Sub Cortical Front Dementia in Huntington's Disease

There is a very pressing need to frankly discuss one of the most debilitating symptoms in the neuropsychiatric sequelae of “softer” symptoms in Huntington’s Disease- sub-cortical “frontal” dementia. Unfortunately, since there are far more patients with Alzheimer’s Disease (AD) and the cortical dementia associated with it, virtually all medical professionals recognize it more easily and disappointingly assume that person’s with other neurological diseases, especially Huntington’s Disease (HD), should look, act and respond the same way to questioning as those with AD do.

Second, in some states, the only way you can receive state subsidized assisted-living or nursing home benefits is to be diagnosed with “dementia” and when someone with HD applies they appear to be okay since they still know their name, what date it is, where they are, who the President of the United States is and other similar questions. Virtually everyone who I have had the privilege of assisting during the Arizona Long-Term Care (ALTCS) interview process were physically capable of performing their Activities of Daily Living (ADLs), such as toileting themselves, brushing their hair and teeth, dressing themselves, cooking, taking their own medications and bathing. However, because of their sub-cortical “frontal” dementia they would never remember to do these things on a regular basis, think of doing them and then forget when distracted, or never think that they needed to be done in the first place, that is, unless they were verbally prompted and/or encouraged by physically offering to assist them. Unfortunately, most states are concerned about the person’s “physical” ability to do the ADLs so the person with HD normally fails their qualification questions miserably. In addition, it does not help the interview process at all when the person with HD is able to tell the interviewer (unlike with Alzheimer’s) that they are able to perform their ADLs every day without any problems. It is not until I gently tell the interviewer that the person with HD is also suffering from “dementia” the state interviewer is able to decrease the high scores they already received on their ability to physically perform their ADLs and their true need for higher care finally realized and approved!

Lastly, since none of the official published books and pamphlets on HD even mention “dementia” as one of HD’s devastating symptoms it is very hard to educate the medical professionals (neurologists, psychiatrists and psychologists) that we come in contact with in trying to care for the Person with HD (Phd) so unfortunately, their quality of life and care received isn’t as high as they deserve. Having medical professionals understand the basic differences between HD and AD is essential for them to understand that even though the person with HD may answer questions in the affirmative, thus indicating that “all is well,” their answers should not be taken for the “gospel” truth. I have many examples of this gleaned from having the privilege of helping dozens and dozens with HD apply and receive state benefits in the State of Arizona.

In an effort to help families and social workers who are dealing with Phds on a daily basis, I have concatenated the most current references on HD’s sub-cortical “frontal” dementia from the internet and other places. These are attached at the end of my personal examples in the next section.

Sometimes, when the whole extent of dementia-damage isn’t easily recognized by the family or medical professionals, because of not knowing the sequelae of HD’s “softer” symptoms (emotional, behavioral and cognitive), the Phd doesn’t receive the care they deserve. The myriad of benefits of utilizing the Neuropsychological exam early in HD are discussed more thoroughly in my previous article: “Using the Neuropsychological Exam and Other Tools To Help Those Struggling With the “Softer” Symptoms of Huntington’s Disease!” which can found on the Huntington’s Disease Advocacy Center website at: (Using the Neuropsychological Exam and Other Tools To Help Those Struggling With the “Softer” Symptoms of Huntington’s Disease )

I wholeheartedly agree with the American Family Physician’s article “Early Diagnosis of Dementia,” found on their web site. They recommend: “Patients with early dementia may benefit from formal neuropsychologic testing to aid in medical and social decision-making.” (Early Diagnosis of Dementia)

Personal Examples Demonstrate Need for Better Understanding of HD’s Sub-Cortical “Frontal” Dementia

Tiff

In Tiff’s case, in addition to virtually to every other interview at which I’ve accompanied the Phd, the person giving the competency test or just asking general “welfare” questions were all given “misleading” positive-sounding answers by the Phd because of their “dementia.” For example, when asked by the interviewer about bathing (one of the ADLs) Tiff responded in the affirmative, that she did so every day. In a “redirect” with the interviewer I pointed out that this couldn’t be true and asked him to look at her dirty legs, face, hair and arms to verify my point. When he asked her if she could brush her teeth she also responded affirmatively. In another “redirect” I asked him to ask her to smile and show him her teeth. They were so poorly taken care of that she had could only eat soft foods- and was only 28 years old! Shortly afterwards I had to take her to have all of her teeth pulled and fitted for dentures that a friend had donated for her. Once again, she was physically able to perform these Activities of Daily Living (ADLs) on the application for long-term care but because of her “dementia” she was mentally and cognitively unable to remember to do them on a daily basis or even care if she ever did them at all. She later qualified for ALTCs benefits once her “dementia” was appropriately factored in.

