Using the Neuropsychological Exam and Other Tools to Help Those Struggling With the “Softer” Symptoms of Huntington’s Disease!



 

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Over the last seven years I have had the privilege of assisting hundreds and hundreds of person’s with Huntington’s Disease (Phds) get the Social Security Disability (SSD) and Long-Term Disability (LTD) benefits they deserved after being initially denied; helping those who were “wrongfully” arrested, accused of crimes, and even kicked out of grade school because of Huntington’s Disease (HD) and Juvenile Huntington’s Disease (JHD); and helping family members understand how much of their loved one’s behaviors and actions were being caused by HD!  Unfortunately, because of everyone’s overall unawareness of the “softer” symptoms (the emotional, behavioral, and cognitive symptoms) of HD and our unwillingness to accept them as probable cause for our loved ones actions, the Person with HD (Phd) and the rest their family members, ultimately end up suffering (financially and emotionally) more than necessary.  I have tried to analyze why most loving family members and friends have a hard time blaming HD for their loved ones questionable behavior and actions.  Here are a few of the reasons I’ve come up with.  As you can see, trying to answer these perplexing questions with our own understandings and paradigms sometimes prevents us from seeing that our loved one is simply a person suffering from HD: 

     1.  How much of their questionable behavior or actions can be attributed to HD and how much can be attributed to non-HD behavior and actions (not being able to understand what is happening to them; denial; fear of losing their independence and ability to work, drive and communicate, etc.)?

2.
  How can their mental and cognitive abilities be compromised when they still look okay physically?

3.  How much of their questionable behavior or actions are due to a combination of HD symptoms, current medications and non-HD-affected behavior or actions?
 

      4.  How can you be sure that your loved one wasn’t already becoming a drug addict, an alcoholic, a compulsive gambler, a cheater or a sexually promiscuousness person
before they started HD symptoms?
 

      5.  How can you be sure that the questionable behaviors or actions weren’t caused from the medications they were taking, or an interaction between their medications?  Some of the side-effects of medications read like the “softer” symptoms of HD themselves.           .
 

      6.  How can you tell how much of
your loved one’s behaviors and actions they can actually control?
 

      7.  How do you know when your loved
one is accountable for their behavior
and actions and when they’re not?  
 

      8.  Many were religious before and shouldn’t their “conscience” or “moral compass” prevent them from doing the questionable things that they’ve done?      
 

     9.  Notwithstanding all of these questions, what tools, if any, are out there that can provide everyone, including lawyers and judges, with enough compelling facts to prove, beyond reasonable doubt, that the behaviors and actions were actually HD-affected and when they started?

A lot of times all you have to do to answer these questions is to use the measuring stick called UNCHARACTERISTIC!   Are the questionable behavior or actions of your loved one uncharacteristic of prior behavior and actions?  By using uncharacteristic as the “measuring stick” to judge whether-or not current behavior or actions were different than prior behavior and actions is a great starting point.  When analyzed closely, virtually all of the criminal-looking behavior I note below was indeed uncharacteristic of everyone’s previous behaviors and actions!

Next, a thorough knowledge of the neuropsychiatric sequelae of HD’s “softer” symptoms is essential to shed light on the most destructive symptoms which, by themselves or in combination with one another, help get the majority of Phds in trouble with their spouses, their children, the law, other family members and friends and their employer! 

However, the most powerful tool we have to determine how much of our loved
one’s behavior and actions were HD-related and how much were not is the Neuropsychological exam:  “A neuropsychological evaluation is a comprehensive assessment of cognitive and behavioral functions using a set of standardized tests and procedures. Various mental functions are systematically tested, including, but not limited to:  Intelligence; Problem solving and conceptualization; Planning and organization
; Attention, memory, and learning; Language; Academic skills; Perceptual and motor abilities; and Emotions, behavior, and personality” (http://www.neuropsychologycentral.com/interface/content/resources/page_
material/resources_faq_pages/resources_neuropsych_eval_faq.html#a )

I’ll never forget Dr. Jane Paulson’s compassionate response five years ago when she spoke at our support group in Phoenix.  One of our caregivers asked her how they could tell if their husband’s recent behavior and actions were being adversely affected by Huntington’s Disease’s symptoms or if he was just being a mean SOB!  Jane’s compassionate response to this concerned caregiver has always stuck with me over the years.  She eloquently replied: “I would error on the side of compassion by blaming HD and then it really doesn’t matter, does it?”  How sublime and brilliant!  Blame HD- it’s our enemy, not our loved one who is merely suffering from it!

