Question

Werdnig-Hoffmann disease presents as a 'floppy baby' with marked hypotonia and tongue fasciculations. Infants have a flaccid 'frog-like' posture. What is the name of this disease?

1) Werdnig-Hoffmann disease
2) Down syndrome
3) Cerebral palsy
4) Muscular dystrophy

Answer 1

Werdnig-Hoffmann disease, also known as Spinal Muscular Atrophy type 1 (SMA1), is a genetic neuromuscular disorder characterized by severe muscle weakness and marked hypotonia. Infants with this disease display a flaccid 'frog-like' posture.

Step-by-step explanation:

Werdnig-Hoffmann disease, also known as Spinal Muscular Atrophy type 1 (SMA1), is the correct answer to the question. It is a genetic neuromuscular disorder that primarily affects the muscles used for movement (skeletal muscles) and results in severe muscle weakness.

Infants with this disease often exhibit marked hypotonia (low muscle tone) and tongue fasciculations (small muscle twitches). They also have a characteristic flaccid 'frog-like' posture.