Introduction - FSH
Facioscapulohumeral muscular dystrophy (FSH) affects facial (facio), shoulder (scapul) and upper arm (humeral) muscles, although the legs can sometimes be affected too. Two previously used terms for FSH are:
FSH is the third most common muscular dystrophy (after Duchenne and myotonic dystrophies). In the UK it's estimated that one person in every 50,000, and probably closer to one in 20,000, has FSH, accounting for between about 1,200 and 3,000 cases at any one time.
FSH is a muscle wasting condition that affects both men and women. The condition is caused by a deletion (loss) of a piece of genetic material (DNA) towards the end of chromosome 4. This area contains a number of repeated units of DNA. Normal individuals have between 10 and 100 of these repeats whereas those with FSH have less that nine. In general, the fewer the number of repeats, the more severe the condition.
The degree of muscle weakness varies greatly from person to person - 10 to 20% of people eventually require a wheelchair while up to 30% can remain unaware of symptoms into old age. The majority of people affected by FSH though will present symptoms somewhere in between these two points.
FSH is inherited as an autosomal dominant pattern.
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- Landouzy-Dejerine
- facioscapuloperoneal muscular dystrophy
FSH is the third most common muscular dystrophy (after Duchenne and myotonic dystrophies). In the UK it's estimated that one person in every 50,000, and probably closer to one in 20,000, has FSH, accounting for between about 1,200 and 3,000 cases at any one time.
FSH is a muscle wasting condition that affects both men and women. The condition is caused by a deletion (loss) of a piece of genetic material (DNA) towards the end of chromosome 4. This area contains a number of repeated units of DNA. Normal individuals have between 10 and 100 of these repeats whereas those with FSH have less that nine. In general, the fewer the number of repeats, the more severe the condition.
The degree of muscle weakness varies greatly from person to person - 10 to 20% of people eventually require a wheelchair while up to 30% can remain unaware of symptoms into old age. The majority of people affected by FSH though will present symptoms somewhere in between these two points.
FSH is inherited as an autosomal dominant pattern.
Back to FSH (Facioscapulohumeral muscular dystrophy) homepage.

