Neurology: Case of the Month

Test Yourself — Patient 33

Lubag Dystonia Parkinsonism

1. The cardinal features of Parkinsonism may be seen in which of the following?
   • [ A ] Lubag dystonia parkinsonism
   • [ B ] Huntington's chorea
   • [ C ] Hallervorden-Spatz disease
   • [ D ] Olivo-ponto-cerebellar atrophy
   • [ E ] All of the above
   • [ F ] None of the above
2. All of the following statements about Lubag dystonia parkinsonism are TRUE, EXCEPT:
   • [ A ] It is an autosomal recessive condition with variable penetrance.
   • [ B ] The condition is largely restricted to inhabitants of several Philippine islands.
   • [ C ] It occurs primarily in young to middle age males.
   • [ D ] Gliosis and neuronal loss are seen in the striatum pathologically.
   • [ E ] Dystonia is common, especially in patients who develop the disease at a young age.
3. True statements regarding treatment for Lubag dystonia parkinsonism include which of the following?
   • [ A ] Dopamine agonists have proven to be highly effective for control of the parkinsonian symptoms.
   • [ B ] Levodopa is the treatment of choice for all signs and symptoms of this disease.
   • [ C ] Physical therapy is of no benefit for patients with this condition.
   • [ D ] Botulinum toxin may be used effectively for focal dystonias.
   • [ E ] None of the above is correct.
4. Pathological findings in Lubag include which of the following?
   • [ A ] Neuronal loss primarily in the substantia nigra
   • [ B ] Diffuse distribution of Lewy bodies
   • [ C ] Gliosis and neuronal loss in the caudate and putamen
   • [ D ] Diffuse cortical neuronal loss and presence of Bunina bodies
   • [ E ] Diffuse cortical and subcortical neuron loss and the presence of Lubag cells
5. Which of the following diseases is not correctly matched with its genetic defect?
   • [ A ] Huntington's chorea — CAG repeat expansion located at 4p16.3
   • [ B ] DRPLA — CTG repeat expansion located at 12p12
   • [ C ] Lubag dystonia parkinsonism — CTG repeat expansion located at 22q14
   • [ D ] Fragile X syndrome — CGG repeat expansion located at Xq27.3
   • [ E ] Familial Alzheimer's disease — missense mutation in presenilin 1 gene located at 14q24.3

 

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