Huntington's Disease 

 

 

Background 

 

  • Autosomal dominant inherited disease characterized by progressive neurodegeneration

  • Clinical features: choreiform movements, depression, & dementia

  • Onset is typically between 35-40 years of age, but can be as late as 80 years. The disease continues to progress for several years with death occuring 17-20 years after diagnosis & is usually from malnutrition or aspiration pneumonitis

 

 

Considerations

 

  • Bulbar dysfunction with risk of aspiration 

  • Dementia, potentially uncooperative, end of life care, advanced directives, DNR status 

  • Anesthetic medications: 

    • All IV anesthetics OK, caution with slower recovery 

    • NDMRs are ok, but may require ↓ dose

    • Succinylchonline is ok, but duration may be longer due to ↓ plasma cholinesterase activity

    • Neuraxial/regional is ok, document pre-existing deficits 

  • Preoperative sedation using butyrophenones such as droperidol or haloperidol may be helpful in controlling choreiform movements