Meige's syndrome

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Meige's syndrome
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This 16th-century painting by Flemish artist Pieter Bruegel the Elder is one of the oldest known depictions of the facial dystonia associated with this syndrome.
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Meige's syndrome is a type of dystonia. It is also known as Brueghel's syndrome and oral facial dystonia. It is actually a combination of two forms of dystonia, blepharospasm and oromandibular dystonia (OMD).

Contents

When OMD is combined with blepharospasm, it may be referred to as Meige's Syndrome named after Henri Meige, [1] the French neurologist who first described the symptoms in detail in 1910. The symptoms usually begin between the ages of 30 and 70 years old and appear to be more common in women than in men (2:1 ratio). The combination of upper and lower dystonia is sometimes called cranial-cervical dystonia. [2] The incidence is about one case in 20,000 people. [3]

Presentation

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.[ citation needed ]

The condition tends to affect women more frequently than men.[ citation needed ]

Symptoms

Oromandibular Symptoms

Blepharospasm symptoms

In addition, in some patients, the dystonic spasms may sometimes be provoked by certain activities, such as talking, chewing, or biting. 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.[ citation needed ]

Diagnosis

Meige's is commonly misdiagnosed and most doctors will not be familiar with this condition due to its rare incidence. Usually, a neurologist specializing in psychomotor disorders can detect Meige's. There are currently no technological diagnostic tools to detect Meige's, as it cannot be identified using blood chemistry analysis or radiological imaging, such as MRI or CT scans. A patient presenting with signs of OMD alone may be misdiagnosed with TMJD.[ citation needed ]

Patients with idiopathic Meige's syndrome do not quickly respond to anticholinergic drug treatments, a diagnostic sign that can help differentiate it from acute dystonia, which does respond to anticholinergic drugs. [2]

Upper and lower dystonia types

The Greek word blepharon 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.[ citation needed ]

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).[ citation needed ]

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 (masseter muscle) 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 (platysmal muscle). This muscle draws down the corner of the mouth and lower lip or other muscle groups.[ citation needed ]

Treatment

In some cases Meige's syndrome can be reversed when it is caused by medication. Botulinum toxin injections can be helpful for the blepharospasm and for masseter spasm. [4]

See also

Related Research Articles

This is a glossary of medical terms related to communication disorders which are psychological or medical conditions that could have the potential to affect the ways in which individuals can hear, listen, understand, speak and respond to others.

A spasm is a sudden involuntary contraction of a muscle, a group of muscles, or a hollow organ, such as the bladder.

<span class="mw-page-title-main">Myoclonus</span> Involuntary, irregular muscle twitch

Myoclonus is a brief, involuntary, irregular twitching of a muscle, a joint, or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus describes a medical sign and, generally, is not a diagnosis of a disease. It belongs to the hyperkinetic movement disorders, among tremor and chorea for example. These myoclonic twitches, jerks, or seizures are usually caused by sudden muscle contractions or brief lapses of contraction. The most common circumstance under which they occur is while falling asleep. Myoclonic jerks occur in healthy people and are experienced occasionally by everyone. However, when they appear with more persistence and become more widespread they can be a sign of various neurological disorders. Hiccups are a kind of myoclonic jerk specifically affecting the diaphragm. When a spasm is caused by another person it is known as a provoked spasm. Shuddering attacks in babies fall in this category.

Writer's cramp or focal hand dystonia (FHD) is an idiopathic movement disorder of adult onset, characterized by abnormal posturing and movement of the hand and/or forearm during tasks requiring skilled hand use, such as writing. Overcontraction of affected muscles, cocontraction of agonist and antagonist pairs, and activation of muscles inappropriate to a task all impair use of the affected hand.

<span class="mw-page-title-main">Dystonia</span> Neurological movement disorder

Dystonia is a neurological hyperkinetic movement disorder in which sustained or repetitive muscle contractions occur involuntarily, resulting in twisting and repetitive movements or abnormal fixed postures. The movements may resemble a tremor. Dystonia is often intensified or exacerbated by physical activity, and symptoms may progress into adjacent muscles.

Dyskinesia refers to a category of movement disorders that are characterized by involuntary muscle movements, including movements similar to tics or chorea and diminished voluntary movements. Dyskinesia can be anything from a slight tremor of the hands to an uncontrollable movement of the upper body or lower extremities. Discoordination can also occur internally especially with the respiratory muscles and it often goes unrecognized. Dyskinesia is a symptom of several medical disorders that are distinguished by their underlying causes.

<span class="mw-page-title-main">Hyperkinesia</span> Excessive movements due to basal ganglia dysfunction

Hyperkinesia refers to an increase in muscular activity that can result in excessive abnormal movements, excessive normal movements, or a combination of both. Hyperkinesia is a state of excessive restlessness which is featured in a large variety of disorders that affect the ability to control motor movement, such as Huntington's disease. It is the opposite of hypokinesia, which refers to decreased bodily movement, as commonly manifested in Parkinson's disease.

<span class="mw-page-title-main">Palatal myoclonus</span> Spasms of the palatal (roof of the mouth) muscles

Palatal myoclonus is a rare condition in which there are rhythmic jerky movements or a rapid spasm of the palatal muscles. Chronic clonus is often due to lesions of the central tegmental tract.

Focal dystonia, also called focal task-specific dystonia, is a neurological condition that affects a muscle or group of muscles in a specific part of the body during specific activities, causing involuntary muscular contractions (spasms) and abnormal postures. There are many different types of focal dystonia, each affecting a different region of the body. For example, in focal hand dystonia, or writer's cramp, the fingers either curl into the palm or extend outward without control. In musicians, the condition is called musician's focal dystonia, or simply, musician's dystonia. In sports, it may be involved in what is commonly referred to as the yips. The condition appears to be associated with over-training, and individualized treatment strategies may involve medications, retraining techniques, and procedures.

