2. Learning objectives
At the end of this lecturer, students
should be able to:
• Define Huntington’s disease
• Explain the pathophysiology of Huntington’s
disease
• Enumerate the clinical manifestation of
Huntington’s disease
• List down the diagnostic evaluation
• Explain management of Huntington’s disease
3. DEFINITION
Huntington’s disease is a chronic, progressive,
hereditary disease of the nervous system
that results in progressive involuntary
choreiform movement and dementia
4. PATHOPHYSILOGY
• Premature death of cells in the
striatum of the basal ganglia, the
region deep within the brain involved
in the control of movement.
• There is also loss of cells in the
cortex.
5. Cont…
• The region of the brain associated
with thinking, memory, perception,
and judgment
• A building block for protein called
glutamine abnormally collects in the
cell nucleus, causing cell death.
6. Cont…
• The cells’ destruction results in a
lack of the neurotransmitters gamma
aminobutyric acid (GABA) and
acetylcholine, which inhibit nerve action
7. CLINICAL MANIFESTATION
• Abnormal involuntary movements
• Emotional disturbance
• A constant writhing, twisting,
• Speech, becoming slurred, hesitant
• Chewing and swallowing are difficult
• Akathisia (motor restlessness)
9. MANAGEMENT
• Pharmacology
• Thiothixene hydrochloride
• Galoperidol decanoate
• Predominantly block dopamine receptors,
improve the chorea in many patients
• Levodopa.
10. SUMMARY
So far, today we have discussed
about definition of Huntington’s disease
,pathophysiology clinical manifestation ,
diagnostic evaluation and management .
11. Bibliography
• Lewis et al, Medical Surgical Nursing, Mosby
Elsevier,7th edition.
• Joyce.M.Black et al, Medical Surgical
Nursing, Saunders publication.
• Brunner and Siddhartha, Medical Surgical
Nursing, Lippincott Williams and Wilkins.
