Huntington Disease Motor Control & Treatment - Erickson

Question 1

What is the clinical phenotype of HD?

Answer 1

• Problems with motor control
  
  • appear restless, fidgety, seem restless
  • chorea, motor impersistence
  • dysphagia, dysarthria
• Psychiatric issues
  
  • depression, anxiety, aggression, irritability, sleep disturbances, mania
• Cognitive decline
  
  • executive dysfunction (decisions, multitasking, insight)
  • ultimately dementia

Question 2

What is the pathological phenotype of HD?

Answer 2

• Neuronal loss in caudate and putamen with gliosis
• Loss of nigrostriatal GABA projection neurons
  
  • Decreased GPi inhibition of thalamus
  • Increased cortical motor output

Question 3

What are the two types/areas of treatment in HD?

Answer 3

Symptomatic (medications)

&

Supportive (multidisciplinary clinics)

Question 4

What are the two broad classes of drugs used to treat chorea and/or psychiatic symptoms?

Answer 4

Dopamine blockers & Dopamine depleters

Question 5

What are the Dopamine Blocker classes of drugs and specific drugs used to treat HD?

Answer 5

• Typical Antipsychotics
  
  • Haloperidol - DA (D2) antagonist
• Atypical Antipsychotics
  
  • Olanzapine
  • Risperidone
  • Clozaril - DA (D4) antagonist

Question 6

What is the Dopamine Depleter drug for treatment of HD?

Answer 6

• Tetrabenazine
  
  • Monoamine depleting agent
  • Highest binding density is in the caudate, putamen, and nucleus accumbens

Question 7

What medications are used soley to help treat the psychatric symptoms of HD?

Answer 7

• Valproic acid (Depakote)
  
  • enhances GABA effects
  • mood stability/weight gain
• Lithium (Lithobid)
  
  • Influences re-uptake of 5HT, NE
  • Affects post-synaptic D2 receptor
  • Effective against mania
• SSRI’s
• Benzodiazepines
  
  • first line treatment for chorea
  • effective doses often cause sedation

Question 8

In the multidisciplinary support team, who usually usually diagnoses HD, prescribes medications, manages medical treatment, educates the patient, and is the multidisciplinary support team leader?

Answer 8

Neurologist

Question 9

In the multidisciplinary support team, who usually usually obtains a detailed family history and provides counseling to at-risk family members?

Answer 9

Geneticist

Question 10

In the multidisciplinary support team, who usually performs the swallow assessment, makes recommendation for dysphagic diet/feeding tubes, and performs a speech evaluation?

Answer 10

Speech Language Pathologist

Question 11

In the multidisciplinary support team, who usually assesses caloric intake, educates about high calorie diets, and makes recommendations as dietary texture changes are needed?

Answer 11

Dietician

Question 12

In the multidisciplinary support team, who usually performs a safety assessment and assesses the patient for assistive devices?

Answer 12

OT

Question 13

In the multidisciplinary support team, who usually evaluates gait and fine motor function and makes mobility recommendations?

Answer 13

PT

Question 14

In the multidisciplinary support team, who usually assists with disability, advanced health care directives, living wills, legal/financial/housing issues; assesses county services; and facilitates nursing home placement?

Answer 14

Social Services (social worker)

Question 15

In the multidisciplinary support team, who usually provides emotional/spiritual support for patient/family and assists with advance directives and end of life planning?

Answer 15

Chaplain

Question 16

In the multidisciplinary support team, who usually assesses disability and/or competency, provides family counseling, and assists with medications?

Answer 16

Psych!

• Neuropsychologist
  
  • disability/competency
• Psychologist
  
  • family counseling
• Psychiatrist
  
  • meds