albert wallace deckel phd, abn university of connecticut medical school professor of...

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




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)



1. Lability and the inability to inhibit impulses (one drink too many) due to frontal circuits not working correctly in HD




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.


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.


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



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.




1.Lability


3. The inability to judge how one relates to others PLUS anosognosia (the inability to realize how you are acting)





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.


Depression/ Irritability(reduction of serotonin and/or norepinephrine—BDNF story in

HD)

Dysphoria

Agitation

Depression

Irritability

Anxiety



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)



IF ANTIDEPRESSANT’S DON’T WORK, TREAT THE IRRITABILITY WITH

NEUROLEPTICS ESP. IF ARE CHOREIC MOVEMENTS

Rage, outbursts, violence, verbal abuse:

Risperdal, Seroquel



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




1.Lability


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


• 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)




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.




1.Lability


3. The inability to judge how one relates to others






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



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.

albert wallace deckel phd, abn university of connecticut medical school professor of...

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