Unfortunately, convincing the criminal justice system and loved ones is not always that easy, especially since there is usually no outward sign that they are experiencing symptoms of HD because they have not begun to experience the traditional hallmark of HD- chorea, or they are just beginning to experience barely noticeable muscle vacillations, twitches or jerks.  Because the Phd still looks good physically, many medical professionals and family members rule out their questionable behavior as being HD-affected and mistakenly think that the Phd could not have been suffering from HD when they broke the law.  The problem is, in their ignorance, spouses and loved ones also tell the arresting officer and other family members the same thing, instead of that their loved one is simply suffering from HD.  This is a very hard paradigm to break, especially since it was only recently that the name of this debilitating disease was changed from Huntington’s Chorea to Huntington’s Disease in recognition that three-quarters of HD is not chorea!

I have found that the only way to effectively and impartially judge whether-or-not their questionable behaviors and actions are actually HD-related is to have the Phd take a Neuropsychological examination. 

I have to admit, before actually reading the results of the first Neuropsychological exams, even I had my doubts about whether-or-not there was any tool available to us that could prove that the questionable behavior and actions of the Phd were indeed HD-related enough so that would clear them wrong doing!  To my surprise, every Phd who has taken one has been completely exonerated!   The Neuropsychological exam even calculates an approximate time when symptoms began.  Fortunately, all of these calculated dates of onset were several years prior to the Phds beginning their behavior that appeared “deviant” and questionable.

Below are examples of how effective the Neuropsychological exam actually is as I have been able to use it to prove that an innocent Phd’s behavior and actions were actually being caused by HD’s “softer” symptoms.  Whether the Phd had been arrested for embezzling millions of dollars, possession of cocaine, for hitting a policeman (and police woman) after being wrongfully arrested, for threatening to “blow up his school” and “kill everyone” because of “uncharacteristic” behavior and actions or just simply turned down for Social Security or Long-Term Disability benefits because they still knew their name and who the President of the United States was, the Neuropsychological exam has proven to be a valuable tool in helping the person with HD and their families!  


Examples:

Criminal Behavior
Embezzling Millions:
One Phd was arrested for embezzling millions of dollars in two states. He had impeccably and ethically run his own insurance agency for over 20 years before letting someone convince him to sell millions of dollars of bogus bonds. After showing that his “deviant” behavior had started after the calculated time of onset by a Neuropsychological exam, his sentence was reduced from 25 years to just 18 months!

Striking Police Officers:
One Phd was arrested while walking home from the store because the officer though his staggering HD-affected gait meant that he was drunk in public. He got upset for being arrested wrongfully that he slugged the arresting officer. Needless to say, this landed him in jail. Unfortunately, this Phd turned out to be an alcoholic too but the Neuropsychological exam proved that his drinking problem started after his calculated date of onset and that he was suffering from loss of inhibitions too which probably caused his problem with alcohol! This incident was thrown out of court. Several months later he was arrested again for the same charges. This time he also got mad and slugged the arresting officer who happened to a woman!  This took a little longer but once again, after she was shown the results of the Neuropsychological exam she dropped all charges against him!

Possession of Cocaine:
This Phd had felony charges against him for possession of cocaine and associated paraphernalia. He had previously served time on a prior conviction before I met him and began helping him. His neuropsychological examination documented a calculated time of onset of HD’s symptoms several years prior to when he began his “uncharacteristic” behavior as a drug addict. His exam also detailed all of the areas in which he was deficient also supported the “softer” symptoms I mentioned above which adversely affected his behavior and caused him to do things that he had never done before prior to his onset of Huntington’s Disease. His current charges were thrown out completely with the stipulation that I get him into an assisted-living home where he wouldn’t be tempted above his limits on the streets again- kind of like a kid running wild in a candy store!

Threatening to “Blow Up His School” and “Kill Everyone!”
A child with Juvenile HD was about to get expelled from grade school for making angry remarks that he was going to “blow up his school” and “kill everyone!” Unfortunately, in our post-9/11 atmosphere his JHD-affected threats were not taken lightly by his teacher and he was promptly expelled from school. Even the closest HD Center of Excellence (COE) wouldn’t send the school a letter that his behavior was probably caused by his JHD as they stated they were unsure. I e-mailed the Principal a letter (like the one below) and asked the Mom to have a Neuropsychological exam done to verify the symptoms mentioned in my letter if necessary. Luckily, the Principal understood and this young child was admitted back to school with just my explanation of what was causing his behavior and a promise that he wasn’t a budding terrorist in disguise.

Rejected by SSD
You look too good to be disabled:
Dozens & dozens of individuals who were rejected by Social Security Disability because they “looked” too well when they went in for the interview have been denied these crucial benefits. They were all definitely suffering from HD’s “softer” symptoms because all couldn’t function at work and had received several verbal and written warnings, some demotions, and some early terminations because of performing their current job below standards! I advised them to take a Neuropsychological exam, reapply for SSD and this time to include the Neuropsychologist who gave them the exam as part of their disability paper trail. Using this additional information every single one of them were awarded SSD coverage, and without paying for an expensive lawyer either!

