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Bethlem Myopathy a rare form of Muscular Dystrophy

 

Bethlem Myopathy can present itself from birth till later adulthood.  BM is usually noticed when contractures of the ankles, fingers, elbows or other joints are present.  Individuals usually have limited physical stamina and strength.  Most of the muscles are effected by this disease with the exception of the heart muscle.  BM is usually a very slowly progressive disease where 2/3 of individuals will need some sort of mobility aid by age 50.  Some individuals may have breathing problems due to the weakness of muscles that aid the breathing process.  The degree of severeness varies.  BM patients often exhibit odd scarring like keloids or raised cigarette paper type scars of the skin.

There are other types of MD that can present symptoms like BM.  One of the main features of BM is that most patients will have contractures of the fingers, which is usually not seen in other forms of MD.

 

 

paraphrased from various articles and personal experience by Mike Newton

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