2.9 Neurological and Sensory HUNTINGTONS

Question 1

Huntington’s Disease

pathophysiology

Answer 1

pathophysiology

Genetic disorder - autosomal dominant

Onset of symptoms @ 30-50years old

Degenerative & Fatal - long duration of illness 15-20 yrs.

Abnormal folded proteins

Damage to basal ganglia which regulates voluntary motor movement, learning, decision making, cognition, & emotion

http://eurohuntington.org/what-is-hd/

Question 2

Huntington’s Disease

manifestations

Answer 2

manifestations

Abnormal muscle movement
-progressive loss of control of movement

• chorea (a neurological disorder characterized by jerky involuntary movements affecting especially the shoulders, hips, and face)
• Intellectual decline
• impaired interaction and communication, memory and attention deficits

Emotional disturbances

• depression
• personality change – can be aggressive, paranoid, unstable mood

Progressive – late stage can’t walk, talk, or eat,
cognitive impairments

Question 3

Huntington’s Disease

DIAGNOSIS:

Answer 3

DIAGNOSIS:
DNA testing
Number of abnormal copies varies & predicts severity of disease
Genetic counseling

Question 4

Huntington’s Disease

TREATMENT

Answer 4

TREATMENT IS SYMPTOMATIC ONLY- no way to delay onset or slow progression
Treating chorea:

https://www.youtube.com/watch?v=UKKhZNyD3fg

Question 5

Huntington’s Disease

Nursing care

Answer 5

Nursing care

Care planning is significant – long duration of illness & family may be dealing with multiple ill members

Risk for Injury

Imbalanced Nutrition – less than body requirements

Risk for aspiration

Impaired mobility – risks due to constant, uncontrolled movements

Anxiety/Grieving

Impaired communication

Disturbed thought processes

Impaired social interaction