Inability to Care for Self and Children
Endangering Two Babies:
I felt that a Phd woman was not capable of taking care of her two little babies (one was two years old and the other was just 9 months old), even though she looked “normal.” I notified Child Protective Services (CPS) and recommended that they have her take a Neuropsychological exam to determine how much HD was adversely affecting her ability to care for her two babies, let alone herself. The results were read in the CPS meeting and showed that she was deficit in many areas which were in turn endangering her babies every day. She and her two young daughters were living with her father, who just recently returned from prison after serving 10 years for being a pedophile. She was unable to recognize why living with him wasn’t the best idea! She was found incompetent to care for them and they were given to a relative. In this case, we had to error on the side of her babies and their welfare!

Guardianship:
A sister of this same Phd (mentioned above) was applying for Guardianship over another symptomatic sister. This sister appeared outwardly okay and could converse quit well still. I felt the judge may look at her and decide that she didn’t need a guardian, even though she was doing lots of questionable things. Her derelict father had also gone into SSD with her and told them she didn’t need a Representative Payee for her checks because as they could see, she looked okay enough to receive her checks herself! I took her to get a neuropsychological examination and it proved, beyond a shadow of a doubt, that she was incapable of taking care of herself and making financial or other decisions on her own because of HD! Her well sister was awarded guardianship over her and also become her representative payee for SSD, thus cutting the greedy father out of the picture. This information was also used to make sure that she had supervised visits each time her children came to visit her, so she would not unknowingly endanger them.

Too Much Stress:
This Phd had a meltdown at work because of the frustrations associated with not being able to multi-task, remember things, plan and organize effectively, and thinking everyone was out to get her. She left work in tears after having a conniption fit one day and didn’t feel she could ever return. The Neuropsychological Exam was used to help her qualify for Short and Long-Term Disability by showing that she was indeed unable to perform her job responsibilities at her usual competency!
 
Overview of “Softer” Symptoms Involved
Because Huntington’s Disease, and it’s myriad of “softer” symptoms, are not well known, I have taken the liberty to put together a summary of the ones I believe adversely affected all of them, including references that verify these devastating symptoms. It is my hope that by understanding the subtle complexities of HD far better than we do today will help everyone to know when to “error” on the side of compassion!

After thoroughly reading dozens of Criminal Complaints against those with HD and also the results of their Neuropsychological Exams, there is no doubt in my mind now that their questionable behaviors and actions such as lying, cheating, stealing, deception, poor judgment, loss of consequential thinking, sexual promiscuity, and  embezzling were ALL caused by HD-Affected behavior and symptoms such as:

          Impaired judgment, reasoning and the ability to know when
          when something is wrong
          Lack of self-control (disinhibition or loss of inhibitions)
          Loss of forward or consequential thinking
          Impulsiveness
          Loss of short-term memory

“HD results from genetically programmed degeneration of nerve cells, called neurons, in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance.” (National Institute of Neurological Disorders and Stroke, NIH,  http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm )


A) Emotional Changes

“They cry one moment and laugh the next. Their emotions just don't match what's around them. They're superficial. They're very shallow. You can't relate to them deeply. They're disinhibited, which adds to the social inappropriate problems. They're very facile and very childish. They also have impaired judgment. They can't judge what socially acceptable behavior is. They can't judge what's appropriate to do at that time. They're frequently disorganized. They can't concentrate and finish anything. They keep moving around and changing things. They can't attempt a conversation for more than a short period of time. They want to go on to something else. They can't plan -- they're incapable of planning ahead. They demand primary gratification.” (Understanding Behavioral Changes in Huntington’s Disease, Dr. Edmond Chiu, Senior Lecturer in Psychiatry, University of Melbourne, Australia, New York University Medical Center, September-12,-1989)


B) Impulse Control
Definition
Dysfunction of the caudate nucleus and frontal lobe of the brain may cause difficulty regulating or controlling emotions and impulses. This is called impulsivity or disinhibition. Impaired impulse control may be the reason that some persons affected with HD easily lose their temper, begin to drink too much or have inappropriate sexual relations. Also, disinhibition can sometimes contribute to illegal behaviors such as stealing. Disinhibition usually presents itself as trouble controlling a sudden desire to do or say something that comes to mind, even when the behavior is hurtful, repetitious or socially or sexually inappropriate.

Damage to the caudate nucleus or circuits connecting the caudate and the frontal lobes can also contribute to impulsivity. One of the primary functions of the caudate is to regulate, or control, the information from the rest of the brain. As the caudate is affected by HD, the regulation mechanisms of the brain break down. Without the caudate, the brain cannot regulate how much movement, how much feeling or how much thinking is required in a certain situation.
Example:

“He was arrested for stealing a pair of blue jeans while a salesperson was right behind him.”   (Understanding Behavior in Huntington’s Disease, 2nd Edition, 1999, p. 35, Jane S. Paulsen Ph.D.)


