HD:

Cognitive Disorders

Cognitive Impairment in HD

Intellectual speed and flexibility
Visuo-spatial sense
Learning strategies (attention, sequencing, memory)
Executive function (organization, regulation, awareness)

Often impaired in motor skill learning
“Unconscious” tasks may require concentration
Strategy: verbalize steps of a task

• ORGANIZATION:
  • attention, decision making, planning, sequencing
• REGULATION:
  • initiation, repetition, temper control
• UNAWARENESS:
  • “denial” of deficits, unintentional “non-compliance”

Difficulty in planning and initiation
Organization and sequencing impaired

Cognitive Deficits attributed to caudate pathology
Verbal fluency
Ability to shift mental sets
Perseverative tendencies

Executive Function Deficits: Regulation in HD

Initiation (getting started)
Try:
Gently guide patient
Offer help to get started
Maintain daily routine
Avoid labeling patient as “lazy”
Educate family and friends

Repetition (getting “stuck”, being stubborn”)
Try:
Gently shift focus away from repetition
Use humor to “unstick”
Establish time limit
Use distraction to shift attention

• Bradyphrenia: slowed thinking
  • Try:
    • allow patient enough time to answer or react
    • be patient during long pauses
    • maintain focus on person
    • limit distractions in environment
• Visual-Spatial Deficit: impaired body sense; loss of body awareness; loss of body in space
  • Try:
    • orient to new neighborhood
    • write directions
    • use signs
    • avoid independent travel in new areas
• Learning: attention deficits; difficulty sequencing complex learned tasks; difficulty learning new tasks
  • Try:
    • break complex information into simple steps
    • write down the steps
    • encourage practice
    • allow extra time to learn
• Memory
  • Try:
    • provide cues, hints, missing word
    • avoid open-ended questions
    • offer choice of two alternatives
    • use "to do" losts or written reminders
    • maintain daily routine
    • establish schedule and calendar
• Attention
  • Try:
    • assign only one task at a time
    • provide a quiet environment
    • maintain eye contact
• Decision Making
  • Try:
    • offer limited choices
    • use short sentences
    • avoid open-ended options
    • avoid power struggles
• Planning, Sequencing, Prioritizing
  • Try:
    • structure tasks
    • organize information numerically, spatially, and so on
    • maintain routines
    • write down activities
• Initiation (getting started)
  • Try:
    • gently guide patient
    • offer help to get started
    • maintain daily routine
    • avoid labelling patient as "lazy"
    • educate family and friends
• Repetition (getting "stuck", being "stubborn")
  • Try:
    • gently shift focus away from repetition
    • use humor to "unstick"
    • establish time limit
    • use distraction to shift attention
• Temper Control
  • Try:
    • redirect from source of anger
    • avoid confrontation or ultimatum
    • keep self and environment calm
    • safety is priority
    • identify "triggers" of outbursts
    • maintain daily routine
    • accept anger and frustration as valid feelings
• Anosognosia (lack of self-awareness) or "denial": unable to recognize deficits; unaable to evaluate own behavior; unintentionally "noncompliant"
  • Try:
    • accept as untreatable part of HD
    • evaluate why it needs to be addressed
    • ask person to choose incentives for compliance
    • involve person in formal written agreement