Juvenile-HD

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INDEX Page
Tools For Viewing
10 The Most Commonly Asked Questions
Clinical Trials & Research
Huntington's Disease~WeMove Info
Advocacy/Donations/Press Info
Clinical Definition & Search
Facing HD~Family Handbook
JHD Handbook-Chapter 1
JHD Info-Stanford Univ.
Physician's Guide To HD
Caring for People with HD
Physical & Occupational Therapy In HD
Understanding Behaviour in HD-Dr. Jane Paulsen
Understanding Behavioral-Dr. Edmond Chiu
Advanced Stages Caregivers Handbook
First Shift-Certified Nursing Assistants
Activities of Daily Living-HD
Unified HD Rating Scale (UHDRS) Motor Section
Westphal Variant
SECTION 1 - AT RISK
Age & Probability Chart
At Risk For HD-What Next?
At-Risk Checklist
Best Interest of Child?
Crystal Ball?
Food For Thought
Parent Hasn't Tested?
Q&A On Risk of Inheriting JHD
Testing Children
SECTION 2 - GENETIC TESTING
Genetic Disorders & Birth Defects
Genetic Testing for HD
Genetic Counseling-In General
Psychological Impact
Intro: Genetics/Genetic Testing
Prenatal & Preimplanation
Prenatal Testing-In General
o Genetic Testing Resources
o Personal Stories
SECTION 3 - JHD
Coping With The Early Years
Age of HD Appearance
Age of Onset-Historical
Family-HD Underestimated
Children of Parents With HD
Child~Parent Ill
Clinical Description JHD
HD - What Kids Are Saying
HD & Me
JHD-Duration of Illness
JHD-Clinical and Research
JHD Symptoms
Parenting With HD
Patients/Families Coping
Talking With Children About HD
5 Stages of HD
JHD Resources
SECTION 4 - SYMPTOM RECOGNITION
Parent Resources
8 Fears of A Chronic Illness
Anxiety/Apathy/Irritability~HD
Anxiety, Fears & Phobias
Apathy-Physician's Guide
Ataxia
Attention-Perceptual/Unawareness Physician's Guide
Bed/Pressure Sores
Bed/Pressure Ulcer Guideline
Behavior Management
Bi-Polar Disorders
Botulinum toxin therapy
Bradykinesia
Caring Tips
Child Abuse-Reconizing Signs
Chorea-Physician's Guide
Chorea
Cognitive/Decision Making/Impulsivity
Cognitive-Short Tips
Contractures~Joints Locking
Dehydration-Physician's Guide
Dehydration
Delirium
Denial of HD
Depression~Physician's Guide
Depression-Understanding It
Depression-How To Help
Depression - Treatment Resistant Patient
Depression-Other Resources
-Read If Your Child Is On Antidepressant
Disgust - Impaired Recognition in HD
Dissociative disorders
Driving - Physician's Guide
Dyslexia
Dyslexia Resources
Dystonia
Dystonia/Rigidity & Spasticity Physician's Guide
Dystonia-Predominant Adult-Onset HD
Epileptic Seizures and Epilepsy
Epilepsy-Seizures~PG
-Seizures ~Special Populations
Falling~Safety
Falling - Subdural Hematoma Risk
Fevers - Unexplained
Fevers, sweating & menstural cycles in HD
GERD (Stomach)
HD Principle Treatments
Hallucinations/Psychosis~PGHD
Hand muscle reflexes in HD
Hypothalamus - A Personal Theory
Insomia ~Physician's Guide
Irritability~Temper Outburst Physician's Guide
Learning Disability
Mania/OCD~Physician's Guide
Mood Disorder Rate In HD
Myoclonus (Movements)
Nails-What To Look For
Night Terrors
Obsessive Compulsive OCD
Panic Disorder
Personality disorders
Pneumonia
Pneumonia-Advanced Stages
Pneumonia - Aspirated (Inhaled)
Prosody - Social Impairment
Sexuality~Physician's Guide
Skins Sensitivity
Sleep Disorders
Smoking-Physician's Guide
Spasticity
Stress
Tremors
Why Certain Symptoms Occur
Symptom & Treatment Resources
SECTION 5 - COMMUNICATION
Communication Resources
Communication Problems
Communication Strategies For HD~Jeff Searle
SECTION 6 - EATING/SWALLOWING/NUITRITION
Hints For Weight Loss in HD
HD & Diet~HSA Fact Sheet 7
Nutrients: Some Possible Deficiency Symptoms
Nutrition and HD~Anna Gaba (Recipes)
Nutrition Information In HD~Naomi Lundeen
Speech & Swallowing~Lynn Rhodes
Swallowing & Nutrition Physician's Guide To HD
Swallowing & Nuitrition Resources
Swallowing Warning Signs
5 Swallowing Problems
Taste changes in HD
Weight Gain
Resources-Drinks/Shakes
-Feeding Tubes~Advanced Stages of HD
-Feeding Tube~Jean Miller
-Feeding Tubes: One More Word ~Jean Miller
-Feeding Tubes & Baby Foods
-Feeding Tube~Dental Care
-Feeding Tube Instructions~Jean Miller
-Feeding Tube Resources
SECTION 7 - THERAPIES
Finding a Therapist - Behavoir
What Is A Physiotherapist?
