Huntington's Disease

Question 1

Etiology

Answer 1

Hereditary neurological condition that leads to severe physical and mental disabilities. Causes progressive loss of nerve cells in the brain, affecting movement, cognition, emotions, and behavior

Question 2

Signs and Symptoms

Answer 2

Motor Difficulties: Involuntary movement patterns, voluntary movement patterns (bradykinesia, akinesia, incoordination); hypertonicity; gait and balance problems; decreased hand-eye coordination; large burst of movement when small movements are intended

Deterioration of cognitive and behavioral abilities- forgetfulness, difficulty concentrating, decreased sequencing and memory, irritability and depression, slowing of saccadic eye movement and occular pursuits, dysphagia

Question 3

Progression

Answer 3

progression occurs over a 15-20 year period eventually requiring long-term care or hospitalization. Death results from secondary causes.

Question 4

Medical Management of Dementia

Answer 4

medical management addresses symptoms

Haldoperidol is used to decrease chorea during functional activity

Question 5

Effects on Occupational performance

Answer 5

Environmental change or additional task demands compromise OP; deterioration of cognition may lead to dismissal from employment; may have difficulty sequencing tasks; loss of function may contribute to depression; performance of voluntary motor tasks is slowed; initiation of task is compromised

Question 6

OT Evaluation

Answer 6

The Unified Huntington’s Disease Rating Scale assesses changes in the areas of motor function, cognitive function, and functional capacity and behavioral abnormalities
OT should include functional daily living skills, cognitive abilities, motor performance, strength, personal interests, and values

Question 7

OT Intervention

Answer 7

Early = address memory and concentration, establish daily routine, avoid open-ended questions, word association, worksite evaluation, home and work environmental modification, encourage support groups and community mobility, total body HEP, home safety

Middle = engagement in purposful activity, arrange for others to handle finances, family to use simple written cues or words to promote completion of self-care, ambulatory device, positioning and AE during feeding, oral motor exercises, dietary consistency, list of steps for self-care tasks, DME and AE for ADLs, dressing aids and reduction of fasteners

Late = pay attention to positioning, use splinting to prevent contractures, smooth transition to tube feeding, environmental controls into place, consistent daily schedules and routine.