This condition, occurring in middle-aged or elderly individuals, is the result of dystonic stimulation medi­ated through the facial nerve. The signs of orofacial mandibular dystonia and blepharospasm are charac­teristic. The changes, which resemble tardive dyski­nesia, are not induced by neuroleptic drugs and are unlike the open mouth dystonia of Bruegel syndrome. Lower pontine lesions can be identified in some cases. There may be a temporary improvement with haloperidol, tetrabenazine, or intravenous Cogentin. Botulinum toxin injection into the affected facial muscles is the treatment of choice.

This condition is the result of dystonic stimulation mediated through the Facial nerve

Meige syndrome is a rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm). The specific symptoms and their severity vary from case to case.

Meige's Syndrome/Brueghel's syndrome or oral facial dystonia
Meige’s Syndrome/Brueghel’s syndrome or oral facial dystonia

Other Names of Meige’s Syndrome

  • Brueghel’s syndrome
  • oral facial dystonia
  • Brueghel Syndrome
  • Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome
  • Segmental Cranial Dystonia

Meige syndrome is a rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm). The specific symptoms and their severity vary from case to case.

Meige syndrome belongs to a group of disorders known as dystonia. Dystonia is a group of movement disorders that vary in their symptoms, causes, progression, and treatments. This group of neurological conditions is generally characterized by involuntary muscle contractions that force the body into abnormal, sometimes painful, movements and positions (postures). The exact cause of Meige syndrome is unknown.

Signs & Symptoms of Meige’s Syndrome/Brueghel’s syndrome or oral facial dystonia

Meige syndrome is characterized by the combination of blepharospasm and oromandibular dystonia.

The signs of orofacial mandibular dystonia and blepharospasm are characteristic

Blepharospasm Symptoms

  • frequent or forced blinking and eye irritation that often occurs as a result of specific stimuli including bright lights
  • fatigue
  • emotional tension
  • environmental factors such as wind or air pollution.
  • uncontrollable squinting/closing of eyes
  • light sensitivity (photophobia)
  • squinting/eyes closing during a speech
  • uncontrollable eyes closing shut (rare instances completely causing blindness)

The frequency of muscle spasms and contractions may increase causing narrowing of the opening between the eyelids or involuntary closure of the eyelids. It may become progressively harder for affected individuals to keep their eyes open. Blepharospasm may originally affect one eye (unilateral) but usually becomes (bilateral). Some individuals with Meige syndrome may experience abnormally dry eyes.

Oromandibular dystonia Symptoms

  • Involuntary, forceful contractions of the jaw and tongue often making it difficult to open or close the mouth.
  • clenching or grinding of the teeth
  • displacement of the jaw
  • grimacing
  • chin thrusting
  • repeated pursing of the lips.
  • Eyelid and facial muscle tone may gradually decline.
  • difficulty opening the mouth (trismus)
  • clenching or grinding of the teeth (bruxism)
  • spasms of jaw opening
  • sideways deviation or protrusion of the jaw
  • lip tightening and pursing
  • drawing back (retraction) of the corners of the mouth
  • deviation or protrusion of the tongue.
  • jaw pain
  • difficulties eating and drinking
  • difficulties speaking (dysarthria)

Occur in middle-aged or elderly individuals

Some people with Meige syndrome may also experience spasms of the tongue and throat, resulting in repeated protrusion of the tongue from the mouth and difficulty swallowing. Muscle spasms of the respiratory tract may lead to breathing difficulties (dyspnea). In some cases, muscles in the neck, arms, legs or other muscle groups may become affected.

Causes of Meige’s Syndrome

The cause of Meige syndrome is unknown. Researchers speculate that the cause of Meige syndrome may be multifactorial (e.g., caused by the interaction of certain genetic and environmental factors).

Malfunctioning of a region of the brain known as the basal ganglia may play a role in the development of Meige syndrome. The basal ganglia is a structure composed of nerve cells located at the base of the brain. The basal ganglia are involved in the regulation of motor and learning functions. The exact problems associated with the basal ganglia in individuals with Meige syndrome is unknown.

The changes, which resemble tardive dyskinesia, are not induced by neuroleptic drugs and are unlike the open mouth dystonia of Bruegel syndrome Lower pontine lesions can be identified in some cases

Some cases of oromandibular dystonia occur in association with or secondary to another disorder such as

  • tardive dyskinesia
  • Wilson disease
  • Parkinson disease.

Clinical Features of Meige’s Syndrome

Patients with blepharospasm may also have lower facial dystonia

  • masticatory (jaw muscles) dystonia
  • lingual (tongue) dystonia
  • pharyngeal (swallowing) dystonia
  • laryngeal (voice box) dystonia
  • cervical (neck) dystonia.
Meige's Syndrome/Brueghel's syndrome or oral facial dystonia
Meige’s Syndrome/Brueghel’s syndrome or oral facial dystonia

Involvement of the lower facial and masticatory muscles is fairly common in patients with blepharospasm. Involuntary lower facial and masticatory movements may include

  • lip pursing
  • chewing
  • jaw thrusting
  • jaw opening
  • jaw closing/clenching.
  • In some patients, the lower facial and jaw movements may be rhythmic or tremor-like.

The combination of upper and lower dystonia is sometimes called cranial-cervical dystonia.