C) How Does the Brain Contribute to Behavior Changes in HD? - Understanding Anatomy and Related Behaviors
Different parts of the brain are responsible for different aspects of our behavior…The frontal lobes make up the largest area of the brain and contain the highest levels of our behaviors. The frontal lobe area of the brain is often considered the “boss,” or chief executive officer (CEO), of the rest of the brain. The following list includes executive functions of the frontal lobes:
          Organizing
          Establishing the priority of various events
          Controlling impulses
          Monitoring self-awareness
          Beginning and ending activities
          Creative thinking
          Problem solving
(Understanding Behavior in Huntington’s Disease, 2nd Edition, 1999, p. 9, Jane S. Paulsen Ph.D.)


D) Cognitive Changes In HD Are Specific and Predictable
“…Degenerative diseases that affect the brain, such as HD, do not damage the entire brain in a non-specific way. Rather they preferentially damage and disrupt the function of certain parts of the brain, while other parts of the brain continue to function well. Since different parts of the brain serve different functions, the type of mental change that a person has will be characteristic for a particular disease and will be governed by the regions of the brain preferentially involved. Thus, mental changes are both specific and predictable…”
“Functions-of-the-different-brain-regions
The front parts are the "captain of the ship". Imagine on a ship there are a variety of instruments, used for navigation and for communication. They are like our instruments or tools of thought. However, even when those instruments are all in working order, the ship does not function on its own. It needs a captain to plan and organise the journey, to attend to the instrument panels and communication systems, abstract out relevant information and ignore what is not relevant, to check incoming information and to have the flexibility to alter a course of action if circumstances change. The captain has a supervisory function, in regulating and controlling what happens. The front parts of the brain are important for those same functions and they are sometimes referred to as the executive or supervisory system of the brain. These regions are necessary for planning, forward-thinking, goal-directed behaviour, for the ability to organise behaviour, attend to what's relevant and ignore what is not relevant, to monitor and check performance, and to adapt behaviour to altered circumstances and different social situations. It is in these areas of cognition that people with HD have particular difficulties…” (Understanding Challenging Behaviour In HD, August 2001, pp. 1-2, Julie Snowden, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Hope Hospital Salford, UK)


E) Thinking Ahead
The ability to think forward means that we sacrifice short-term rewards for longer-term goals...That is, we are able to see the future consequences of a course of action and we modify our behaviour accordingly. If the capacity for thinking forward is lost, as in HD, then the person does not see future consequences, and behaviour is governed much more by immediate needs and desires rather than longer-term goals. The person with HD may seem to want "immediate gratification". The person is not being deliberately demanding. It is just that they are no longer able to think long-term…It is that decisions are based much more on immediate than long-term considerations…” (Understanding Challenging Behaviour In HD, August 2001, p. 3, Julie Snowden, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Hope Hospital Salford, UK)


F) The Frequency of Behavioral Problems In HD
“…We found that some behavioral changes are reported very commonly: in up to 80% of people who attend our regional HD clinic. These symptoms include loss of drive and initiative, reduced efficiency of task performance, impaired judgment, mental inflexibility and self-centredness. Since many of the people who attend the clinic are still in the early stages of HD, the high frequency of those symptoms suggests that they are likely to be fundamental to the disease process…”(Understanding Challenging Behaviour In HD, August 2001, p. 6, Julie Snowden, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Hope Hospital Salford, UK)


G) Disinhibition

“Some HD sufferers may act in a disinhibited way which is embarrassing to others. Disinhibited behaviour may take a variety of forms. Patients may act impulsively or rashly without thought, such as making a sudden purchase of a car which they cannot afford. They may make socially in appropriate remarks, for example making personal comments about a person who is within earshot. They may behave in a sexually disinhibited way, such as making sexual advances to a partner in front of the children. Such behaviour results from a breakdown in patients’ social awareness and ability to think through and appreciate the social consequences of actions…” “They do not see the repercussions of their own actions. HD patients may also no longer experience so acutely the feelings of embarrassment, guilt and shame which under normal circumstances place constraints on social behaviour. Patients cannot simply be "made to see" the consequences of their actions if the capacity to do so has been damaged by the disease process; nor can patients be made to feel guilt, shame or embarrassment if those emotions have been taken from them by the disease…” (Understanding Challenging Behaviour In HD, p. 8, August 2001, Julie Snowden, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Hope Hospital Salford, UK)


