Which of the following is characterized by denervation atrophy of the muscles?
Correct Answer: Werdnig-Hoffman disease
Description: Werdnig-Hoffman disease is an autosomal recessive, congenital, muscular hypotonia characterized by marked neurogenic muscular atrophy. This condition is also called "infantile spinal muscular atrophy" and is primarily due to the destruction of the anterior horn cells in the spinal cord. All other diseases listed are metabolic myopathies, representing inborn errors of carbohydrate metabolism (McArdle and Pompe disease), lipid metabolism (carnitine palmityl transferase deficiency), and purine metabolism (myoadenylate deaminase deficiency). Ref - Harrison's internal medicine 20e pg 3147
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