Physical Therapy In HD
Speech-Language Therapy
Therapy Descriptions
Therapy Resources- Easter Seal
Therapy Resources
SECTION 8 - MEDICATIONS
HD Treatments
Medications-Movement Disorders
Medication/Emergency Info Forms
Cutting Prescriptions
Drugs-Look 'Em Up
-Adolescents Under 25
-Antidepressant Adverse Effects
-Anti-psychotic
-Anxiety-Antidepressant
A-Z Mental Health Drugs
-Creatine
-EPA~Fish Oil
-Haldol/Haloperidol - Clinical Sheet
-Haldol~Clinician Description
-Haldol & HD
-Haldol/HD Patient Experiences
-Haldol~ Patient Handout
-Mood Stabilizers: ASK 3 Questions
-Neuroleptic Malignant Synd WARNING
-Olanzipine-Risperidone/blood tests
-Celexa/Luvox/Paxil/Prozac/Zoloft
-Psychiatric Drugs & Children
Sertraline ~Zoloft
-Spasticity Meds/Treatments
-SSRI Medications
-Tardive Dyskinesia WARNING
-Weight Gain Medications
-Sites/Help the Medicine Go Down
-Vitamin & Mineral Deficiencies
SECTION 9 - SURGERIES
Surgery-Movement Disorders
o Surgery Resources
SECTION 10 - PROCEDURES
Clinic Visits-How To Prepare
CT Scans, MRI's etc.
Swallowing Tests
Tests Commonly Used
o Procedures Resources
SECTION 11- ALCOHOL/DRUGS
Alcohol-Parent's Guide
Alcohol-Talking To Your Child
Drugs-What To Do?
Drugs-Talking To Your Child
Disciplining-Ages 0-13 & Up
SECTION 12- SUICIDE
Straight Talk On Suicide
Teen Suicide-You Need To Know
o Suicide Resources
SECTION 13 - DIVORCE
Divorce & Child Stress
Tips For Divorcing Parents
SECTION 14 - DISABILITY ISSUES
Guides To Disability Issues
Caring-Child & Medical Technology
Caring for a Seriously Ill Child
Child Long Term Illness
Disability-Special Education Plan
IFSP Early Intervention Process
Disability Resources
Financial Planning
Wishes Can Come True-Children's Wish Foundations
Special Needs Resources
Special Needs Camp - About
Special Needs Camp - Finding One
SECTION 15 - ASSISTIVE TECHNOLOGY
Child Assistive Technology
Adaptive Equipment Resources
Products
SECTION 16 - EMOTIONAL ISSUES
Signs of Unhealthy Self-Esteem
Emotional Behavior Links
o Emotional Support Resources
SECTION 17 - GRIEF
Helping Child Deal With Death
o Grief Addtional Resources
SECTION 18 - ADD/ADHD
ADD & Teens
Conduct Disorders
FAQS & Related Info
Understanding AD/HD
What Is AD/HD?