H) Effects Of Disease Versus Reaction To Disease

“…Often, behaviour is not just the direct effect of HD or just a reaction to it. It is a combination of the two. This is well illustrated by an incident involving a man with HD.  As he was walking along he was stopped by the police and accused of being drunk. As he was entirely sober the man felt insulted and outraged, hit the policeman who then arrested him on a charge of assault. Many people with HD have parallel experiences in which their symptoms are misinterpreted by others. The man's feelings of anger can be seen as an understandable reaction, and it is a feeling that most people would share. Nevertheless, in a comparable situation most people who do not have HD would immediately be aware of the potential repercussions of hitting a policeman and realise that it would not be in their best interests to do so. They would, moreover, have the capability of suppressing their feelings of outrage. The man with HD could neither foresee the consequences of his actions, nor could he keep his feelings of anger in check. Those features are the direct effects of HD.” (Understanding Challenging Behaviour In HD, August 2001, p. 10, Julie Snowden, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Hope Hospital Salford, UK)


I) Decision Making

“HD may affect the individual's judgment, memory, and other cognitive functions. Early signs might include having trouble driving, learning new things, remembering a fact, answering a question, or making a decision... As the disease progresses, concentration on intellectual tasks becomes increasingly difficult.” (National Institute of Neurological Disorders-and-Stroke,-NIH, http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm )


J) Neurobehavioral Changes

“Emotional or behavioral disturbances tend to develop gradually over time and may become apparent before or concurrent with the motor manifestations of HD.

In some patients, initial symptoms of HD may include personality changes, such as:
          Increased irritability
          A tendency to find fault with others
          Constant complaining
          Suspiciousness
          Impulsiveness
          Lack of self control"
(http://www.mdvu.org/library/disease/hd/hd_sym_neuch.html )


K) Cognitive Decline

“HD is often characterized by progressive dementia or gradual impairment of the mental processes involved in comprehension, reasoning, judgment, and memory.

Early signs of cognitive decline may include:
          Forgetfulness
          Difficulty maintaining focus and attention
          As HD progresses, additional symptoms may develop including:
          Increased difficulty concentrating
          Inability to absorb and understand new information
          Impaired ability to engage in problem solving
          Diminished memory retrieval
          Progressively impaired judgment and impulse control
          Diminished language skills with disorganized speech
          Increasingly impaired ability to plan, initiate, or perform certain
          purposeful movements (apraxia)”
(http://www.mdvu.org/library/disease/hd/hd_sym_cog.html )

 
L) Impulsivity
“Some persons with HD experience difficulties with impulse control and may develop problem behaviors such as irritability, temper outbursts, sexual promiscuity and acting without thinking. Some degree of impulsivity and dysregulation of behaviors is quite common in HD.” (A Physician’s Guide to the Management of Huntington’s Disease, 2nd Edition, 1999, p. 29, Adam Rosenblatt, M.D.; Neal G. Ranen, M.D.; Martha A. Nance, M.D.; Jane S. Paulsen, Ph.D.)

M) The Psychiatric Disorder, Introduction
“Patients with Huntington’s disease who have psychiatric disorders generally suffer from underdiagnosis and undertreatment…Many, if not most people with HD also experience less well defined, non-specific changes in personality and mood, such as irritability, apathy, or disinhibition.” (A Physician’s Guide to the Management of Huntington’s Disease, 2nd Edition, 1999, p. 37, Adam Rosenblatt, M.D.; Neal G. Ranen, M.D.; Martha A. Nance, M.D.; Jane S. Paulsen, Ph.D.)

N) Mild Psychotic and Behavioral Symptoms Precede Frank Chorea By Up To 10 Years
“The classic signs of Huntington disease are progressive chorea, rigidity, and dementia, frequently associated with seizures. A characteristic atrophy of the caudate nucleus is seen radiographically. Typically, there is a prodromal phase of mild psychotic and behavioral symptoms which precedes frank chorea by up to 10 years…” (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=143100 )

O) Evidence Of Presymptomatic Cognitive Decline In Huntington's Disease
“Asymptomatic persons at risk for Huntington's disease (HD) (N = 28) were assessed with neuropsychological, psychiatric, and neurologic tests while undergoing genetic linkage studies to determine their probability of carrying the HD gene…The results suggest the presence of cognitive decline prior to identifiable motor impairments in HD.” (J Clin Exp Neuropsychol. 1992 Nov; 14 (6): 961-75. Diamond R, White RF, Myers RH, Mastromauro C, Koroshetz WJ, Butters N, Rothstein DM, Moss MB, Vasterling J. Department of Neurology, Boston University School of Medicine, MA 02118-2394. PMID: 1452640 )

P) Behavioral Changes In Huntington Disease
Objectives:
This study aimed to gain a better understanding of behavioral abnormalities in Huntington disease (HD) and to develop a method for reliably assessing these changes.

