Meige syndrome is an organic neurological movement disorder that belongs to the group of focal dystonia. The French neurologist Henry Meige (1866 – 1940) already 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 malfunction of certain brain structures, which triggers an involuntarily controlled eyelid spasm. See AbbreviationFinder for abbreviations related to Meige Syndrome.
Causes
Neurological muscle disease is not a generalized disease. Since it usually occurs in combination with dystonia of the lower half of the face, doctors try to see the trigger or triggers in context. They explore the complexity of the various causes considering numerous possibilities.
Starting with drugs (such as neuroleptics ), degenerative brain diseases ( Parkinson’s disease ), circulatory disorders or brain damage in childhood to accidental injuries in the head and brain area. In relation to the Meige syndrome in the individual state, however, no cause is currently known.
Symptoms, Ailments & Signs
The syndrome manifests itself in involuntary eyelid spasms that occur on one or both sides of the eyes. This permanent contraction is caused by the orbicularis oculi muscle (closing muscle of the eyelids). He is responsible for opening and closing the eyes.
When this muscle spasms, the eyes close. This often occurs with frequent blinking in the form of forced blinking, sometimes sustained. Both eyes are usually affected, with one eye always more severe than the other. The facial expressions are very disfiguring due to the muscle spasms. As the muscle contraction progresses, the ability to see is limited, which means that many of those affected can only go out on the street with an escort.
Symptoms of Meige syndrome also include spasms in the lower half of the face, facial, jaw, and throat muscles. Sudden facial movements, making faces, grinding teeth, sticking out tongue, contracting cheeks, pulling lower lip over upper lip, pursing mouth are just a fraction of the problems that sufferers have to struggle with in everyday life.
Such manifestations are in no way associated with pain or comparable to a malignant disease. But the helplessness caused by the sudden, unpredictable occurrence of cramps puts those affected under enormous psychological strain. Because the conspicuous illness is met with a lack of understanding by other people, who cannot assess it correctly or misinterpret it, those affected usually avoid the public. They withdraw, become shy of contact or, in the worst case, develop depression.
Diagnosis & disease progression
In the initial diagnosis, the treating neurologist must proceed in a process of exclusion. He thus clarifies whether it is a symptomatic, idiopathic, or genetic origin. In most cases, the “idiopathic”, non-comprehensible form appears. A decisive indication of this clinical picture is the increased occurrence within the family. These facts must be communicated to the treating neurologist during the initial anamnesis.
In order to establish an exact diagnosis, differential diagnostic forms of lid closure and lid opening disorders must be ruled out. This applies in particular to inflammatory eye diseases or tics. Conclusions on a confusion with the ocular myasthenia, a disturbed transmission of nerve impulses to the muscle, are considered.
Complications
Meige syndrome causes those affected to suffer from severe cramps that occur on the eyelids. They occur spontaneously, so that a general course of the disease cannot usually be predicted. There can also be discomfort when closing or opening the eyes, so that the everyday life of those affected is significantly restricted by the Meige syndrome.
Furthermore, the discomfort in the eyes leads to a severe limitation of vision and, in the worst case, to blindness. Due to these restrictions, many everyday activities can no longer be carried out easily, so that the patients are dependent on the help of other people. The quality of life is therefore significantly reduced by Meige syndrome.
Furthermore, the Meige syndrome can also lead to various spasms in the patient’s face, so that the teeth can also be damaged. In everyday life there is pain, which can often lead to depression and other mental disorders. The symptoms of Meige syndrome can be reduced with the help of medication.
However, those affected are dependent on very lengthy therapy. Whether this leads to a reduction in life expectancy depends heavily on the severity of the Meige syndrome and cannot usually be predicted in general.
When should you go to the doctor?
If you have trouble opening and closing your eyes, you should see a doctor. A doctor should be consulted in the event of disturbances in voluntary movement, cramps in the face or permanent contractions of the eyelid. If the symptoms lead to reduced vision, the person concerned needs help. If the general risk of accidents and injuries increases, consult a doctor.
If your chewing function is impaired, you have problems swallowing or you have problems with your jaw, you should see a doctor. If teeth grind, it is advisable to see a doctor for a check-up. If the affected person experiences that he cannot subject his facial features to voluntary regulation, he needs a doctor. People present often describe that the sick person makes faces.