Research Articles
Resources
SECTION 19 - HD SUPPORT GROUPS
HD Support Groups
National Youth Association
SECTION 20 - HD LINKS
HD Links
Related Resources
Tips For Friends
SECTION 21 - BENEFITS/INSURNACE
HD Disability
Benefits Check UP - See What You Can Get
Medical Insurance Bureau's Facts On You!
Medicare-Medicaid
Medicare Rights-Home Health & Hospice
Medicare Rights Center Resources
No Insurance? Try This!
Prescription Drug Cards Part I
Prescription Drug Cards Part II
Social Security-Children With Disabilities
SECTION 22 - ARTICLES/JHD
JHD and ADD
SECTION 23 - CAREGIVING
Articles-Resources
Caregiver Self-Assessment
Caregiver's Handbook
"First Shift With A Person With HD"
Getting Respite Care/Help At Home
Helpful Forms-Info
Home Emergency Preparations
Symptom Management
Ten Tips
Useful Tools
SECTION 24 - BIO
Our Personal Experience
Coping At The End
Kelly E. Miller
Song & Verse
Letter From My Heart
GUESTBOOK
Why Certain Symptoms Occur

INDEX Page

Also see
The Brain - How it Functions (in understandable language)
http://kidshealth.org/kid/body/brain_SW_p2.html
 
The Huntington's Society of Canada wrote: The website you sent me was the correct one for the article on the brain, and our Director of Communications is now going to try to get permission for us to use it with our HD families. I think that it's really good, and it will be helpful for kids as well as for adults who do not have a medical background. 


This article is from  the HDSA Arizona Chapter website: http://www.hdsa-az.org/
 
From discussions, we've seen more "sensorary" functions such as itching, hot and/cold,  noises/hearing. etc as directly related to HD symptoms. Others, such as Kelly in later stage, had occipital/vision changes or sensitivity to light.
~~~~~~~~~~~
Why Do Certain Symptoms Occur In Huntington's Disease (HD)
- A Tour Through the Brain.

John N. Caviness, M.D.
Medical Advisor to HDSA-AZ board
Mayo Clinic Scottsdale
13400 E. Shea Boulevard  
April 11, 2001
 
 
George Huntington described Huntington's disease (HD) is 1872.  He took note of the involuntary  movement (chorea) that occurred in families.  Family members seemed to inherit the disorder in a certain pattern that we now call autosomal dominant.  These movements caused decreased control of voluntary movements and balance.  In addition, the mental abilities of people with HD changed.  
 
Today, we know that HD comes from the abnormal genetic code in a certain location on chromosome four.  We hope that this knowledge will allow a cure for HD to be found.  Even so, why do certain symptoms occur in HD?  It is important to have an understanding  of why these symptoms occur in people with HD.  Recognizing the symptom allows one to better  adapt and deal with it.
 
The brain sits in the skull and has a jelly-like consistency.  Over its surface, there are many folds and crevices. Multiple folds are grouped together under the term, "lobe."  The lobes of the brain have names and take on certain functions.  These functions are not completely understood and often have more than one location, but some general statements can be made:
 
 LOBE NAME             BEHAVIORAL FUNCTION
 
Frontal lobe                        Concentration, Judgement, Planning, Motor  Coordination
Parietal lobe                       Sensory function, perception, recognition of  space relations
Temporal lobe                    Memory, emotional control, language
Occipital lobe                    Vision
 
In HD, there is a tendency for the frontal and temporal lobes to be affected.  Nothing is absolute and each case is different.  However, after realizing that the frontal and temporal lobes experience major problems with executing their function in HD, some common symptoms in HD become more understandable. 
 
Some of the common symptoms in HD are coordination problems, concentration difficulties, less judgement, planning difficulties, decrease memory ability, emotional withdrawal, and less emotional control.  These symptoms are at least partially tied to dysfunction of the frontal and  temporal lobes. 
 
Sensory function, perception of touch and recognition of space relations are usually not affected or minimally involved in HD.
 
Deeper in the brain, below the lobes, are places referred to subcortical nuclei.  These centers have to do with motor execution and control, among other functions.  These are also affected in HD and their dysfunction can create involuntary movements, abnormal swallowing, balance problems, and speech difficulty.
