Meige's syndrome

Meige's syndrome
ICD-10	G24.4
ICD-9	333.82
DiseasesDB	31428
MeSH	D008538

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Not to be confused with Meigs syndrome

Dystonia is a neurological disorder that causes involuntary movements of muscles due to a disturbance in the brain's neural network. Meige's syndrome(MS) is a type of facial dystonia. It is also known as Brueghel's syndrome, however, this term is more often used for the condition of oromandibular dystonia only in the absence of blepharospasm. Meige's syndrome affects the tongue, eyes, jaw, pharynx, and neck muscles. It is also called blepharospasm, oromandibular, platysma, and cervical dystonia, respectively.It was first identified by French neurologist Henry Meige in the 20th century, who referred to it as “spasm facial median”. Later, in 1972, Dr. George Paulson introduced the term Meige's syndrome and defined it as the involuntary movements of the oromandibular and blepharospasm.

Epidemiology

The disease appears between the age of 30-70 years, with a mean onset age of 55.7 years. It is prevalent in older females than males, with a ratio of 2:1. This may be due to certain estrogen receptors that affect involuntary motor function and make women more vulnerable to experiencing involuntary muscle spasms. The disease is observed in approximately 100 cases per 100,000 people.

Etiology

Alabenese et al. explain the etiology of Meige's syndrome and divide the condition into two categories “hereditary dystonia” and “acquired dystonia”.

Genetic dystonia:

It is caused by gene variants including TOR1A, TOR2A, GNAL, OR1A, REEP4, and THAP1, respectively. TOR1A and THAP1 play a significant role in the pathophysiology of the disease.

Acquired dystonia:

It is caused by environmental factors such as medical conditions including (trauma, cerebral palsay, stroke, encephalopathy, etc), medications, and poisons including (heavy metal toxicity, antipsychotic medication, and others).

Presentations

Blepharospasm affects the eyelid and increases blinking along with ocular pain but due to the disease progressing to oromandibular dystonia patient experiences involuntary tongue movements and has difficulty with eating, swallowing, laughing, speaking, and yawning. These symptoms persist until the patients consciously relax. Emotional stress can worsen diseases in around 33% of patients this can be shown by symptoms such as sleeplessness, sadness, and increased muscular tension(6).

Blepharospasm related symptoms  

•     Eyelid fluttering
•     Exaggerated blinking
•     Apraxia  of eyelid opening
•      Involuntary closing of the eye
•      Discomfort in opening eye
•      Blindness
•      Photophobia
•      Dry eyes

Oromandibular dystonia related symptoms:

• Unilateral retraction of the lip cheek, and jaw
•     Jaw pain
•     Difficulty in opening and closing mouth
•     Trismus,
•      Bruxism
• Stuck teeth

Diagnosis

Meige's syndrome is a rare disease normally diagnosed using a patient's medical history, external observations, and neurological examination. There is no particular test to identify Meige's syndrome, several procedures are conducted to rule out other probable causes. These might include genetic screening, face electromyography, VBM-MRI, CT, serum testing for SSA/SSB levels, serum copper and ceruloplasmin levels, Beck's depression inventory, and serum drug screening.

Treatment

There is currently no known cure for MS, however medical therapy can help decrease discomfort and improve a patient's quality of life. The sort of treatment is administered based on the patient's medical condition and history. Treatment options include surgery(e.g partial resection of the periorbital muscle, which surrounds the eye) medication (given orally) are anticholinergics (trihexyphenidyl and benztropine), benzodiazepines (clonazepam, diazepam, and lorazepam), GABA receptor agonist (baclofen), dopamine precursor(bromocriptine, and tiapride), vesicular monoamine transporter 2 inhibitor (tetrabenazine), anticonvulsant such as levetiracetam. and rehabilitative treatment (include physiotherapy, occupational therapy) and BoNT injection it is used as a primary treatment option for MS it help to reduced muscle spasm. When other treatments are failed to control the disease then Deep brain stimulation of the globus pallidus interna (GPi) is a therapeutic approach.

See also

• Blepharospasm
• Mogigraphia