Michael

In another case, an interviewer, who allegedly had an excellent knowledge of HD, thought that Michael, who had HD, was competent to stand trial because he was able to answer all of her questions about his knowledge of right and wrong and how the court system worked- after five or six verbal prompts or helps by her. After my “redirect” I pointed out that Phds are indeed successful in answering questions after lots of prompts, unlike those with Alzheimer’s Disease, but the blank stare and silence she got after most of her questions were indeed his real “answer” that indicated his real inability to mentally process and find the answer to each of her questions. She could not be swayed and it wasn’t until after Michael had a Neuropsychological exam done that not only was he found “incompetent” to stand trial, but it also showed an estimate of when he began his HD symptoms, which just happened to be several years prior to when his “uncharacteristic” use of illegal drugs began, including his most recent arrest for possession of cocaine.

The Neuropsychological Exam showed that because of his “dementia” he was indeed incompetent to stand trial. The judge threw all of his charges out. The judge also mandated that I get Michael off the street and into a safe assisted-living home, since she concluded very wisely, that if allowed to return to the streets without inhibitions and forward thinking, not to mention his “dementia,” he would end up right back in her courtroom again! That would have been like putting him back on the streets with three (3) strikes already hanging over his head. Educating his defense attorney and the judge of HD’s “softer” symptoms, including “dementia” had helped Michael get into a safe and secure place. He could no longer roam the streets like a kid in a candy store being exploited by drug pushers and the likes.

Don

Don became a great teacher to me, regarding a lot of HD’s “softer” symptoms, including “dementia.” I was made aware of his piteous plight by his former wife who I was already helping because their 7 year old daughter had Juvenile HD. She was concerned about his welfare and asked me to please help him. When trying to get Don onto our state welfare program and food stamps I found out he needed an original birth certificate to prove his U.S. citizenship. The only problem was, it had to be delivered to the person’s official address on file. I called and ordered him a certified birth certificate and had it delivered Next Day Air to expedite his application process since he was completely out of food, only to have Federal Express call and tell me the delivery was unsuccessful because the occupant refused to answer the door and sign for its delivery.

When I ask him why, he bluntly told me that he never opened the door for anyone before 10:30 am. Now I knew. I called Fed Ex and asked them to attempt delivery one more time, this time I threatened to never take him to McDonald's again if he didn’t answer the door. Since he didn’t want to miss our weekly trips there, he grudgingly conceded. I spent about 30 minutes rehearsing with him why he needed to open the door and then prayed that he would remember.

In continued attempts to help Don achieve a higher standard of living than he presently had, I talked with his Neurologist about his failing condition, “dementia” and slovenly living conditions and asked for his support in helping get Don into a safe and secure assisted living home. However, his neurologist wouldn’t sign the paperwork saying he needed assisted living because during his last appointment (which I accompanied him to) he was able to answer basic questions from the doctor, like, “How are you today?” and “Isn’t it hot outside?” Vowing not to be undermined I then made an anonymous call to adult protective services to have them go check out his abhorrent living situation. Someone went out, asked him if he needed help and then you guessed it, he said “No,” that he was doing fine and didn’t need any help! Surely the investigator could have smelled him, he honestly hadn’t bathed in about two years! Later, because HD’s “dementia” and “softer” symptoms left him easy to exploit, he was arrested several times because of allowing homeless drug addicts to live with him while they look for work. His apartment manager called me and told me we had to do something or she was going to have to evict him because of complaints from his neighbors about his unsavory roommates. In the past he adamantly refused to even fleetingly consider moving.

The apartment manager and I met several more times before deciding that she would send him a 30-day notice to move out and during that time I would get him into an assisted-living facility. I made an appointment for him with ALTCs. During this interview for benefits the examiner asked the normal questions about ability to perform ADLs. When he asked him whether-or-not he could cook he stated that he had no problems cooking and did so every day. Upon my “redirect” I showed the interviewer his filthy fridge containing about ten packages of expired “green” bologna stacked on top of each other and a few slices of bread on the counter and asked the interviewer if he still thought that Don was capable of cooking on his own. Green bologna sandwiches and Fritos was not my idea of a nutritious, balanced meal. After hearing that he also bathed himself daily (the smell proved otherwise) I showed the interviewer his bathroom with its filthy dirty shower and toilet that had strange “aliens” growing out of it. He was finally approved but the whole process was impeded because of general lack of knowledge that there are other dementias out there besides that seen in persons with Alzheimer’s! Don was physically capable of performing his Activities of Daily Living (ADLs) but mentally, he could never remember to do so, or sadly, even able to see a need to do them anymore. He was granted ALTCs.

