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Barnes' syndrome
Associated persons:
Stanley Barnes
Description:
Also known as: Barnes' muscular dystrophy. A rare type of muscular dystrophy, in which muscles are often hypertrophic and stronger than normal, but later become weak and atrophic.
The Oxford Medicine: Stanley Barnes, in 1932, described 284 individuals in seven generations of one stock. Features of a curious myopathic dystrophy were present amongst the first five generations. The average age of onset is between 35 and 50 years. Males and females are affected in about equal numbers, and inheritance is of the dominant type. The disease is transmitted by males and females. The first stage is a true muscular hypertrophy, associated with abnormal strength and later weakness. Pseudo-hypertrophy follows, usually in middle life, and the muscles, though enlarged, are weak. The third stage is terminal and characterised by a spreading atrophy, beginning in the large muscles of the thighs and calves and ultimately involving the intrinsic muscles of the hands and forearms. The latter
groups of muscles waste without antecedent pseudo-hypertrophy. The disease progresses slowly. Loss of tendon reflexes occurs in the first stage and may be the only abnormal sign in some members of the stock. There is an almost constant association with adipositas universalis and a relative absence of polymorphism.
Bibliography:
- S. Barnes
A myopathic family, with hypertrophic, pseudohypertrophic, atrophic and terminal (distal in upper extremities) stages.
Brain, Oxford, 1932, 55: 1-46.
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