Transcript Slide 1

HD EDUCATION DAY, 11-7-09
Copyright 2009, Dr. Albert W. Deckel
Walking on Egg Shells in Huntington’s Disease:
Albert Wallace Deckel PhD, ABN
University of Connecticut Medical School
Professor of Psychiatry/Neuroscience (Retired)
Neuropsychology Services
Neuropsych Services, 96 W Main Street, Northboro, MA
Tele: 508-393-3820
Walking on Eggshells in HD Families
Copyright 2009, Dr. Albert W. Deckel
HD: THE PERFECT STORM
1. Lability of emotions
2. Depression/Irritability
3. Social Judgment. The inability to judge
how one relates to others PLUS
anosognosia (inability to realize how you’re
acting)
4. Inability to perceive “disgust” on faces
Walking on Eggshells in HD Families
Copyright 2009, Dr. Albert W. Deckel
HD: THE PERFECT STORM
1. Lability of emotions :
Overly express your emotions
If you think it you say it or do it.
If you feel it you express it.
If you want it you take it.
A two year old has extreme lability.
(one drink too many)
Walking on Eggshells in HD Families
Copyright 2009, Dr. Albert W. Deckel
1. Lability and the inability to inhibit impulses
(one drink too many) due to frontal circuits
not working correctly in HD
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
1. Lability
A little anger becomes a lot and turns into a fight
Annoyance becomes a rage, verbal or otherwise
A small disagreement spirals out of control
Loud/active/messy children provoke major irritation
Moderate stress becomes a disaster.
Copyright 2009, Dr. Albert W. Deckel
Treatment of lability requires a
coordinated plan of attack by the person
with HD, the family and the mental
healthcare staff (a behavioral plan)
1.Identify triggers.
2.Modify the environment accordingly.
Copyright 2009, Dr. Albert W. Deckel
Treatment of lability should begin:
EARLY, EARLY, EARLY in the process
1. Early development of coping strategies.
2. Time out/space for when emotions rise.
3. Structuring the environment.
4. Use of simple, structured behavioral
therapy.
a. Identify triggers.
b. Modify the environment accordingly.
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
1. For rages, verbal abuse, physical abuse,
the CAREFUL use of appropriate
medications.
2. NOT a druged, sedated, lethargic patient—
and not a rageful, abusive, out of control
person.
3. The goal is a calm, controlled mind.
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
1. Lability
2. Depression/Irritability
3. The inability to judge how one relates to
others PLUS anosognosia (the inability to
realize how you are acting)
4. Inability to perceive “disgust” on faces
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
2. Depression/ irritability
Journal of Neurology, Neurosurgery, and Psychiatry 2001;71:310-314;
RESULTS Ninety eight per cent of the patients exhibited
neuropsychiatric symptoms, the most prevalent being
dysphoria, agitation, irritability, apathy, and anxiety. Symptoms
ranged from mild to severe and were unrelated to dementia and
chorea.
Walking on Eggshells in HD Families
Depression/ Irritability
(reduction of serotonin and/or norepinephrine—BDNF story in
HD)
Dysphoria
Agitation
Depression
Irritability
Anxiety
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
TREAT THE 1’ST SIGNS OF DEPRESSION
AGGRESSIVELY
At the 1’st sign of
depression in HD gene
positive people, treat
aggressively with
antidepressants:
Zoloft
Celexa (serotonin)
Effexor (serotonin + norepi)
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
IF ANTIDEPRESSANT’S DON’T WORK,
TREAT THE IRRITABILITY WITH
NEUROLEPTICS ESP. IF ARE CHOREIC
MOVEMENTS
Rage, outbursts, violence,
verbal abuse:
Risperdal, Seroquel
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
SOME LOGICAL CONCLUSIONS
1. No traditional family therapy. It throws fuel
on the fire and the fire is difficult to put out.
2. Start medicines early, adjust them
frequently—avoid, avoid, avoid oversedation.
3. Identify triggers.
4. Modify the environment accordingly.
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
1. Lability
2. Depression/Irritability
3. Social judgment. The inability to judge
how one relates to others PLUS
anosognosia (the inability to realize you
have a problem)
4. Inability to perceive “disgust” on faces
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
Medial dorsal prefrontal
cortex—an important site
of “social judgment”—how
am I relating to the outside
world? (Grafman and
others)
• Anasognosia—neurologically you can’t see what you are
doing wrong
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
Copyright 2009, Dr. Albert W. Deckel
HD: THE PERFECT STORM
4) If you can’t see it happening can you change it?
This is why its important to start early in treating and
anticipating this progression of HD. If you have
anosognosia, you won’t believe it when your
psychologist or family tells you that your reacting
in a way that isn’t right. But this won’t seem right
to you. Why believe him or her when “your eyes”
tell you something different?
a. Identify the need (HD person may not see it)
b. Modify the environment.
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
1. Lability
2. Depression/Irritability
3. The inability to judge how one relates to
others
4. Inability to perceive “disgust” on faces
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
HD: THE PERFECT STORM
4. Inability to perceive “disgust” on faces
Brain (1996), 119, 1647-1665
Loss of disgust
Perception of faces and emotions in Huntington's disease
Reiner Sprengelmeyer,1 Andrew W. Young,1 Andrew J. Calder,1 Anke
Karnat,3 Herwig Lange,3
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
HD: THE PERFECT STORM: The antidote
1. Early intervention with your HD team.
2. Aggressive treatment of
depression/irritability with antidepressants
3. Use the neuroleptics if there is violence or
“injury”, emotional or physical.
4. Develop behavioral plan: Identify triggers,
modify the environment accordingly.