Craig

One evening I got a call from Deputy Flannigan from the Maricopa County Sheriff’s Department. They had received a call from Craig’s Mom who said he had a rage attack, became uncontrollable, and pushed her. She was afraid that he would harm her the next time she disagreed with him. Craig is currently living alone and when I called to check on him he said that everything’s okay and that he doesn’t need any help. He is also not taking any medications for aggression, or anything else, because he doesn’t need to. Sound familiar?

The sheriff’s deputy expressed lots of concern because Craig had lots of weapons in his apartment and “what if he answers the door with a weapon if they’re ever called again.” Well, let’s not say “what if,” let’s say “when they’re called again” because I know it’s only a matter of time before this scenario happens again. Both Craig’s Mom and sister know that he is incapable of caring for himself and think he should be in an assisted-living facility. I also know that Craig is incapable of caring for himself and that, despite what he thinks, he should be somewhere safe. His sister wants to apply for guardianship over him but she will never get it without neuropsychological testing which will prove, beyond a shadow of a doubt, that he is indeed incapable of caring for himself and that our concerns for him are very well founded. Only afterwards will she be able to easily be awarded guardianship over him.

I know that if he goes to court now, he will undoubtedly tell the judge he is perfectly fine and doesn’t need any help and the judge will assume nothing is the matter with him because he can still talk and answer a few questions, again unlike those with Alzheimer’s, so therefore he must still be okay. The judge will err on the side of Craig’s independence and autonomy.

There are several problems with this: he will never consent to go for neuropsychological testing (because he honestly feels he doesn’t need it because of the HD symptom called “anosognosia” which has rendered him unable to comprehend the severity of his symptoms and how they are adversely affecting him and two, we cannot force him to go either. He’s a powder keg waiting to explode the next time he has an altercation with his Mom or someone else and the deputies have to be called to his home to arrest him. I did tell his Mom to not hesitate to call 911 if she ever feels threatened by him again in the future but to tell 911 that he’s suffering from “dementia” and HD which is a “mental” disease and that they need to bring “non-lethal force” or taser guns, when they come to question and/or arrest him the next time. However, we all know that if he has to be confronted in his home, he may come to the door brandishing one of his own guns and the deputies will then be forced to not use “non-lethal” force if they feel threatened by him or his actions.

In defiance of his Mom and Sister, Craig recently moved to Prescott to get away from them all. I advised them to call the local police and sheriff departments to advise them of his condition. The waiting game begins as the next time he has a rage attack and is potentially jailed or hospitalized, then his sister can request temporary guardianship from a judge and we’ll finally have him where we can then begin helping him more! Unfortunately, in the meantime, he is still living by himself and shouldn’t be!

David

Another example that comes to mind is David. His Mom lives in Silver City, New Mexico and has called me several times for help with him as he lives in the Duncan/Virden area of Arizona. He was arrested and put into a psychiatric unit twice while living in New Mexico but when I talked with the psychiatrists there and told them that he had “dementia” and would they please recommend that he be put into a safe assisted-living home they laughed at me because they said he was able to respond, knew his name, where he was, felt sorry for what he had done, etc. so therefore could not possibly have “dementia” and be unable to care for himself. I asked them to please have a neuropsychological test done on him, while he was committed for observation, and they stated that New Mexico would not pay for that test, because it was unnecessary in their professional opinions. Where are the educational books and pamphlets stating the opposite? I told them that because of his “dementia” that he would never remember to continue taking the medications they prescribed for him, once on his own again. They assured me that he told them he would continue taking them and that he understood the need to take them. In my opinion, David would probably tell them anything to get released. He is also suffering from anosognosia too and isn’t aware of all of his problems. They didn’t have the benefit of hearing about the problems he was having from his Mom because he wouldn’t put her on his visitation or communication list so she was never allowed to tell the doctors how he had been acting recently, like intentionally wrecking and totaling his new truck and ATV and threatening his brother’s pregnant wife if they tried to interfere with him!