Background:
Behavioral changes are a central feature of HD and often cause considerable distress and difficulty to patients and their relatives. However, they have received little attention from research despite their prevalence and clinical significance.

Methods:
One hundred thirty-four patients with HD were assessed using the Problem Behaviors Assessment for Huntington Disease (PBA-HD), an instrument for rating the presence, severity and frequency of behavioral abnormalities in HD.

Results:
The findings confirm that behavioral problems are common among patients with HD. The most common symptoms were loss of energy and initiative, poor perseverance and quality of work, impaired judgment, poor self-care and emotional blunting.” (Neuropsychiatry Neuropsychol Behav Neurol. 2001 Oct-Dec; 14 (4):219-26. Behavioral changes in Huntington Disease, Craufurd D, Thompson JC, Snowden JS. Academic Unit of Medical Genetics and Regional Genetic Service, St Mary's Hospital, Manchester, UK. PMID: 11725215 )
 
Q) Cognitive Changes In People Living With Huntington Disease
“The changes in thinking (known as ‘cognition’) in HD are characterised by a pattern of both preserved and impaired­ abilities. Considerable differences­ occur among affected individuals­ in terms of the presence, severity and progression of cognitive­ impairment.  For example, some may remain relatively untouched by memory­ disturbance throughout the course of their illness­ (certainly until the later stages); whereas others may experience memory loss very early.

Preserved Abilities
It is worth emphasising that many cognitive­ functions remain relatively unaffected in HD. For example, an affected person’s long-term memory can remain relatively intact and they can therefore recount experiences from the past or still have a good general knowledge.

Cognitive Decline in HD
Unfortunately, HD is characterised­ by specific cognitive­ deficits. Memory appears especially affected, with problems occurring for both verbal and non-verbal memory. For example, a person with HD may have trouble remembering­ what you have said to them, or story lines in TV programmes or movies. Similarly, they may have extreme difficulty interpreting­ maps or remembering places. They often­ have trouble finding their way around and frequently get lost in familiar­ places. Thus, they may have difficulties when transferred to a new environment, such as a nursing home and need time to adapt to their new surroundings.

Because the disease affects the frontal lobes of the brain, planning ability­, judgment and decision-making are affected. As a result, people with HD often have trouble monitoring their own behaviour and do not realise that they are making errors or mistakes. Another­ characteristic is that people with HD have trouble motivating themselves­ and others often mistake this as stubbornness or laziness. Planning and problem solving become increasingly difficult and they may, for example, now have more trouble cooking­ a new recipe or fixing a fuse.”


(Behavioural and Cognitive Changes in HD I: Fact Sheet Series Australian HD Association, Carmela Connor, Manager Neurosciences Unit, John Rudge, Clinical Psychologist,-Neurosciences-Unit,
http://www.ahda.asn.au/content/factsheet/download/fs9.doc )
R)-Fast-Facts-About-HD,-What-Is-Huntington’s-Disease?

Symptoms:
Depression, personality changes, and mood changes
Forgetfulness, and impaired judgment
Unsteady gait and involuntary movements
Slurred speech and intoxicated demeanor
Difficulty Swallowing

(http://www.hdsa.org/images/content/1/1/11268.pdf )

CONCLUSION:
I hope that what I have said has given you additional insight into the increased uses of how to use the Neuropsychological exam to help those suffering from Huntington’s Disease. Remember, our friends and loved ones who are suffering from Huntington’s Disease are NOT hardened criminals who are consciously able to plan and perpetrate criminal offenses against others, but are simply human beings suffering from the horrible symptoms of Huntington’s Disease, which has caused them to be unable to see, or sometimes even care, that what they are doing is wrong, therefore rendering them unaccountable for their actions. May God bless everyone with Huntington’s Disease and those who know them, with more compassion and understanding!
 

If you have additional questions or would like the entire texts of what I have quoted above, or would like to use me as an expert witnesses, please feel free to call me at 602-309-3118 or e-mail me at phardt1@cox.net  …….Phillip Hardt
 



Excellent Article on
Neuropsychological Exam Testing

What Is Assessed
Neuropsychological testing is used to assess a number of cognitive domains in diagnosing, screening, and following patients with mild cognitive impairment, Alzheimer's disease, frontotemporal lobe dementia, and Lewy-body dementia, among other brain disorders. The first of these cognitive domains is premorbid intellectual functioning, prior the onset of any brain dysfunction, which is evaluated from several pieces of information: level of education, level of occupational functioning, and certain cognitive skills that can be relatively resistant to aging effects, such as vocabulary or basic reading of words. We also look at current intellectual functioning: how the individual processes, classifies, and integrates information. The level of functioning on those skills gives us an idea of where that patient or individual should be, given no symptomatology.