This leads to interpersonal conflicts and triggers withdrawal behavior on the part of those affected. In the case of mental or emotional irregularities, a doctor’s visit should also take place. Depressive moods, mood swings, reduced self-esteem, anger, shame or aggressive tendencies should be discussed with a doctor.
If the person concerned suffers, experiences a reduced quality of life or is unable to cope with everyday tasks due to the disorders, a doctor should be contacted. If the ability to see decreases with increasing contraction of the eye muscles, an ophthalmologist must be consulted so that he can be given help in everyday life.
Treatment & Therapy
Botulinum toxin (BTX) injection therapy has been used for almost two decades. Previously, medication was given with tablets, which relieved the muscle cramps. However, there were enormous side effects. Thus, today these preparations are only used as a support in addition to injection therapy.
There used to be attempts to surgically interrupt nerve fibers or remove fibers of the circular muscle in the eye, for example. Both 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, thin needles into the tense muscles around the eyes. The process is not painful; if necessary, however, a local anesthetic is available.
After the injection, the effect does not start immediately. Only after a few days does the eyelid spasm decrease. The duration of the effect depends on the severity or the state of the cramping of the muscle. Unfortunately, this therapy is only insufficiently effective in about 10 percent of those affected, so that these patients are supported by medication.
On average, the patient affected by Meige syndrome needs repeat injections of BTX four times. The treatments per year are usually reduced by half in the case of a one-sided eyelid spasm. Although the interval between treatments cannot be extended, the beneficial effects continue even after numerous repeat injections.
At the current state of medicine, BTX therapy is the most effective of all for these symptoms. It is a service provided by all private and statutory health insurance companies.
Outlook & Forecast
The prognosis of Meige syndrome is unfavorable. So far, the cause of the disease could not be fully elucidated. What is certain is that a neurological muscle disease is present, but it often manifests itself in very different ways. This complicates the treatment process to a considerable extent. Physicians focus on alleviating the existing symptoms and can therefore only initiate symptomatic improvements. However, no way has yet been found to achieve permanent freedom from symptoms of the disease.
Several treatment methods are initiated in parallel in order to achieve an improvement in overall health. The combination of different treatment approaches has proven to be advantageous for the patient. Overall, they lead to an alleviation of existing health irregularities and thus promote general well-being. The patient is dependent on long-term therapy and experiences a worsening of the situation as soon as treatment is stopped.
Overall, however, the course of the disease is strongly influenced by how intensively the individual symptoms occur. It has been shown that despite the same diagnosis, patients suffer from the health impairments to different extents. It must also be taken into account that due to the numerous irregularities, states of mental and emotional stress develop in many cases. Therefore, the risk of a psychological sequelae was increased in patients with Meige syndrome.
Prevention
Since no causes are known for Meige syndrome, only the symptoms can be alleviated in the first place. This is mainly done through medication. At the same time, lifestyle also plays an important role in a large number of diseases. Stress is a great danger. That is why adhering to a regulated everyday life with sufficient rest phases for the body’s defenses is the be-all and end-all. It’s worth a try – even in the hectic.
Aftercare
Meige syndrome can lead to various symptoms and complaints, all of which can usually have a very negative impact on the sufferer’s quality of life. In most cases, the Meige syndrome causes severe discomfort in the eyes, whereby they can no longer be closed and opened properly. The facial expressions of those affected also suffer from this syndrome, so that most patients also suffer from aesthetic complaints.
Since the treatment of Meige syndrome is relatively complex and lengthy, follow-up care focuses on dealing with the condition with confidence. Those affected should try to focus on a positive healing process despite the adversity. To establish the right attitude, relaxation exercises and meditation can help calm and focus the mind. This is a basic requirement for recovery.
You can do that yourself
People suffering from Meige syndrome definitely need comprehensive medical treatment. Under certain circumstances, the individual symptoms can be treated by those affected themselves, accompanied by medication.
If muscle cramps occur, the first thing to do is to calm the person affected. If possible, the patient should lie down and take the medication prescribed by the doctor. If this has no effect, relaxation measures can be used to reduce the spasms. In some cases, massage or a hot bath can help, depending on the severity of the disease 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, since these tics represent an enormous burden on the mental state. In some cases, behavioral therapy can help, in which those affected learn to avoid the involuntary movements.
The best way to counteract a decrease in vision is to wear glasses. Nevertheless, many patients need support in everyday life. Especially in the later stages of the disease, accommodation in a nursing home makes sense.