Sadly, he is back on the streets now, vulnerable and incapable of caring for himself, just waiting to be arrested again- however, this time I will have this paper and all of its references to provide to those who will care for him and he will end up where he can be safe and kept from harm’s way, not in a prison cell.

Sherri

Another sad example that comes to mind is Sherri. Unfortunately, as I was boarding the bus to head to Phoenix International Airport to fly to Costa Rica for my honeymoon, I got a frantic call from her Grandmother stating that she was being arraigned at that very moment for drug charges. I frantically made a call to her defense attorney before Dina and I boarded our plane and explained to him about all of HD’s “softer” symptoms and “dementia” and told him she should not be sent to prison for possession of cocaine, but should be given a Neuropsychological exam to see if she was competent enough to stand trial. He laughed loudly and told me that he knew that she was capable of standing trail and did not have dementia and had never heard of HD, let alone all of the “softer” symptoms I had just rambled off to him. Well, because of his pompous attitude he didn’t bring up HD or dementia with the judge and she was sentenced to several years in prison.

Upon returning from my honeymoon I found that we had 90 days to appeal the judge’s decision. At this point I immediately contacted this lawyer again and asked him to file an appeal for her. He got real pompous again and told me that, because of the number of criminal defendants which he represented daily, that he was qualified to tell which ones were competent and which ones were not. Imagine that! Since he had made this medical diagnosis on Sheri I asked if he had performed a Neuropsychological Exam on her, or if he also had degrees in neurology or psychiatry since he was so confident with his diagnosis. I mentioned that it usually took one of those to determine how severely a person was suffering from the neuropsychiatric sequelae of “softer” symptoms associated with Huntington’s Disease! He assured me that he didn’t need any additional degrees to know that she was competent and how dare I challenge this “gift” which he had. Sadly, he refused to take the paperwork to Sherri to sign because he believed it was waste of his time and I couldn’t get approved in time to take it in to her myself because of bureaucratic red tape. Unfortunately, she served out her two years behind bars where she was undoubtedly exploited sexually and in other ways because of her HD and its associated “dementia!”

If you do not know of any examples locally like the ones I’ve mentioned then I recommend you get to know your families better. I shudder to think how many additional individuals with HD and their families have had to go through “hell” because of lack of education on HD’s “dementia” and “softer” symptoms. Please help me spread the word to give those struggling with HD the quality of life they deserve!

“Dementia” References:

The first section (I) is from the Medline website of the National Institutes of Health (NIH) and clearly details some of the “softer” symptoms, including “dementia” that those with HD suffer from.

Section two (II) is taken from the “We Move Movement Disorder Virtual University” website and also details HD’s “softer” symptoms, including “dementia!”

Section III is taken from the American Psychiatric Association’s DSM-IV 294.1 Dementia Due to Other General Medical Conditions- Huntington’s Disease. It details which symptoms you have to be experiencing to be diagnosed as having dementia and even has a specific section noted for “Dementia Due to Huntington's Disease – DSM-IV 294.1.”

Section IV details general symptoms of dementia, taken again from the Medline website of the National Institutes of Health (NIH). I find it very interesting that their list of symptoms of dementia is almost all-inclusive of HD’s “softer” symptoms!

Sections Five through Thirteen are representative references of governmental sites to national organizations, each noting “dementia” as one of HD’s most debilitating symptoms!

I. Medline Plus Medical Encyclopedia

A Service of the U.S. National Library of Medicine and the National Institutes of Health

Huntington’s Disease

Symptoms:

  • Abnormal and unusual movements
    • Head turning to shift eye position
    • Facial movements, including grimaces
    • Slow, uncontrolled movements
    • Quick, sudden, jerking movements of arms, legs, face, and other body parts
    • Unsteady gait
  • Behavior changes
    • Antisocial behaviors
    • Hallucinations
    • Irritability
    • Moodiness
    • Restlessness or fidgeting
    • Paranoia
    • Psychosis
  • Dementia that slowly gets worse, including
    • Loss of memory
    • Loss of judgment
    • Speech changes
    • Personality changes
    • Disorientation or confusion
  • Additional symptoms that may be associated with this disease:
    • Anxiety, stress, and tension
    • Difficulty swallowing
    • Speech impairment

Found on http://www.nlm.nih.gov/medlineplus/ency/article/000770.htm

II. Movement Disorder Virtual University

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:

  1. Increased difficulty concentrating
  2. Inability to absorb and understand new information
  3. Impaired ability to engage in problem solving
  4. Diminished memory retrieval
  5. Progressively impaired judgment and impulse control
  6. Diminished language skills with disorganized speech
  7. Increasingly impaired ability to plan, initiate, or perform certain purposeful movements (apraxia)

In addition, communication difficulties may include problems expressing thoughts in words, initiating conversations, or comprehending others' words and responding appropriately.

