albert wallace deckel phd, abn university of connecticut medical school professor of...
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Albert Wallace Deckel PhD, ABN
University of Connecticut Medical School
Professor of Psychiatry/Neuroscience (Retired)
Neuropsychology Services
Walking on Egg Shells in Huntington’s Disease:
Neuropsych Services, 96 W Main Street, Northboro, MA
Tele: 508-393-3820
HD EDUCATION DAY, 11-7-09
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
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
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
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)
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
1. Lability and the inability to inhibit impulses (one drink too many) due to frontal circuits not working correctly in HD
Copyright 2009, Dr. Albert W. Deckel
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
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.
Treatment of lability should begin:
EARLY, EARLY, EARLY in the process
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/ irritabilityJournal 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
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 over-sedation.
3. Identify triggers.
4.Modify the environment accordingly.
SOME LOGICAL CONCLUSIONS
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 facesCopyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
• Anasognosia—neurologically you can’t see what you are
doing wrong
Medial dorsal prefrontal cortex—an important site of “social judgment”—how am I relating to the outside world? (Grafman and others)
Copyright 2009, Dr. Albert W. Deckel
Walking on Eggshells in HD Families
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.
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
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, 1647-1665Loss of disgustPerception of faces and emotions in Huntington's diseaseReiner 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.
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