Opmd is a rare hereditary muscular dystrophy
with onset usually in the mid forties to the mid fifties, although the
abnormal gene is present from birth. The main symptoms are drooping
of the eyelids (ptosis), and difficulty swallowing (dysphasia) - initially
mainly dry and solid foods. Later fluids may prove difficult and this
can include saliva. Care must be taken to avoid aspiration of food or
saliva as this may lead to chest infections or pneumonia. Problems may
also be experienced with fatigue, facial weakness, the voice, and the
muscles of the shoulders and hips. Mobility may be affected.
Some surgery is available to alleviate the symptoms.
Ptosis can be treated by a 'nip and tuck' operation on the eyelids,
but increasingly a frontalis sling is available. This involves stitches
in the eyelid, eyebrows, and forehead. The muscles of the forehead lift
the eyelids. Dysphasia can be helped by dilation of the throat, or by
a cricopharyngeal myotomy which involves cutting one of the throat muscles
internally.
The heredity factor means that each child of
an affected person has a 50% chance of inheriting the disorder. Either
sex can be affected: diagnosis is now usually by a blood test.
In the UK and the rest of Europe opmd is rare.
It is much more common in Canada due to an affected family who emigrated
from Poitou in France in the C17th. It spread widely in Canada, particularly
in Quebec where it is reported to be 1 per 1000 of the population.