Neurology: Case of the Month

Test Yourself — Patient 41

Hallervorden-Spatz syndrome

  1. Hallervorden-Spatz syndrome and Wilson's disease may be reliably distinguished on the basis of WHICH of the following clinical features:
    • [ A ] Bulbar onset and predominance of extrapyramidal features
    • [ B ] Predominance of dystonia in Hallervorden-Spatz syndrome
    • [ C ] Predominance of ataxia in Hallervorden-Spatz syndrome
    • [ D ] Autosomal recessive inheritance pattern
    • [ E ] None of the above
  2. Which of the following statements is MOST correct?
    • [ A ] In Hallervorden-Spatz syndrome, lenticular degeneration results from excessive systemic iron accumulation.
    • [ B ] Iron chelating agents may produce significant clinical improvements in patients with the Hallervorden-Spatz syndrome.
    • [ C ] The excessive iron accumulation observed in patients with Hallervorden-Spatz syndrome may be secondary to injury of the striatum or other basal ganglia by the disease process.
    • [ D ] MRI evidence of excessive iron accumulation in the globus pallidus or putamen is rarely observed in patients with diagnoses other than Hallervorden-Spatz syndrome.
  3. Which of the following clinical features in a child would be MOST suggestive of a diagnosis of Hallervorden-Spatz syndrome?
    • [ A ] Macrocephaly
    • [ B ] Dystonia
    • [ C ] Pigmentary retinopathy
    • [ D ] Proximal weakness
  4. The presence of a clinical response to levadopa in a child with a dystonic movement disorder makes the diagnosis of dopa-responsive dystonia highly likely.
    • [ A ] True
    • [ B ] False
  5. Which of the following features would be MOST likely to rule out a diagnosis of Hallervorden-Spatz syndrome?
    • [ A ] Maternal pattern of inheritance
    • [ B ] Normal or near-normal MRI of the brain
    • [ C ] Hispanic ethnic background
    • [ D ] Normal cognitive function
    • [ E ] Extensor plantar responses

 

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