Huntington Disease Motor Control & Treatment - Erickson Flashcards Preview

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Flashcards in Huntington Disease Motor Control & Treatment - Erickson Deck (16)
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1
Q

What is the clinical phenotype of HD?

A
  • 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
2
Q

What is the pathological phenotype of HD?

A
  • Neuronal loss in caudate and putamen with gliosis
  • Loss of nigrostriatal GABA projection neurons
    • Decreased GPi inhibition of thalamus
    • Increased cortical motor output
3
Q

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

A

Symptomatic (medications)

&

Supportive (multidisciplinary clinics)

4
Q

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

A

Dopamine blockers & Dopamine depleters

5
Q

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

A
  • Typical Antipsychotics
    • Haloperidol - DA (D2) antagonist
  • Atypical Antipsychotics
    • Olanzapine
    • Risperidone
    • Clozaril - DA (D4) antagonist
6
Q

What is the Dopamine Depleter drug for treatment of HD?

A
  • Tetrabenazine
    • Monoamine depleting agent
    • Highest binding density is in the caudate, putamen, and nucleus accumbens
7
Q

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

A
  • 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
8
Q

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?

A

Neurologist

9
Q

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

A

Geneticist

10
Q

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

A

Speech Language Pathologist

11
Q

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

A

Dietician

12
Q

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

A

OT

13
Q

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

A

PT

14
Q

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?

A

Social Services (social worker)

15
Q

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

A

Chaplain

16
Q

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

A

Psych!

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