The nurse is admitting a child with Werdnig-Hoffmann disease (spinal muscular atrophy type 1). Which signs and symptoms are associated with this disease?
a. Spinal muscular atrophy
b. Neural atrophy of muscles
c. Progressive weakness and wasting of skeletal muscle
d. Pseudohypertrophy of certain muscle groups
ANS: C
Werdnig-Hoffmann disease (spinal muscular atrophy type 1) is the most common paralytic form of floppy infant syndrome (congenital hypotonia). It is characterized by progressive weakness and wasting of skeletal muscle caused by degeneration of anterior horn cells. Kugelberg-Welander disease is a juvenile spinal muscular atrophy with a later onset. Charcot-Marie-Tooth disease is a form of progressive neural atrophy of muscles supplied by the peroneal nerves. Progressive weakness is found of the distal muscles of the arms and feet. Duchenne muscular dystrophy is characterized by muscles, especially in the calves, thighs, and upper arms, which become enlarged from fatty infiltration and feel unusually firm or woody on palpation. The term pseudohypertrophy is derived from this muscular enlargement.
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