Neurology: Case of the Month

Test Yourself — Patient 40

Stiff-Person Syndrome

1. Which of the following is a diagnostic feature of the classical stiff-person syndrome?
   • [ A ] Rigidity with superimposed spasms
   • [ B ] Rigidity with myoclonus
   • [ C ] Dystonia and intermittent spasms
   • [ D ] Depression and spasms
2. Which of the following endocrine abnormalities have been described in patients with stiff-person syndrome?
   • [ A ] Insulin-dependent diabetes mellitus
   • [ B ] Hypothyroidism
   • [ C ] Premature ovarian dysfunction
   • [ D ] All of the above
3. Which of the following electromyographic findings stongly suggests a diagnosis of stiff-person syndrome?
   • [ A ] Increased motor unit activity on exercise
   • [ B ] Decreased muscle activity or electromyographic silence
   • [ C ] Signficant decrement or repetitive nerve stimulation
   • [ D ] Increased spontaneous motor unit activity which cannot be abolished on relaxation
4. Which of the following autoantibodies have been associated with the stiff-person syndrome?
   • [ A ] Anti-glutamic acid decarboxylase (GAD)
   • [ B ] Anti-amphiphysin
   • [ C ] Anti-thyroglobluin
   • [ D ] A and B
   • [ E ] All of the above
5. Which of the following treatments would be LEAST useful for patients with the stiff-person syndrome?
   • [ A ] Anticonvulsants
   • [ B ] Benzodiazepines
   • [ C ] Glucocorticoids
   • [ D ] Plasma exchange
   • [ E ] Buspirone

 

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