Tuesday, March 27, 2007

De Gaper or Yawning man: Blepharospasm-Oromandibular Dystonia?

Meige’s Syndrome is also known as Breughel’s disease. So, what about it? Hmm. It is the dreaded dystonia, again. That’s what!!

Okay, this is what a couple of little nasties can do for you when you least expect it! We are talking about a combination of oromandibular dystonia and blepharospasm. So what are they, I hear you say? When both these forms of dystonia present together the result is called Meige’s Syndrome.

Firstly, oromandibular dystonia may affect the face, tongue, palate and jaw. It presents with abnormal mouth or tongue movements, grimacing expressions or forced opening or closing of the mouth. The many small muscles around the areas affected make this type of dystonia difficult to treat. Secondly, blepharospasm results from eye muscles spasming thus causing the eyelids to close suddenly without warning.

Blepharospasm is a form of dystonia that frequently starts with the eyelids blinking uncontrollably. Once the spasms close the eyelid, the person experiencing this may have great difficulty opening the eye or eyes again until the spasms ease. Vision is not affected. However, sight is seriously impaired when the eyelid is resistant to opening! Sunlight is known to aggravate this condition.

The name Breughel’s disease comes from the 16th Century Flemish painter, Breughel. He managed to portray the syndrome on canvas. In some cases, people with this form of dystonia are also affected by dysphonia, resulting from spasming vocal cords. Treatment is possible to relieve distressing symptoms associated with this rare kind of dystonia.

Botox treatment has proven successful for many people over a long period of time. The doses given are very small so there is seldom accumulation of antibodies. Surgery may also be an option for some people. However, both forms of treatment only provide relief of symptoms. At this time, there is no cure.

On-going research is needed to find a cure for all forms of dystonia. Dystonia research has not as yet attracted sufficient funding to allow appropriate follow up to the studies already underway.

Sue Bayliss. Cairns, Australia.

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