This post is about FSH called faciohumeroscapular)
(FSHMD, FSHD or FSH), and it also known as Landouzy-Dejerine, This
is a common form of muscular dystrophy impacting approximately 7 out of every
100,000 people. This form of muscular dystrophy in inherited from one of
the parents and effects the skeletal muscles of the face (facio), upper arms
(humeral) and the scapula (scapula)
The disease was discovered in 1884 by French
physicians Louis Landouzy and Joseph Dejerine. In their research paper of 1886, Landouzy
and Dejerine drew attention to the fact that this form of muscular dystrophy
ran in family groups. Although, there are cases where there is no
family history of muscular dystrophy.
Symptoms usually
develop in early childhood. Approximately, 95% of the individuals have
symptoms are that are apparent starting in their teen years. This
disease advances with progressive weakness to the face, shoulders and upper
arms. It also may impact other skeletal muscles.
FSH is characterized
at first by weakness in the facial and shoulder muscles. Then over a
period it can spread to other muscle groups. Muscle weakness in the
pelvis and hips can make it difficult to climb stairs or walk for extended
distances.
The person in the picture above is a classic example of FSH. Note that the weak shoulder muscles tend
to make the shoulder blades protrude from the back, a common sign
known as scapular winging. Due to the weakness in muscles of the shoulders and upper arms it can be difficult to raise the arms over the head or throw a ball.
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