Huntingtons Disease

Question 1

Huntingtons Overview / Introduction

Answer 1

• Incurable
• Adult-onset
• Progressive
• Neurodegenerative disorder
• Presents with involuntary movements, dementia, and behavioral changes

Question 2

Huntingtons Pathophysiology

Answer 2

• Degeneration and atrophy of basal ganglia and cerebral cortex
• Neurotransmitters become deficient and are unable to modulate movement
• Hereditary and dominate gene

Question 3

Huntingtons Clinical Presentation

Answer 3

• 35-45 years of age (can begin at any age from childhood to old age)
• Movement disorder, cognitive disorder, and behavioral disorder
• Early stages have short term memory, followed by motor dysfunction and a variety of cognitive changes in the intermediate stage of dementia

Question 4

Huntingtons Movement Disorder

Answer 4

• Chorea (restless, fidgeting, progressive, uncontrollable writhing, flailing of extremities)
• As disease progresses, chorea is replaced by ataxia, bradykinesia, rigidity, and postural instability
• Late disease features spasticity, clonus, and extensor plantar response
• Dysarthria and dysphagia are common
• Abnormal eye movements seen early in disease

Question 5

Huntingtons Cognitive Effects

Answer 5

• Cognitive decline in characteristic
• Dementia and psychiatric features are frequently the earliest symptoms
• Early onset behavioral changes, such as irritability, untidiness, and loss of interest
• Slowing of cognition, impairment of intellectual function, and memory disturbances usually occur later

Question 6

Huntingtons Mood Effects

Answer 6

• Depression
• Small percentage of patients experience bipolar, psychosis, hallucinations, delusions, and schizophrenia
• Changes in personality

Question 7

Huntingtons Diagnosis

Answer 7

• Measurment between bicaudate diameter (distance between heads of two caudate nuclei) by CT Scan or MRI
• Genetic testing

Question 8

Huntingtons Medical Management

Answer 8

• No therapy or medication available that will delay the onset of symptoms or prevent progression
• Control of symptoms with meds required during middle and late stages
• Chorea = Benzodiazepines (slows brain activity to produce a calming or drowsy effect, commonly used to treat anxiety, insomnia, seizures, and muscle spasms)
• Rigidity and Bradykinesia = Levodopa
• Depression = SSRIs, antidepressants
• Hallucinations or Delusions = Anti-psychotics

Question 9

Huntingtons PT Management

Answer 9

• These pt’s require extra time to complete tasks
• Gait re-education
• Balance retraining
• Fall prevention
• Aerobic capacity
• Muscle strengthening
• w/c prescription and training
• Respiratory function
• Task specific reach, grasp, and manipulation

Question 10

Huntingtons Outcome Measures

Answer 10

• TUG
• Berg Balance
• Tinneti Mobility Test