Question 347 · LEK · Spinal muscular atrophy

Select the correct statement(s) about spinal muscular atrophy (SMA):

  1. A. Werdnig-Hoffmann disease (SMA I) is the most common type.
  2. B. typical onset of Kugelberg-Welander disease (SMA III) is >18 months of age.
  3. C. without causal treatment the prognosis in Werdnig-Hoffmann disease (SMA I) is poor.
  4. D. Kugelberg-Welander disease (SMA III) is typically associated with muscle tremor.
  5. E. all answers are correct.
Hint

Recall the SMA classification by age of onset and best motor milestone, and the typical signs of lower motor neuron disease.

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