Neurology: Case of the Month

Test Yourself — Patient 88

X-linked dystonia-parkinsonism (Lubag syndrome, DYT3)

1. What is the mode(s) of hereditary transmission in Lubag syndrome?
   • [ A ] Autosomal Dominant
   • [ B ] Autosomal Recessive
   • [ C ] X-Linked Dominant
   • [ D ] X-Linked Recessive
   • [ E ] Both A and B
2. Lubag syndrome must be considered in any male afflicted with a progressive movement disorder with ancestral ties to which country?
   • [ A ] Haiti
   • [ B ] The Philippines
   • [ C ] Madagascar
   • [ D ] Maldives
   • [ E ] Indonesia
3. One would expect which abnormal laboratory result from a patient diagnosed with Lubag syndrome?
   • [ A ] Low levels of serum ceruloplasmin
   • [ B ] Macrocytic anemia
   • [ C ] Elevated liver enzymes
   • [ D ] Low levels of serum ferritin
   • [ E ] None of the above
4. In the majority of patients with Lubag syndrome, the dystonia remains focal and does not generalize.
   • [ A ] True
   • [ B ] False
5. The best first option for treatment of a patient with Lubag syndrome would be:
   • [ A ] Deep brain stimulation to the bilateral Gpi
   • [ B ] Only carbidopa/levodopa works for symptoms
   • [ C ] Carbidopa/levodopa, an anticholinergic, or a benzodiazepine
   • [ D ] Nothing works for symptomatic treatment

 

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