Meige's syndrome

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

WikiDoc Resources for Meige's syndrome
Articles
Most recent articles on Meige's syndrome Most cited articles on Meige's syndrome Review articles on Meige's syndrome Articles on Meige's syndrome in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Meige's syndrome Images of Meige's syndrome Photos of Meige's syndrome Podcasts & MP3s on Meige's syndrome Videos on Meige's syndrome
Evidence Based Medicine
Cochrane Collaboration on Meige's syndrome Bandolier on Meige's syndrome TRIP on Meige's syndrome
Clinical Trials
Ongoing Trials on Meige's syndrome at Clinical Trials.gov Trial results on Meige's syndrome Clinical Trials on Meige's syndrome at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Meige's syndrome NICE Guidance on Meige's syndrome NHS PRODIGY Guidance FDA on Meige's syndrome CDC on Meige's syndrome
Books
Books on Meige's syndrome
News
Meige's syndrome in the news Be alerted to news on Meige's syndrome News trends on Meige's syndrome
Commentary
Blogs on Meige's syndrome
Definitions
Definitions of Meige's syndrome
Patient Resources / Community
Patient resources on Meige's syndrome Discussion groups on Meige's syndrome Patient Handouts on Meige's syndrome Directions to Hospitals Treating Meige's syndrome Risk calculators and risk factors for Meige's syndrome
Healthcare Provider Resources
Symptoms of Meige's syndrome Causes & Risk Factors for Meige's syndrome Diagnostic studies for Meige's syndrome Treatment of Meige's syndrome
Continuing Medical Education (CME)
CME Programs on Meige's syndrome
International
Meige's syndrome en Espanol Meige's syndrome en Francais
Business
Meige's syndrome in the Marketplace Patents on Meige's syndrome
Experimental / Informatics
List of terms related to Meige's syndrome

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 is a type of facial distonia. It is also known as Brueghel Syndrome; however this term is more often used for the condition of oromandibular distonia only in the absence of blepharospasm. Meige's syndrome affects the muscles in the tongue, eyes, jaw, pharynx, and neck. It is also referred to as blepharospasm, oromandibular, platysma, and cervical dystonia, respectively. These are different but closely related moment disorder,.The disorder 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 Magee'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,

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.

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