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Self-Assessment Questions


Ataxia in Children: Review Questions

David Hsu, MD, PhD

Dr. Hsu is an assistant professor of pediatric neurology,
University of Wisconsin Medical School, Madison, WI.

Choose the single best answer for each question.

1. A 6-year-old boy awakens in his normal state, but by noon becomes so unsteady that he cannot walk. On examination, he looks scared but is well-spoken and cooperative. He has mild nystagmus on lateral gaze bilaterally, past-pointing on finger-nose-finger test, and normal reflexes. Strength is intact, but he cannot balance while standing, and his gait is wildly lurching. He developed chicken pox 10 days ago, but he is otherwise healthy. Developmental milestones are normal. What is this child’s most likely diagnosis?
  1. Acute postinfectious cerebellitis
  2. Friedreich ataxia (FRDA)
  3. Medulloblastoma
  4. Miller Fisher syndrome
  5. Multiple sclerosis
Click here to compare your answer.

2. A 2-year-old girl has sudden attacks of falling multiple times a day. During these attacks, she “walks like she’s drunk,” looks scared, and turns pale, but there is no loss of consciousness. Her parents have noticed that sometimes with an attack, her eyes will appear to “wobble.” She will grab at the walls, and, when on the floor, grab at the floor. If she falls asleep after onset of an attack, she wakes up normal. Vomiting also helps relieve symptoms transiently. She has these episodes about once every few weeks to few months, in between which she is normal. Physical examination is normal. What is this child’s most likely diagnosis?

  1. Benign paroxysmal positional vertigo
  2. Benign paroxysmal vertigo (BPV) of childhood
  3. Brainstem encephalitis
  4. Ependymoma
  5. Frontal lobe epilepsy
Click here to compare your answer.

  3. An 11-year-old boy still cannot ride a bike and has become increasingly clumsy over the past 4 to 5 years. A distant uncle was also clumsy and died in young adulthood of heart disease. Physical examination shows dysarthric speech, head thrusts with visual tracking, past-pointing on finger-nose-finger test, disorganization on rapid pronation-supination of the hand, jerky and illegible handwriting, and lurching gait. Deep tendon reflexes are not elicitable. What test is most likely to be diagnostic in this child?
  1. Magnetic resonance imaging (MRI) of the brain and lumbar puncture
  2. Electromyogram (EMG)/neurologic deficit score (NCS)
  3. Lipid panel, apolipoproteins A and B
  4. Test for trinucleotide repeat of FRDA gene
  5. Plasma amino acids and urine organic acids
Click here to compare your answer.

4. A 4-year-old girl presents for involuntary writhing movements in her arms with small, random, superimposed twitches. She has a history of asthma and frequent pneumonia and sinusitis. Physical examination shows hypometric saccades, forced blinking to initiate saccades, truncal ataxia, and choreoathetosis. Skin examination shows telangiectasias on the bulbar conjunctivae, on the tops of the ears, along the malar prominences of the face, and within the flexor creases of the elbows and knees. What test is most likely to be diagnostic?

  1. Alpha-fetoprotein (AFP) and carcinoembryonal antigen (CEA)
  2. Chest radiograph
  3. EMG/NCS
  4. MRI of the brain and lumbar puncture
  5. Urine for catecholamines
Click here to compare your answer.


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