<span class="mw-page-title-main">Fasciculation</span> Spontaneous, involuntary muscle twitch

A fasciculation, or muscle twitch, is a spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. They are common, with as many as 70% of people experiencing them. They can be benign, or associated with more serious conditions. When no cause or pathology is identified, they are diagnosed as benign fasciculation syndrome.

Torsion dystonia, also known as dystonia musculorum deformans, is a disease characterized by painful muscle contractions resulting in uncontrollable distortions. This specific type of dystonia is frequently found in children, with symptoms starting around the ages of 11 or 12. It commonly begins with contractions in one general area such as an arm or a leg that continue to progress throughout the rest of the body. It takes roughly 5 years for the symptoms to completely progress to a debilitating state.

Blepharospasm is a neurological disorder characterized by intermittent, involuntary spasms and contractions of the orbicularis oculi (eyelid) muscles around both eyes. These result in abnormal twitching or blinking, and in the extreme, sustained eyelid closure resulting in functional blindness.

<span class="mw-page-title-main">Spasmodic torticollis</span> Medical condition

Spasmodic torticollis is an extremely painful chronic neurological movement disorder causing the neck to involuntarily turn to the left, right, upwards, and/or downwards. The condition is also referred to as "cervical dystonia". Both agonist and antagonist muscles contract simultaneously during dystonic movement. Causes of the disorder are predominantly idiopathic. A small number of patients develop the disorder as a result of another disorder or disease. Most patients first experience symptoms midlife. The most common treatment for spasmodic torticollis is the use of botulinum toxin type A.

<span class="mw-page-title-main">Propantheline bromide</span> Drug for functional gastrointestinal disorders

Propantheline bromide (INN) is an antimuscarinic medication used for the treatment of excessive sweating (hyperhidrosis), cramps or spasms of the stomach, intestines (gut), or bladder, and involuntary urination (enuresis). It can also be used to control the symptoms of irritable bowel syndrome (IBS) and similar conditions. This medication can also be used for patients who experience intense gastrointestinal symptoms while tapering off of tricyclic antidepressants.

<span class="mw-page-title-main">Myokymia</span> Involuntary quivering of a muscle

Myokymia is an involuntary, spontaneous, localized quivering of a few muscles, or bundles within a muscle, but which are insufficient to move a joint. One type is superior oblique myokymia.

Synkinesis is a neurological symptom in which a voluntary muscle movement causes the simultaneous involuntary contraction of other muscles. An example might be smiling inducing an involuntary contraction of the eye muscles, causing a person to squint when smiling. Facial and extraocular muscles are affected most often; in rare cases, a person's hands might perform mirror movements.

Pleurothotonus, commonly known as Pisa syndrome, is a rare neurological disorder which occurs due to prolonged exposure to antipsychotic drugs. It is characterized by dystonia, and abnormal and sustained involuntary muscle contraction. This may cause twisting or jerking movements of the body or a body part. Although Pisa syndrome develops most commonly in those undergoing long-term treatment with antipsychotics, it has been reported less frequently in patients receiving other medications, such as an acetylcholinesterase inhibitor. However, it has also been seen in those with other diseases causing neurodegeneration and in those who are not receiving any medication. The characteristic development of Pisa syndrome consists of two types of dystonia: acute dystonia and tardive dystonia. The underlying pathology of drug-induced Pisa syndrome is very complex, and development may be due to an underlying dopaminergic-cholinergic imbalance, or serotonergic/noradrenergic dysfunction.

Oromandibular dystonia(OMD) is an uncommon focal neurological condition affecting the jaws, face, and mouth. Oromandibular dystonia is characterized by involuntary spasms of the tongue, jaw, and mouth muscles that result in bruxism, or grinding of the teeth, and jaw closure. These conditions frequently lead to secondary dental wear as well as temporomandibular joint syndrome. In addition, problems with chewing, speaking, and swallowing may result from jaw opening, involuntary tongue movements, or jaw deviation.

In ophthalmology, apraxia of lid opening (ALO) is an inability to initiate voluntary opening of the eyelid following a period of eyelid closure, with normal function at other times. Manual lifting of the eyelid often resolves the problem and the lid is able to stay open.

Alan Brown Scott was an American ophthalmologist specializing in eye muscles and their disorders, such as strabismus. He is best known for his work in developing and manufacturing the drug that became known as Botox, research described as "groundbreaking" by the ASCRS.

References

  1. synd/547 at Who Named It?
  2. 1 2 3 Ananth J, Edelmuth E, Dargan B (April 1988). "Meige's Syndrome Associated with Neuroleptic Treatment". American Journal of Psychiatry. 145 (4): 513–515. doi:10.1176/ajp.145.4.513. PMID   2894781.
  3. Boodman, Sandra G. (April 23, 2012). "Medical mysteries: I opened my laptop and my eyes snapped shut". Washington Post. Retrieved April 24, 2012.
  4. Czyz, Craig N; Burns, John A; Petrie, Thomas P; Watkins, John R; Cahill, Kenneth V; Foster, Jill A (2013). "Long-term Botulinum Toxin Treatment of Benign Essential Blepharospasm, Hemifacial Spasm, and Meige Syndrome". American Journal of Ophthalmology. 156 (1): 173–177.e2. doi:10.1016/j.ajo.2013.02.001. PMID   23541393.