DMOP: a Dutch study specifies the evolutionary profile of the disease

Oculopharyngeal muscular dystrophy (or OPDM) is a relatively rare myopathy transmitted on an autosomal dominant mode. It results in the gradual onset, as a rule from the age of fifty, of a progressive and bilateral eyelid ptosis, swallowing disorders and, very often, a muscle deficit of the pelvic girdle. The progression of symptoms is slow but varies from one person to another. 

In an article published in August 2021, Dutch researchers looked at the natural history of the disease as a prelude to possible treatment trials. Muscle strength measurements, functional tests and a quality of life questionnaire (the SF-36) were used in a representative sample of 43 people with BPD living in the Netherlands. The observation period, spanning 20 months, was long enough to show significant deterioration in certain muscle groups, including the deltoid, quadriceps, ilio-psoas and lingual muscle. The ten staircase test and the MFM D1 score also performed unfavorably, as did the SF-36. The authors emphasize the interest of studying dynamometric parameters in future clinical trials.


Longitudinal Assessment of Strength, Functional Capacity, Oropharyngeal Function, and Quality of Life in Oculopharyngeal Muscular Dystrophy. Kroon RHMJM, Kalf JG, de Swart BJM et al. Neurology. 2021 (Août)