In addition, the involuntary lower facial and jaw movements seen in patients with blepharospasm may not result in sustained postures and, as such, may not be compatible with the definition of dystonia. Instead, these involuntary movements are oftentimes described as dyskinesias, a poorly-defined term which may include several forms of involuntary movements like

  • chorea
  • athetosis
  • myoclonus

each of which carries a more precise meaning.

Most of the time, dystonia begins focally. Over time, however, dystonia may spread to involve other muscles of the body. Most commonly, dystonia spreads to contiguous muscles. For example, when blepharospasm does spread, it typically spreads to the lower face and/or masticatory muscles. Rarely, dystonia may spread to more distant muscles in the arms and legs. Although the probability of spread is highest within the first 3 years after dystonia onset, the spread may occur a decade or later. In patients with blepharospasm, older age-of-onset and female gender may increase the risk of spread. Overall, patients with initial onset of dystonia in the eyelids (i.e., blepharospasm) have at least a 50% lifetime probability of spread.

Segmental craniocervical dystonia or Meige’s Syndrome should be diagnosed by a clinician with extensive experience in movement disorders. Although usually idiopathic, segmental craniocervical dystonia may be associated with neurodegenerative disorders such as progressive supranuclear palsy or caused by medications which block dopamine receptors in the brain such as antiemetics (e.g., metoclopramide) and antipsychotics (e.g., haloperidol).

Oftentimes, a brain MRI scan and blood tests are ordered to exclude the possibility of secondary causes such as Wilson’s disease or a stroke. In most cases, all diagnostic studies will prove to be normal. The term “idiopathic” does preclude a distinct genetic etiology or genetic contributions, however, since blepharospasm and segmental craniocervical dystonia clearly has a hereditary component. In this regard, some studies have reported that over 10% of patients with blepharospasm and other forms of craniocervical dystonia have at least one first- or second-degree relative with dystonia.

Differential diagnosis of Meige’s Syndrome

  •  focal (affecting an isolated body part)
  • segmental (affecting adjacent body areas)
  • generalized (affecting many major muscle groups simultaneously)
  • Temporomandibular joint (TMJ) dysfunction
  • Benign essential blepharospasm (BEB)
  • Tardive dyskinesia (TD)
  • Hemifacial Spasm

Genetic as well as non-genetic factors contribute to all forms of dystonia.

Diagnosis of Meige’s Syndrome

No tests exist to diagnose Meige syndrome. A diagnosis is made based on a thorough clinical evaluation, a detailed patient history and identification of characteristic symptoms.

Meige’s is commonly misdiagnosed and most doctors will not have seen this condition before. Usually, a neurologist who specializes in movement disorders can detect Meige’s. There is no way to detect Meige’s by a

  • blood test
  • MRI
  • CT scans.

OMD by itself may be misdiagnosed as TMJ.

The lack of prompt response to anticholinergic drugs in cases of idiopathic Meige’s syndrome is important in differentiating it from acute dystonia, which does respond to anticholinergics.

Meige's Syndrome/Brueghel's syndrome or oral facial dystonia
Meige’s Syndrome/Brueghel’s syndrome or oral facial dystonia

How to Treat Meige’s Syndrome

Segmental craniocervical dystonia is commonly treated with injections of botulinum toxins. Successful treatment of dystonia with an injection of botulinum toxins is both art and science and not all artists and scientists are created equal. Results depend on the accurate targeting of affected muscles with an appropriate amount of toxin. Electromyographic guidance is normally used when injecting jaw, laryngeal, and neck muscles.

There may be temporary improvement with haloperidol, tetrabenazine, or intravenous Cogentin.

A long list of oral medications has been used to treat segmental craniocervical dystonia. Unfortunately, the magnitude of improvement typically obtained with commonly used drugs such as the:

  • anticholinergics (e.g., trihexyphenidyl and benztropine)
  • benzodiazepines (e.g., clonazepam, lorazepam)
  • baclofen
  • tetrabenazine are often modest at best.

Botulinum toxin injection into the affected facial muscles is the treatment of choice.

In recent years, deep brain stimulation (DBS) has garnered increasing attention as a therapeutic option in patients with intractable dystonia. DBS has been associated with dramatic benefit in patients with DYT1 generalized dystonia. Currently, available data suggests that DBS is also an effective treatment for many, but not all patients with segmental craniocervical dystonia. DBS is associated with a small risk of stroke, infection, and the development of new neurological signs and symptoms. DBS should be seriously considered only if other treatment options, administered by skilled and experienced neurologists, are ineffective.

In some cases, Meige’s syndrome can be reversed when it is caused by medication. It has been theorized that it is related to craniomandibular orthopedic misalignment, a condition that has been shown to cause a number of other movement disorders

  • Parkinon’s
  • tourettes
  • torticollis

This theory is supported by the fact that the trigeminal nerve is sensory for blink reflex, and becomes hypertonic with craniomandibular dysfunction. Palliative treatments are available, such as botulinum toxin injections.

Prognosis of Meige’s syndrome

The first symptoms of Meige syndrome may occur anywhere from about 30 to 70 years of age, but they most often occur in the sixth decade of life. Blepharospasm (dystonia of the eyelids) is the most frequent initial complaint of affected people.

The long-term outlook (prognosis) for people with Meige syndrome is hard to predict because the specific symptoms, severity, and progression of the condition vary from person to person. The prognosis likely depends on several factors, such as the age of onset; the rate of progression; muscles affected; how early treatment is started; and how a person responds to treatment. Some affected people improve over time.

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