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

VisualWikitext

Revision as of 03:45, 14 June 2024

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(1). These are different but closely related moment disorder(2, 3).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,(4, 5).

Epidemiology

The disease appears between the ages of 30-70 years , with the mean onset age 55.7 years(6). It is more 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.(1).

Etiology (7, 8)

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

Genetic distonia: It is caused by gene variant including TOR1A, TOR2A, GNAL, OR1A, REEP4, THAP1, respectively. TOR1A and THAP1plays a significant role to the pathophysiology of the diseases.

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

Presentations (9-11)

Blepharospam affect the eyelid and increase blinking along with ocular pain but due to diseases progress to oromandibular dystonia patient experience involuntary tongue movements and have 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 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 syndrome is rare diseases it is normally diagnosed using a patient's medical history, external observations, and neurological examination. There is no particular test to identify Meige syndrome, a number of 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(6).

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 administered on the basis of patient's medical condition and history. Treatment options include surgery(e.g partial resection of the periorbital muscle, which surround 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(2). and rehabilitative treatment (include physiotherapy, occupational therapy) (12) and BoNT injection it is used as a primary treatment option for MS it help to reduced muscle spasm(10). . When other treatment are failed to control the disease then Deep brain stimulation of the globus pallidus interna (GPi) is a therapeutic approach(13).

References

External links

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de:Meige-Syndrom nl:Brueghelsyndroom

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@@ Line 18: / Line 18: @@
 :''Not to be confused with [[Meigs syndrome]]''
-'''Meige's syndrome''' is a type of [[dystonia]]. It is also known as '''oral facial dystonia'''. It is actually a combination of two forms of dystonia, blepharospasm and oromandibular dystonia (OMD).
+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(1). These are different but closely related moment disorder(2, 3).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,(4, 5).
-When OMD is combined with Blepharospasm, it may be referred to as Meige’s Syndrome named after Henri Meige,<ref>{{WhoNamedIt|synd|547}}</ref> the French neurologist who first described the symptoms in detail in 1910. The symptoms usually begin between the ages of 40 and 70 years old and appear to be more common in women than in men. The combination of upper and lower dystonia is sometimes called cranial-cervical dystonia.
+== '''Epidemiology''' ==
+The disease appears between the ages of 30-70 years , with the mean onset age 55.7 years(6). It is more 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.(1).
-==Symptoms==
+== '''Etiology (7, 8)''' ==
-'''*Oromandibular Symptoms'''
+Alabenese et al. explain the etiology of Meige's syndrome and divide the condition into two categories “hereditary dystonia” and “acquired dystonia”.
-**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).
-'''*Blepharospasm symptoms'''
-**uncontrollable squinting/closing of eyes
-**light sensitivity(photophobic)
-**squinting/eyes closing during speech
-**uncontrollable eyes closing shut(rare instances completely causing blindness)
-In addition, in some patients, the dystonic spasms may sometimes be provoked by certain activities, such as talking, chewing, or biting. As discussed earlier, particular activities or sensory tricks may sometimes temporarily alleviate OMD symptoms, including chewing gum, talking, placing a toothpick in the mouth, lightly touching the lips or chin, or applying pressure beneath the chin.
+'''Genetic distonia''': It is caused by gene variant including TOR1A, TOR2A, GNAL, OR1A, REEP4, THAP1, respectively. TOR1A and THAP1plays a significant role to the pathophysiology of the diseases.
-==A few upper and lower dystonia types==
+'''Acquired distonia'''. It is caused by environmental factors such as medical condition including (trauma, cerebral palsay, stroke, and encephalopathy etc), medications and poisons including (heavy metal toxicity, antipsychotic medication and others).
-Blepharo means "eyelid". Spasm means "uncontrolled muscle contraction". The term blepharospasm ['blef-a-ro-spaz-m] can be applied to any abnormal blinking or eyelid tic or twitch resulting from any cause, ranging from dry eyes to Tourette's syndrome to tardive dyskinesia. The blepharospasm referred to here is officially called benign essential blepharospasm (BEB) to distinguish it from the less serious secondary blinking disorders. "Benign" indicates the condition is not life threatening, and "essential" is a medical term meaning "of unknown cause". It is both a cranial and a focal dystonia. Cranial refers to the head and focal indicates confinement to one part. The word dystonia describes abnormal involuntary sustained muscle contractions and spasms. Patients with blepharospasm have normal eyes. The visual disturbance is due solely to the forced closure of the eyelids.
-Oromandibular dystonia (OMD) is a form of focal dystonia that affects varying areas of the head and neck including the lower face, jaw, tongue and larynx.  The spasms may cause the mouth to pull open, shut tight, or move repetitively.  Speech and swallowing may be distorted.  It is often associated with dystonia of the cervical muscles (Spasmodic Torticollis), eyelids (Blepharospasm), or larynx (Spasmodic Dysphonia).
+== '''Presentations (9-11)''' ==
+Blepharospam affect the eyelid and increase blinking along with ocular pain but due to diseases progress to oromandibular dystonia patient experience involuntary tongue movements and have 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).
-In patients with OMD, involuntary contractions may involve the muscles used for chewing (masticatory muscles). These may include the thick muscle in the cheek that closes the jaw (buccinator muscles) and the broad muscle that draws back the lower jaw and closes the mouth (temporalis muscle). Some patients may also experience involuntary contractions of the wide muscle at the side of the neck that close the jaws. This muscle draws down the corner of the mouth and lower lip (platysmal muscles) or other muscle groups.
+'''Blepharospasm related symptoms  '''
-==Diagnosis==
+·       Eyelid fluttering
-Meige's is commonly misdiagnosed and most doctors will have not 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 blood test or MRI or CT scans. OMD by itself, maybe misdiagnosed as TMJ.
-==Presentation==
+·       Exaggerated blinking
-The main symptoms involve involuntary [[blinking]] and [[chin]] thrusting. Some patients may experience excessive tongue protrusion, squinting, light sensitivity, muddled speech, or uncontrollable contraction of the platysma muscle.  Some Meige's patients also have "laryngeal dystonia" (spasms of the [[larynx]]). Blepharospasm may lead to embarrassment in social situations, and oromandibular dystonia can affect speech, making it difficult to carry on the simplest conversations. This can cause difficulty in both personal and professional contexts, and in some cases may cause patients to withdraw from social situations.
-The condition tends to affect women more frequently than men.
+·       Apraxia  of eyelid opening
+·       Involuntary closing of 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 syndrome is rare diseases it is normally diagnosed using a patient's medical history, external observations, and neurological examination. There is no particular test to identify Meige syndrome, a number of 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(6).
 ==Treatment==
-There is no cure for Meige's Syndrome, nor can it be prevented, except in cases where it is caused by medication.  Although [[palliative]] treatments are available, such as [[Botox]] injections. In some instances, acupuncutre has been known to help. Even though it is a form of alternative medicine, there have been some reports of acupuncture minimizing the effects of Meige's.
+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 administered on the basis of patient's medical condition and history. Treatment options include surgery(e.g partial resection of the periorbital muscle, which surround 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(2). and rehabilitative treatment (include physiotherapy, occupational therapy) (12) and BoNT injection it is used as a primary treatment option for MS it help to reduced muscle spasm(10). . When other treatment are failed to control the disease then Deep brain stimulation of the globus pallidus interna (GPi) is a therapeutic approach(13).
 ==See also==