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Huntington's Chorea Scenario

These pages are demonstration pages of mixed mode deli very for an on-campus course. The curriculum and course structure has changed substantially for 1998, but these pages have been retained here as an example of a particular use of the Web in teaching.

Huntington's Chorea is caused by an autosomal dominant gene near the short arm of chromosome 4. There is a recently available accurate test to determine if you are a carrier. The disease has close to 100% penetration in carriers. The disease has an insidious onset in mid-life (around fourth decade - after reproduction for most women and many men). Family members do not always have accurate information on family history because many families are accepting of "odd" relatives, or hush up knowledge of mental disorder.

Symptoms: (Average duration from onset to death is 13 - 16 years)

• Motor:
  • akinesia (slowness in initiation of movement)
  • chorea (uncontrolled non-goal-directed movements)
  • bradykinesia (slowness in execution of goal-directed movement)
• Cognitive:
  • dementia (loss of memory and general intellectual loss)
  • personality change
  • psychiatric symptoms
  • marked psychotic features (e.g. paranoia)

Huntington's-related Sites

• R.Laycock's Huntington's Page
• Western PA Chapter of the HDSA
• Facing Huntington's Disease
• Huntington's Disease Society of America

Scenario for Role Play

You are presenting as a young couple, one of whom has an aunt recently diagnosed as having Huntington's Chorea. This is a late-onset disease that occurs in approximately 50% of offspring. Your cousins have been advised not to have children in case they are carriers. You had been planning to start your own family, but are now worried that

1. you might develop the disease
2. you might pass it on to the child

What are your options?

Would you want to know whether you carry the gene if a reliable test were available?