Found on Movement Disorder Virtual University

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:

  1. Increased irritability
  2. A tendency to easily find fault with others
  3. Constant complaining
  4. Suspiciousness< br>
  5. Impulsiveness
  6. Lack of self-control
  7. Lack of interest in acts that previously provided pleasure (anhedonia)
Additional behavioral, emotional, or psychiatric disturbances may also become apparent, including:
  1. Anxiety
  2. Depression
  3. Mania
  4. Depression accompanied by mania
  5. Obsessive-compulsive behaviors
  6. Agitation
  7. Hostile outbursts
  8. Sleep disturbances
  9. Increasing social withdrawal
Severe distortions in thinking may also occur, including false beliefs or delusions, such as fears of persecution. In addition, hallucinations may develop, such as the perception of sounds, sights, or other sensations in the absence of external stimuli.

Found on http://www.mdvu.org/library/disease/hd/hd_sym_neuch.asp

III. DSM-IV

Diagnostic and Statistical Manual of Mental Disorders, 4th Edition

Dementia Due to Other General Medical Conditions

  1. The development of multiple cognitive (Thinking, memory and other functions of the mind.
    1. memory (The cognitive function of capture, registration, assimilation, retention, and retrieval of mental, emotional, and physiological information. Impairment (impaired ability to learn new information or to recall previously learned information)
    2. one (or more) of the following cognitive disturbances:
      1. aphasia (While this term taken literally means absence of speech, it may include all forms of speech or language impairment. (language disturbance)
      2. apraxia (Impairment of ability to execute complex muscle movement despite preserved strength. (impaired ability to carry out motor activities despite intact motor function)
      3. agnosia (failure to recognize or identify objects despite intact sensory function)
      4. disturbance in executive functioning ( Neuropsychological functions including, but perhaps not limited to, decision making, planning, initiative, assigning priority, sequencing, motor control, emotional regulation, inhibition, problem solving, planning, impulse control, establishing goals, monitoring results of action, self-correcting. (i.e., planning, organizing, sequencing, abstracting)
    3. The cognitive deficits in Criteria A1 and A2 each cause significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning.
    4. There is evidence from the history, physical examination, or laboratory findings that the disturbance is the direct physiological consequence of one of the general medical conditions listed below.
    5. The deficits do not occur exclusively during the course of a Delirium. Code based on presence or absence of a clinically significant behavioral disturbance.

DSM-IV 294.1 Dementia Due to Huntington's Disease

A characteristic movement disorder accompanies the Dementia resulting from this progressive inherited disease.
Diagnostic Criteria: Dementia Due to Other General Medical Conditions
American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition. Washington, DC, American Psychiatric Association, 1994

Found on http://www.behavenet.com/capsules/disorders/huntingtons.htm

IV. Medline Plus Medical Encyclopedia

A Service of the U.S. National Library of Medicine and the National Institutes of Health

Dementia

Symptoms:

Problems may involve language, memory, perception, emotional behavior or personality, and cognitive skills (such as calculation, abstract thinking, or judgment). Dementia usually first appears as forgetfulness.

Symptoms include:

  • Changed feeling (sensation) or perception
  • Changed sleep patterns
    • Change in sleep-wake cycle
    • Insomnia
    • Need for increased sleep
  • Decrease in problem-solving skills and judgment
  • Disorientation
    • Confused about people, places, or times
    • Unable to pick up cues from the environment
  • Disorders of problem-solving or learning
    • Trouble making calculations
    • Unable to learn
    • Unable to think abstractly
    • Unable to think in general terms
  • Impaired recognition (agnosia)
    • Trouble recognizing familiar objects or people
    • Trouble recognizing things through the senses
  • Lack of or poor language ability (aphasia)
    • Unable to form words
    • Unable to name objects
    • Unable to read or write
    • Unable to repeat a phrase
    • Unable to speak (without muscle paralysis)
    • Unable to understand speech
    • Have impaired language skills
    • Repeat phrases
    • Speak poorly (enunciation)
    • Use slang or the wrong words
  • Memory problems
    • Unable to remember new things (short-term memory problems)
    • Unable to remember the past (long-term memory problems)
  • Motor system problems
    • Gait changes
    • Impaired skilled motor function (apraxia)
      • Unable to copy geometric figures
      • Unable to copy hand positions
      • Unable to dress self
    • Inappropriate movements
    • Other motor system problems
  • Seeing or hearing things that aren't there (hallucinations) and having false ideas (delusions)
  • Severe confusion
  • Personality changes
    • Anxiety
    • Decreased ability to care for oneself
    • Decreased interest in daily living activities
    • Depression
    • Inappropriate mood or behavior
    • Irritability
    • No mood (flat affect)
    • Not flexible
    • Only concerned with self (self-centered)
    • Poor temper control
    • Unable to function or interact in social or personal situations
    • Unable to keep a job
    • Unable to make decisions
    • Withdrawal from social interaction
  • Unable to be spontaneous
  • Unable to concentrate