Cognitive Functions Assessed by Neuropsychological Evaluation

     • premorbid intellectual functioning
     • intellectual functioning
     • attention/concentration
     • memory functioning
     • language functioning
     • executive functioning
     • visual perception
     • motor functioning
     • personality functioning

Neuropsychological testing is used to assess a number of domains in diagnosing, screening, and following patients with dementia.

There are a number of tests used in making this assessment. You may be familiar with the Wechsler tests; there have been several revisions of those, one of which is an abbreviated version that gives you an estimated level of IQ rather than the full IQ assessment that we would obtain from the full tests. An average IQ is 90 to 109. We also look at attention, predominantly sustained attention, by looking at the ability to subtract serial sevens or to recite a list of numbers in reverse order.


IQ Ranges

               130 and above          very superior
      120–129                             superior
      110–119                             high average
        90–109                             average
        80–  89                             low average
        70–  79                             borderline
        69 and below                   defective

Ranges of IQ scores as used in neuropsychological testing.

To evaluate memory, we look at several specific aspects: verbal memory and visual memory, then immediate verbal memory and delayed verbal memory, and immediate visual memory and delayed visual memory. For these we also use the Wechsler scales.

We look at verbal memory, using contextual cues, such as prose passages, and at rote verbal learning using a word-list learning task. For visual memory, we most commonly look at memory of geometric designs, both immediate and delayed, and occasionally at memory of faces and spatial memory. The Wechsler scales are the most frequently used, and sometimes we use the Rey Complex Figure Test.


Memory Functioning

     • Verbal memory
          o Wechsler Memory Scale-Revised
          o Wechsler Memory Scale-III
                Logical Memory I and II (prose)
          o Word-List Learning
                Selective Reminding Test
                California Verbal Learning Test
     • Visual memory
          o Wechsler Memory Scale-Revised
          o Wechsler Memory Scale-III
                Visual Reproduction I and II:
                   memory for geometric designs
          o Rey Complex Figure (copy and delay)

Neuropsychological tests look at verbal memory in context, using cues such as prose passages, and at rote verbal-learning tasks, using a word-list learning task. Visual memory is assessed in neuropsychological testing by memory of geometric designs, memory of faces, and spatial memory.

We look at language functioning for both receptive and expressive language by assessing auditory comprehension, repetition of words, phrases and sentences, and confrontation naming.

To assess visual-spatial skills, or how the individual processes visual stimuli, we look at spatial orientation and visual construction. You may be familiar with the test in which a patient draws a clock to command and to copy. To evaluate executive functioning, we look at how or whether someone is able to initiate, monitor, and complete a task. For motor functioning we look at speed, dexterity, and strength. To assess personality with respect to dementia, we screen for mood disorders, psychosis, and sometimes personality disorders.
 
Penne Sims, Ph.D., “Neuropsychology of Mild Cognitive Impairment, Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia,”  http://ci.columbia.edu/c1182/web/sect_6/c1182_s6_1.html


Neuropsychology in Differential Diagnosis

Neuropsychology is helpful in differentiating between normal aging (which we call age-associated memory impairment or AAMI), mild cognitive impairment (MCI), and dementia. For AAMI, the memory deficits that we find in formal testing are usually not greater than one standard deviation below the mean. We compare these individuals to a young normative sample, not necessarily to an age-matched sample. Patients with AAMI usually have no dementia or other cognitive deficits in any domains.

In contrast, patients with MCI on formal testing have memory deficits at least one and a half standard deviations below the mean, and the range depends on the stringency of criteria you choose. You may not consider less than two standard deviations a significant deficit. For this particular definition, unlike for AAMI, patients were compared to an age-matched sample. This is an important distinction, and MCI patients will show a greater degree of impairment.

There may be other cognitive deficits with MCI, but these are usually not significant, falling less than one standard deviation below the mean. We usually look for a verbal-memory impairment, since this is one of the primary memory deficits in someone who will progress to AD. Immediate recall is not a good predictor of progression to AD, so we usually look at delayed recall.
The common definition of dementia, with which you are probably familiar, is that memory loss is substantial, one and a half to two standard deviations below the mean, with impairment in at least one other cognitive domain.

Penne Sims, Ph.D., “Neuropsychology of Mild Cognitive Impairment, Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia,”  http://ci.columbia.edu/c1182/web/sect_6/c1182_s6_2.html


Screening Measures
In 2001 the American Academy of Neurology published practice guidelines for early detection of dementia, and these include recommendations of screening measures for cognitive functioning. In addition to neuropsychological testing, they recommended a number of commonly used tests.


