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Huntington disease

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Slowly progressive, hereditary brain disease that causes changes in movement, ... Aricept, Reminyl, memantine, others. Apathy. Structure, behavioral treatments ... – PowerPoint PPT presentation

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Title: Huntington disease


1
Huntington disease
  • Vicki Wheelock MD
  • HDSA Center of Excellence at UC Davis

2
Definition of HD
  • Slowly progressive, hereditary brain disease that
    causes changes in movement, thinking and behavior
  • Onset commonly ages 30-50
  • Diagnosis is made by neurologist based on
    symptoms and examination, and may be confirmed by
    a blood test for genetic testing

3
Understanding the symptoms of HD.
  • Gene changes lead to. brain changes

4
Genetics of HD
GENE
Protein
Normal number lt31 CAG Gray area 32-38
CAG Huntington disease gt 38 CAG
5
Brain changes in HD
Aylward EH. Brain Research Bulletin
200362137-141
MRI scan
Microscopic view
6
Huntington Disease Stages
  • Stage 1
  • Slightly lower performance at work independent
    at home
  • Stage 2
  • Can still work (lower level), still mostly
    independent at home
  • Stage 3
  • Difficult to work, starts to needs help with
    financial, home activities
  • Stage 4
  • Unable to work. Needs major assistance with care
  • Stage 5
  • Full-time nursing care required

7
Symptoms in HD
MOTOR
COGNITIVE
EMOTIONAL
8
Behavior changes in HD
Starting/stopping activities
Multi-tasking
Creativity
Episodic anger Irritability
Organizing Concentrating Prioritizing
Memory
Changes in sleep/wake cycle
  • Normal
  • Advanced HD

Impulsiveness
9
Movement changes in HD
Chorea involuntary movements
Balance problems
Slow eye movements
Restless, fidgets
  • Normal
  • Advanced HD

Slowness of movement
Fine motor tasks
Trouble swallowing
10
Motor disorder
  • Too much movement
  • Motor restlessness
  • Chorea
  • Dystonia
  • Tremor
  • Too little movement
  • Slowness of movement

11
Motor symptomsLoss of control of movement
  • Decreased control, especially in
  • Planning and selecting
  • Sequencing
  • Learning new movements
  • Performing movements during interference

12
Chorea repetitive, involuntary fragments of
movement
  • Grimacing
  • Blinking or opening eyes
  • Mouth, lip or tongue movements
  • Arm, hand movements
  • Trunk, hips, pelvis move
  • Leg movements
  • Mostly seen during relaxed state or when focusing
    on other things

13
Slowness of movement in HD
  • Loss of facial expression
  • Slow to stand from seated position
  • Difficulty turning over in bed
  • Finger and hand movements are slower
  • Buttoning
  • Writing
  • Eating

14
Change in balance and falls
  • Loss of balance due to minor tripping is
    sometimes an early HD symptom
  • More often, falls dont occur until later stages
    of HD
  • Chorea can make it easier to fall
  • TIPS to help
  • Balance exercises
  • Slow down!
  • Use vision to compensate look where you are
    walking, light up the runway between bedroom
    and bathroom at night
  • Be careful when carrying things

15
Swallowing problems
  • Assessment by speech and language therapist is
    extremely helpful
  • Specific advice about strategies to reduce
    choking
  • Head position
  • Textures of food
  • Medications have limited role ?Prozac

16
Thinking changes in HD
  • Early Stage
  • Normal IQ only specialized tests show problems,
    but work/home performance may decline
  • Middle Stage
  • Memory, visuospatial deficits, impaired judgment
  • Late Stage
  • The person with HD is aware, but communication is
    difficult

17
The Cognitive Disorder Executive Function
  • Organizing
  • Prioritizing
  • Controlling impulses
  • Monitoring self-awareness
  • Beginning and ending activities
  • Creative thinking
  • Problem-solving

18
Emotional problems in HD
  • Depression 40 - 80
  • Anxiety 30 - 40
  • Obsessions/compulsions 10 - 20
  • Irritability common
  • Apathy 20
  • Episodic anger common
  • Psychosis 5

19
What can we do to help people with HD?
H S GHuntington Study Group
SET-HD
HDSA
20
Treatments for HD
  • No definite treatments exist now to help slow
    down HD
  • Research is leading to possible treatments
  • Minocycline
  • Cysteamine
  • Ethyl EPA (fish oil)
  • Coenzyme Q10
  • Creatine
  • Others..

21
Symptomatic treatment for HD
  • Motor symptoms
  • Cognitive symptoms
  • Emotional/behavioral symptoms
  • Swallowing problems
  • Nutrition

22
Motor symptoms
  • Chorea
  • Amantadine, Klonopin, Zyprexa, Haldol, Prolixin,
    Tetrabenazine
  • Slowness, rigidity
  • Amantadine, Sinemet
  • Dystonia
  • Klonopin, Botox

23
Behavioral/thinking symptoms
  • Memory problems
  • Aricept, Reminyl, memantine, others
  • Apathy
  • Structure, behavioral treatments
  • Stimulant medications
  • Irritability
  • Seek triggers, set structure
  • ?Depressed or anxious treat those symptoms
  • Episodic anger
  • See above
  • Sometimes mood stabilizers (Zyprexa)

24
Emotional symptoms
  • Depression
  • SSRI meds (Prozac, Paxil, Zoloft, Welbutrin etc)
  • Anxiety
  • SSRI meds, sometimes Klonopin
  • Obsessions/compulsions
  • SSRI meds
  • Paranoia, delusions (false beliefs),
    hallucinations
  • Antipsychotics (Zyprexa, Prolixin, Haldol,
    Risperdal, etc)

25
Resources for families and patients
  • www.HDSA.org
  • www.huntingtonproject.org
  • www.hdsanortherncalifornia.org
  • www.HDteam.org
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