Other symptoms that may occur with dementia:

  • Incontinence
  • Swallowing problems

Found on http://www.nlm.nih.gov/medlineplus/ency/article/000739.htm

V. Colombia University’s College of Physicians and Surgeons

Dementia Update for the Practitioner

Other Frontotemporal Dementia: Huntington’s Disease

  • personality change (disinhibition, substance/ETOH use, promiscuity
  • psychosis (delusions, but rarely hallucinations)
  • subcortical dementia (decreased drive, executive function)
  • verbal output decline (spontaneous speech)
  • chorea (UE > LE)
  • gait change

Huntington's disease is not strictly a Frontotemporal dementia but may still be considered one of the frontal dementias.

Huntington's disease is also within the spectrum of frontal-like dementias. It is principally a subcortical dementia in which psychosis and personality changes are the common presenting and prominent features.

Found on http://ci.columbia.edu/c1182/web/sect_5/c1182_s5_5.html

VI. University of Kansas Medical Center

Behavioral Problems in Huntington's disease

The three most profound behavioral problems in Huntington's disease come from the dementia (loss of the ability to reason), the altered perception of the world and the changes in family dynamics…The dementia is technically called a sub-cortical dementia.”

Dementia

The dementia is technically called a sub-cortical dementia. Translated into real world terms this means that a person with Huntington's disease has difficulty performing a sequence of tasks, especially when distracted, while their ability to form new memory and to recall old memories is relatively well preserved. Thus a patient maybe able to do the wash if there is a list of instructions posted in the laundry room, but may fail at doing the wash if they are also trying to make a meal.

Distracting influences can impair the ability of a person with Huntington's disease to accomplish a task. For example, one may be able to eat easily in a quiet environment, but have great difficulty eating in a noisy, crowded room with both a television set and a radio playing.

Found on http://www.kumc.edu/hospital/huntingtons/behavior.html

VII. About.com: Alzheimer’s

Subcortical Dementia

As the term suggests, these are dementias believed to result in structures below the cortex. Huntington's disease, Parkinson's and AIDS dementia complex are three examples. It is more common to see changes in personality and a slowing down of thought processes with this classification of dementia. Language and memory functions appear largely unaffected.

Found on http://alzheimers.about.com/od/typesofdementia/a/cortical_sub.htm

Huntington's disease a fatal progressive form of dementia

Huntington's disease is an inherited progressive form of dementia in which personality, memory and moods change as the disease advances. First described by Dr. George Huntington in 1872, Huntington's disease affects an estimated 30,000 Americans, a prevalence of about 1 in every 10,000 people. The prevalence of Huntington disease is between three and seven per 100,000 in populations of Western European descent. Huntington’s is a degenerative disease characterized by jerking uncontrollable movement of the limbs, trunk, and face, progressive psychiatric difficulties and loss of mental abilities.

Found on http://alzheimers.about.com/od/typesofdementia/a/huntington_dise.htm

VIII. Emedicine

Huntington’s Disease Dementia

Cognitive symptoms include the following:

  • Memory frequently is not impaired until late in the disease, but attention, judgment, and executive functions may be seriously deficient at an early stage.
  • Early signs of dementia often include forgetfulness, disorganization, and affective disorders.
  • Free recall may be severely impaired.
  • Memory deficits involve both recent and remote function. Studies show that patients with HD can accurately recognize recently presented verbal material only when cues are used in memory retrieval. Other studies show that the patients sometimes recall words the next day that they had not recalled during testing sessions, further supporting a delayed-retrieval hypothesis.
  • Patients have significant problems with frontal executive functions, such as problems with maintenance, cognitive flexibility, abstraction, judgment, reasoning, sequencing, organizing, planning, and adapting.
  • Skilled motor-procedural learning deficits are reported. Working memory may be affected in patients with HD because of frontal lobe dysfunction associated with the bidirectional connections with the caudate.
  • Insight, orientation, factual information, and overall intelligence quotient scores are preserved relatively well into the disease process.

