Which condition is characterized by early contractures before weakness?

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Multiple Choice

Which condition is characterized by early contractures before weakness?

Explanation:
Early joint contractures before evident muscle weakness point to Emery-Dreifuss muscular dystrophy. In this form, tight tendons around the elbows, Achilles tendons, and the back of the neck appear in childhood, restricting movement long before substantial proximal weakness shows up. Only afterward do the muscles begin to weaken and waste become noticeable. This pattern helps distinguish it from other muscular dystrophies: Duchenne tends to start with proximal weakness as the first sign; myotonic dystrophy features myotonia and a broader myopathy with distal involvement and systemic features; facioscapulohumeral MD commonly presents with scapulohumeral weakness and facial weakness early on. The hallmark sequence—contractures appearing early, with weakness later—best matches Emery-Dreifuss muscular dystrophy.

Early joint contractures before evident muscle weakness point to Emery-Dreifuss muscular dystrophy. In this form, tight tendons around the elbows, Achilles tendons, and the back of the neck appear in childhood, restricting movement long before substantial proximal weakness shows up. Only afterward do the muscles begin to weaken and waste become noticeable. This pattern helps distinguish it from other muscular dystrophies: Duchenne tends to start with proximal weakness as the first sign; myotonic dystrophy features myotonia and a broader myopathy with distal involvement and systemic features; facioscapulohumeral MD commonly presents with scapulohumeral weakness and facial weakness early on. The hallmark sequence—contractures appearing early, with weakness later—best matches Emery-Dreifuss muscular dystrophy.

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