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Limb-girdle muscular dystrophy or Erb’s muscular dystrophy is an autosomal class of muscular dystrophy that is similar but distinct from Duchenne muscular dystrophy and Becker’s muscular dystrophy. Limb-girdle muscular dystrophy encompasses a large number of rare disorders.
The term “limb-girdle” is used to describe these disorders because the muscles most severely affected are generally those of the hips and shoulders — the limb girdle muscles.
Common symptoms of limb-girdle muscular dystrophy are muscle weakness, myoglobinuria, pain, myotonia, cardiomyopathy, elevated serum CK, and rippling muscles.
The muscle weakness is generally symmetric, proximal, and slowly progressive.
Generally pain is not present with LGMD, and mental function is not affected.
LGMD can begin in childhood, adolescence, young adulthood or even later. The age of onset is usually between 10 and 30. Both genders are affected equally. When limb-girdle muscular dystrophy begins in childhood the progression appears to be faster and the disease more disabling. When the disorder begins in adolescence or adulthood the disease is generally not as severe and progresses more slowly.
The distal muscles are affected late in LGMD, if at all. Over time (usually many years), the person with LGMD loses muscle bulk and strength. Eventually, s/he may need a power wheelchair or scooter, especially for long distances.
While LGMD isn’t a fatal disease, it may eventually weaken the heart and lung muscles, leading to illness or death due to secondary disorders.
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