Other Conditions Associated With Dementia

When classifying degenerative dementias, 2 major profiles may be considered. The subcortical type primarily affects attention, judgment, and behavior. This dementia pattern resembles such pathologies as HD, Parkinson disease (PD), supranuclear palsy, and dementia of the frontal lobe. Cortical dementias (eg, AD) involve the gray matter cortex and manifest with early-onset memory losses and language disturbances. Patients with subcortical dementias (eg, HD, PD, subcortical vascular dementias) have more motor symptoms than those with temporoparietal or frontal dementias. Unfortunately, clinical characteristics of cortical and subcortical dementias show a subtle overlap, which complicates a distinct diagnosis.

Patients with AD and HD can be differentiated on the basis of specific neurocognitive deficits that are independent of severity. Using the Mattis Dementia Rating Scale, at all levels of severity, patients with HD are found to be more impaired on initiation and perseveration but less impaired on memory. At moderate and severe levels of dementia, patients with HD are more impaired in constructional praxis than patients with AD. These differences relate to the cortical involvement of AD versus the sub-cortical involvement of HD.

Patients with HD perform better than patients with AD on the immediate and delayed trials of logical memory and on the delayed trial of visual reproduction, but only at the mild stage. Patients with HD have better retention of a word list than patients with AD relative to age- and education-matched control groups. While patients with AD do poorly on the Mini-Mental State Examination, patients with HD may do as well as controls. In particular, patients with AD are impaired in learning or encoding and retention. Patients with HD show better retention and fewer tendencies toward cued intrusions. While patients with AD show deficient word storage, they tend to show particular difficulty with word retrieval in the face of relatively better word storage.

When patients with HD and patients with PD are compared, patients with HD are more impaired than patients with PD in immediate free recall and they do not exhibit the expected learning curve across trials. Patients with HD tend to exhibit a strong recency effect (ie, recalling more items from the end of the stimulus list than from the middle or beginning). Patients with PD make slightly fewer so-called errors of perseveration (ie, repetition), which is significantly fewer than the patients with HD. Patients with HD demonstrate significantly more improvement on recognition testing.

In summary, patients with HD are most deficient on tests of delayed recall, followed by performance on measures of memory acquisition, cognitive flexibility and abstraction, manual dexterity, attention and/or concentration, performance skills, and verbal skills.

Found on http://www.emedicine.com/med/topic3111.htm

IX. National Institutes of Neurological Disorders and Stroke

National Institutes of Health

What is Dementia?

Dementia is not a specific disease. It is a descriptive term for a collection of symptoms that can be caused by a number of disorders that affect the brain. People with dementia have significantly impaired intellectual functioning that interferes with normal activities and relationships. They also lose their ability to solve problems and maintain emotional control, and they may experience personality changes and behavioral problems, such as agitation, delusions, and hallucinations. While memory loss is a common symptom of dementia, memory loss by itself does not mean that a person has dementia. Doctors diagnose dementia only if two or more brain functions - such as memory and language skills -- are significantly impaired without loss of consciousness. Some of the diseases that can cause symptoms of dementia are Alzheimer’s disease, vascular dementia, Lewy body dementia, Frontotemporal dementia, Huntington’s disease, and Creutzfeldt-Jakob disease. Doctors have identified other conditions that can cause dementia or dementia-like symptoms including reactions to medications, metabolic problems and endocrine abnormalities, nutritional deficiencies, infections, poisoning, brain tumors, anoxia or hypoxia (conditions in which the brain’s oxygen supply is either reduced or cut off entirely), and heart and lung problems. Although it is common in very elderly individuals, dementia is not a normal part of the aging process.

Found on http://www.ninds.nih.gov/disorders/dementias/dementia.htm

X. About-Dementia.com

Symptoms of HD

The symptoms of Huntington's disease profoundly affect the lives of patients and their families. HD interrupts a person's ability to walk, think, and communicate. Over time, a person with the symptoms of Huntington's disease becomes completely dependent on others for care. These symptoms take a toll on entire families emotionally, socially and economically.

