Huntington's Disease
Background
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Autosomal dominant inherited disease characterized by progressive neurodegeneration
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Clinical features: choreiform movements, depression, & dementia
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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
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Bulbar dysfunction with risk of aspiration
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Dementia, potentially uncooperative, end of life care, advanced directives, DNR status
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Anesthetic medications:
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All IV anesthetics OK, caution with slower recovery
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NDMRs are ok, but may require ↓ dose
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Succinylchonline is ok, but duration may be longer due to ↓ plasma cholinesterase activity
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Neuraxial/regional is ok, document pre-existing deficits
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Preoperative sedation using butyrophenones such as droperidol or haloperidol may be helpful in controlling choreiform movements