Mini Mental State Exam:

Orientation
          (5) What is the year, season, date, and month?
          (5) Where are we: state, country, town or city, hospital, floor?
Registration
          (3) Repeat the following: apple, table, and penny.
Attention and Calculation
          (5) Serial 7s: 93, 86, 79, 72, 65 or spell world backwards.
Recall
          (3) What were the three objects: apple, table, and penny?
Language
          (2) Name a pencil and watch.
          (1) Repeat the following: No ifs, ands, or buts
          (3) Three-step command: Take this piece of paper in your right hand, fold
                 it in half, and put it on the floor.
          (1) Read and obey: Close your eyes.
          (1) Write a sentence.
          (1) Copy the design:
          (30) [Total Points Possible]
 
The Folstein Mini Mental State Exam is a thirty-item test used to evaluate patients for cognitive problems and dementia.


The Folstein Mini Mental exam is one of the most common measures and is used when evaluating a patient who has functional complaints. The Folstein is a thirty-item test that takes about five minutes to give. We generally look for scores below 25 out of 30 to be considered impaired, but this can be misleading for many patients depending on age and level of education. For example, a patient who is 70 years old with an eighth-grade education who scores a 24 out of 30 may be less of a concern than someone age 70 with an M.D. or Ph.D. who also scores 24. It is important to utilize the age and education-corrected norms when using the MMSE. On average, patients with dementia decline three points per year on the MMSE.

Many studies have found that people with cognitive impairment perform on neuropsychological tests much like people who are normal or have no complaints at all. Neuropsychological testing using the MMSE is not necessarily as useful in discriminating normal patients from those you think may have MCI.

There are limitations with Folstein MMSE. It is heavily weighted toward verbal items, and those tend to be very simple. Also, if you have monitored a patient fewer than three years, any changes you see in the score may be somewhat misleading because there tends to be a large measurement error. There is a substantial variation in change over a short period of time depending on how the patient is feeling—maybe the weather was bad, maybe the examiner was tired, maybe the patient was tired. Without extended monitoring, you will not know whether a three- or four-point change is as significant as it seems.

Other cognitive domains like distractibility, abstraction or executive functioning, and visual-spatial skills may be limited or completely omitted from the Folstein. There have been some modifications to the test, and one of those is a more comprehensive 57-item measure that we have used at Columbia. One of the more common overall measures of cognitive functioning in use is the Mattis Dementia Rating Scale. It is much more comprehensive and is better than the Folstein at picking up some of the cognitive domains omitted from the MMSE. The Brief Focus Cognitive Assessment includes tests such as clock-drawing, telling time, or making change, but these are limited in their utility because they only assess a very specific cognitive skill.

The structured informant interview is extremely important in terms of providing information on the history and nature of the difficulties and change or decline over time. Neuropsychological testing is a much more comprehensive measurement of cognitive functioning than the tests discussed above. As such, it is important to identify what constitutes a deficit and what signifies a decline in neuropsychological performance. As before, the standard cutoff is 1.5 standard deviations below the mean, and some people require 2 standard deviations to consider a deficit significant. Here the deficit is based on a comparison of the patient's performance with normative data that tend to be stratified by age, education, and sometimes by sex.

As with any test, there are going to be limitations. Sometimes the normative sample is not appropriate for very low- or very high-functioning individuals. In these cases, we prefer to make a within-subject comparison, by which I mean we have to compare the individual to himself or herself. One way to do this is to estimate the individual's premorbid level of functioning by assessing his or her level of education, level of occupational functioning, and/or performance on tasks like vocabulary or reading that can be relatively resistant to aging effects. Alternatively, we can look at two or more consecutive evaluations of the individual to identify more specific changes in cognitive functioning over time.

Penne Sims, Ph.D., “Neuropsychology of Mild Cognitive Impairment, Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia,” http://ci.columbia.edu/c1182/web/sect_6/c1182_s6_3.html


Benefits of Testing

The role of neuropsychology in dementia is not only helpful in verifying initial diagnoses, but it also helps to measure the rate of progression, provide indications on how deficits are changing over successive evaluations, and measure the efficacy of intervention therapies over repeated evaluations. It is helpful in research, providing sensitive tools for early detection and discrimination of deficits, and it helps provide powerful tools for clinical trials.
With respect to patient care, it can also help identify cognitive strengths and weaknesses and how deficits impact the activities of daily living. This can help us suggest to the family and the patient what he or she is still good at, how to remain productive, and what he or she should and should not try to do. For example, should the patient try to do finances, does he or she need a little help, or should that task be taken over by someone else? Neuropsychological testing also helps estimate the need for and extent of future care.

Neuropsychological testing, however, should not be used as a lone diagnostic tool; it is always most useful in conjunction with a multidisciplinary approach, including psychiatry, psychology, radiology, social work, or others that I have not mentioned.
 
Penne Sims, Ph.D., “Neuropsychology of Mild Cognitive Impairment, Alzheimer's Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia,” http://ci.columbia.edu/c1182/web/sect_6/c1182_s6_7.html



 

 
 
         
   

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