MEIGE'S SYNDROME: A PARKINSONISMCategory: Newsworthy Notes
An Encyclopedia Of Other Diseases And Conditions Resembling Parkinson’s Disease Addendum
In the August 2015 issue we listed a great number of A-Typical, or ParkinsonISMS, or diseases that mimic Parkinson’s. Through our work, we’ve found another.
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).
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).
In more than half of all people with benign essential blepharospasm, the symptoms of dystonia spread beyond the eyes to affect other facial muscles and muscles in other areas of the body. When people with benign essential blepharospasm also experience involuntary muscle spasms affecting the tongue and jaw (oromandibular dystonia), the combination of signs and symptoms is known as Meige syndrome.
When OMD is combined with blepharospasm, it may be referred to as Meige’s Syndrome named after Henri Meige, 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. The incidence is about one case in 20,000 people.
• 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)
• The first symptom to appear is an increased rate of blinking
• Uncontrollable squinting/closing of eyes
• Light sensitivity (photophobia)
• 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. 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.
Meige’s is commonly misdiagnosed and most doctors will have not seen this condition before. Usually a neurologist who specializes in movement disorders, or a dentist who is an expert in craniofacial pain or oral facial pain (see Wellness Village below) can detect Meige’s. There is no way to detect Meige’s by blood test or MRI or 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.
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.
Treatment: In some cases Meige’s syndrome can be reversed when it is caused by medication. It has been theorized that it is related to cranio-mandibular orthopedic misalignment, a condition that has been shown to cause a number of other movement disorders (Parkinson’s, Tourette’s, and 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.
Information gathered from Dr. David Shirazi, DDS, MS, LAc, MA, RPSGT, DABCP, DABCDSM, The National Institutes of Health, and Wikipedia, the free encyclopedia.
To find a dentist who is an expert in craniofacial pain or oral facial pain go to http://www.ParkinsonsResource.org/tmj-dentist/