Neuron loss can cause some of the following symptoms:

  • abnormal facial movements
  • abnormal body movements
  • mood swings
  • depression
  • irritability
  • jaw clenching (bruxism)
  • slurred speech
  • difficulty walking
  • personality changes
  • loss of mental capacity (dementia)

All of these symptoms of Huntington's disease become more severe over time as the cells in the brain continue to die. Sometime within fifteen to twenty years of the onset of the symptoms of Huntington's disease, the patient dies. Death is usually due to infection. Sadly, suicide is also a common cause of death, due to the suffering related to the heartbreaking symptoms of Huntington's disease.

Found on http://www.about-dementia.com/articles/about-huntingtons/huntingtons-symptoms.php

XI. Family Caregiver Alliance

National Center on Caregiving

Degenerative (Irreversible) Dementias

If reversible dementias are ruled out and it is determined that the person has a degenerative or irreversible dementia, it is important that families and medical personnel seek the cause of the problem. This will help ensure that the person affected receives proper medical care, and families can plan their Caregiving and find appropriate support and resources.

The following are the most common degenerative dementias:

  • Alzheimer’s Disease
  • Ischemic Vascular Dementia (IVD)
  • Dementia with Lewy Bodies (DLB)
  • Frontotemporal Dementia (FTD)
  • Creutzfeldt-Jakob Disease (CJD)
  • Parkinson’s Dementia
  • Progressive Supranuclear Palsy (PSP)
  • Normal Pressure Hydrocephalus (NPH)
  • Huntington’s disease (HD) Huntington’s disease is a fatal disease typically characterized by involuntary movements (chorea) and cognitive decline (dementia). It is caused by a genetic mutation that can be passed down from generation to generation. HD is an illness with profound neurological and psychiatric features affecting certain structures deep within the brain, particularly the basal ganglia, responsible for such important functions as movement and coordination. Structures responsible for thought, perception and memory are also affected, likely due to connections from the basal ganglia to the frontal lobe of the brain. As a result, patients may experience uncontrolled movements (such as twisting and turning), loss of intellectual abilities, and emotional and behavior disturbances.
  • Mixed Dementias

Found on http://www.caregiver.org/caregiver/jsp/content_node.jsp?nodeid=569

XII. New Techniques for Understanding Huntington's Disease

J Neuropsychiatry Clin Neurosci 11:173-175, May 1999

All patients with HD develop a progressive subcortical dementia that is characterized by frontal lobe executive dysfunction and memory deficits. Neuropsychological testing reveals deficits in recent and remote memory; impaired visuospatial function; difficulty with shifting sets, planning, and organization; and overall decreasing IQ. Psychiatric syndromes (present in up to 79% of patients) most commonly include impulse control disorders, depression, personality changes, and, more rarely, psychosis or mania. Symptoms include disinhibition, irritability, aggression, apathy, and Neuro-vegetative markers of depression. The suicide rate has been reported to be up to 20 times that of the general population over age 50. Increased criminal behavior and hypersexuality have often been reported in HD patients. A recent Danish study found that there is a statistically significant increase in nonviolent crime in male HD patients compared with their non-affected relatives and control subjects. These criminal behaviors, as well as the psychiatric syndromes, are felt to be related to the destruction of the medial caudate, which disrupts the memory and emotion tracts descending from the frontal lobes.

XIII. eMedicine

Huntington Disease:
Cognitive decline is characteristic of HD, but the rate of progression among individual patients can vary considerably. Dementia and the psychiatric features of HD are perhaps the earliest and most important indicators of functional impairment.

The dementia syndrome associated with HD includes early onset behavioral changes, such as irritability, untidiness, and loss of interest. Slowing of cognition, impairment of intellectual function, and memory disturbances are seen later. This pattern corresponds well to the syndrome of subcortical dementia, and it has been suggested to reflect dysfunction of frontal-subcortical neuronal circuitry. (The so-called cortical dementias primarily involve the cerebral cortex and are associated with aphasia, agnosia, apraxia, and severe amnesia.)

Early stages of HD are characterized by deficits in short-term memory, followed by motor dysfunction and a variety of cognitive changes in the intermediate stages of dementia. These deficits include diminished verbal fluency, problems with attention, executive function, visuospatial processing, nd abstract reasoning. Language skills become affected in the final stages of the illness, resulting in a marked word-retrieval deficit.

Found at http://www.emedicine.com/neuro/topic81.htm

Compiled and written by Phillip J. Hardt, BA, MA Huntington’s Disease Advocate, Author and Diplomat on 6/11/2009 phardt1@cox.net or 602-309-3118.

Phil Hardt
phardt1@cox,net