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13 Cards in this Set

  • Front
  • Back
How is Huntington's disease contracted?
dominantly inherited (genetic)
What is the specific cause of Huntington's disease?
abnormal polyglutamine expansion within protein Huntingtin
What part of the brain degenerates in Huntington's?
striatum
What type of neurons are targeted in Huntington's therapy?
dopamine neurons
What are the two mechanisms of dopamine therapy for Huntington's?
deplete central monoamines (dopamine)
block dopamine receptors
What are two (2) drugs that deplete central monoamines?
reserpine
tetrabenzene
What are two (2) drugs that block dopamine receptors?
perphenazine
haloperidol
Which drug is contraindicated in Huntington's?
levodopa
(exacerbates it)
Antidepressants with which type of side effects should be avoided in Huntington's?
antidepressants with significant cholinergic effects (fluoxetine, carbamazepine)
What might be the cause of ALS?
toxic gain of function mutation in superoxide dismutase
What is the pathophysiology of ALS?
degeneration of motor neurons of the spinal cord and the cortical neurons that provide input to them (muscle neurons)
What is responsible for death in most ALS patients?
respiratory compromise
What is used to treat ALS?
Baclofen - GABAb agonist helps with spasticity