaces. This leads to interpersonal conflicts and triggers a withdrawal behavior of the affected person. In case of mental or emotional irregularities, a visit to the doctor should therefore also take place. Depressive moods, mood swings, reduced self-confidence, anger, shame or aggressive tendencies should be discussed with a doctor. If the affected person suffers, experiences a reduced quality of life or feels unable to perform everyday tasks due to the disorders, a doctor should be contacted. If vision decreases with increasing contractions of the eye muscles, an ophthalmologist must be consulted to provide assistance with daily living.
Treatment and therapy
Treatment with injection therapy using botulinum toxin (BTX) has been used for nearly two decades. Previously, medication was used to treat the condition with tablets, which provided relief from muscle spasms. However, enormous side effects occurred. Today, these preparations are therefore only used as a supportive measure in addition to injection therapy. In the past, there were attempts to interrupt nerve fibers or to remove fibers of the eye ring muscle by surgical means. Healing sleep, hypnosis and acupuncture, as well as psychotherapy or psychoanalysis were and are currently used for relief. Botulinum toxin is injected with very fine and thin needles into the tense muscles in a ring around the eyes. The procedure is not painful; however, local anesthesia is available if needed. After the injection, the effect does not start immediately. Only after a few days does the eyelid spasm diminish. The duration of the effect depends individually on the severity or the condition of the spasm of the muscle. Unfortunately, this therapy is only insufficiently effective in about 10 percent of those affected, so that these patients have access to supportive medication. On average, patients affected by Meige syndrome require repeat injections of BTX four times. Treatments per year are usually reduced by half for unilateral eyelid spasm. Although the inter-treatment period cannot be extended, the positive mode of action is present even after numerous repeat injections. BTX therapy is the most effective treatment for this symptomatology at the current state of medicine. It is a benefit of all private and statutory health insurance companies.
Outlook and prognosis
The prognosis of Meige syndrome is unfavorable. To date, the cause of the disease has not been fully elucidated. What is certain is that a neurologic muscle disease is present, but it often presents in quite different ways. This complicates the treatment process to a considerable degree.Medical practitioners focus on alleviating the existing symptoms and can therefore only initiate symptomatic improvements. Nevertheless, no way has yet been found to achieve permanent freedom from the symptoms of the disease. Several treatment methods are initiated in parallel to achieve an improvement in overall health. The combination of different treatment approaches have proven to be beneficial for the patient. They lead to an overall alleviation of existing health irregularities and thereby promote overall well-being. The patient is dependent on long-term therapy and experiences immediate deterioration with the discontinuation of a treatment intervention. Overall, however, the course of the disease is strongly influenced by the intensity of the individual complaints. It has been shown that despite the same diagnosis, patients suffer from the health impairments to varying degrees. It must also be taken into account that, due to the numerous irregularities, states of psychological and emotional stress develop in many cases. Therefore, the risk of secondary mental illness was increased in patients with Meige syndrome.
Prevention
Since there are no known causes for Meige syndrome so far, only the symptoms can be alleviated in the first instance. This is mainly done by medication. At the same time, lifestyle also plays an important role in a variety of diseases. Stress is a great danger. Therefore, maintaining a regular daily routine with sufficient rest periods is the be-all and end-all for our body’s defenses. It’s worth trying – even in the most hectic of times.
Aftercare
Meige syndrome can lead to various symptoms and complaints, all of which can have a very negative impact on the quality of life of the affected person. In most cases, Meige syndrome causes severe discomfort to the eyes, with the eyes unable to close and open properly. The affected person’s facial expressions also suffer as a result of this syndrome, so that most patients also suffer from aesthetic complaints. Since the treatment of Meige’s syndrome is relatively complex and lengthy, aftercare focuses on a self-confident approach to the disease. Affected individuals should try to focus on a positive healing process despite the adversity. To build the appropriate mindset, relaxation exercises and meditation can help calm and focus the mind. This is fundamental to recovery.
Here’s what you can do yourself
Individuals suffering from Meige syndrome definitely require comprehensive medical treatment. Individual symptoms may be able to be self-treated by those affected, accompanied by drug treatment. When muscle cramps occur, the first thing to do is to reassure the sufferer. If possible, the sufferer should lie down and take the medication prescribed by the doctor. If this shows no effect, relaxation measures can be used to reduce the cramps. In some cases, massages or a hot bath help, although this depends on the severity of the condition and the patient’s constitution. No treatment is possible for teeth grinding, tongue sticking out and similar typical symptoms. The patient usually needs psychological help, as these tics put an enormous strain on the mental state. In some cases, behavioral therapy helps, in which the affected person learns to avoid the involuntary movements. A decrease in vision can best be counteracted by wearing a visual aid. Nevertheless, many patients need support in everyday life. Especially in the later stages of the disease, placement in a nursing home makes sense.