 
Of course, the best way to treat a disease is to treat its basic cause.  This is the approach being taken by many researchers in HD.  Studies, such as PHAROS which is an observational study for persons at-risk for the HD gene, is being performed at Mayo Clinic Scottsdale as part of the Huntington's Disease Study group, will help attain that goal.
 
NOTE:    To find out more about the HD Study Group and on-going
clinical trials, go to  http://www.huntington-study-group.org

HD Patients' Symptoms Vary
By Laura Bruni
http://www.lib.uchicago.edu/~rd13/hd/symptoms.html

Transcribed from the Dimensions section of the Park Forest Star, March 14, 1996, with permission from the author. Dr. Kathleen Shannon, a neurologist with a genetic testing center for Huntington's disease, lists the range of symptoms the disease commonly causes. Victims, who span all races and ethnicities, may show any combination of symptoms,complicating diagnoses and symptomatic treatments.
 
Common symptoms fall into three categories.

Motor problems include clumsiness, decrease in coordination, slurred speech, difficulty in swallowing, chorea (involuntary movements), poor balance, problems walking, intoxicated appearance, and twitchy and fidgety spasms.

Personality changes -- irritability, temper tantrums, sometimes violent outbursts, depression, paranoia and obsessive compulsive behavior.

Cognitive difficulties, which tend to show up later on in the disease, can involve memory, mental flexibility, fluency of thought, organization, an inability to make a mental strategy, and poor communication. The latter can be especially frustrating to the patients exacerbating other areas. "They can feed off each other a lot," Shannon notes.

There are now 25,000 to 30,000 people afflicted with the disease in the United States. An additional 150,000 are at risk. Research on the disease, Shannon explains, involves several areas of study. One is better treatment of symptoms. "This doesn't affect the long-term degenerative nature of the disease," she notes, "but it vastly improves the quality of life for the patients."

Other research is now focusing on blocking the chemicals that may be damaging the brain cells, on strengthening the cells to resist damage, and on cell regeneration by either helping cells repair themselves or replacing dead or damaged cells.

 
Following is from the Blake Medical Center
On the left hand side, under "Family & Friends" click on "Virtual Body"
 
The Human Brain
Skeleton
Human Heart
Digestive Tract
 
The  Human Brain
 
The human brain controls every action, thought, and process of the body; but what makes the brain function? 
 
A network of nearly one hundred billion individual cells called neurons, or nerve cells, allows the brain to think, remember, hear a symphony, enjoy a sunset, love and imagine.  Neurons tell our bodies to move, breathe, and create.  The amazing network of neurons that forms the human brain makes it the most complicated brainon the planet.
 
This system is based on electrochemical impulses.  Like a computer, these impulses travel along circuits.  Unlike computers, these signals are created chemically.  These chemical transmissions are called neurotransmitters, and they are responsible for the incredibly rapid communication betweenneurons.  The speed and accuracy of these neurotransmissions are what let us react to the world around us, and ultimately, to survive.
 
Faulty functioning of this communication between neurons may be responsible for all sorts of illnesses, from depression to Parkinson's disease.  An overabundance of neurotransmitters has been linked to schizophrenia.  Various medications that increase or decrease neurotransmitters are now available and have been shown to be effective in treating some of these conditions.

 
 
The basic nutrient employed by neurons is glucose, a simple sugar.  In order to metabolize the glucose, neurons need lots of oxygen.  While the brain is only two percent of the body's weight, it uses twenty percent of its oxygen.  Because the liver actively makes glucose from carbohydrates, fats and sugars, there is rarely any shortages of glucose for the brain.  However, the brain needs Thiamine or vitamin B1 in order to process glucose.
 
Neurotransmitters, the chemical messengers of the brain are produced by the various nutrients in our food.  These neurotransmitters include serotonin, epinephrine, norepinephrine, dopamine and acetylcholine.  The
brain can continue processing these even if a person does not eat for a while, but nutrition contributes greatly to one's brain functions.
 
Milk and cauliflower contain choline, which helps produce acetylcholine, which aids in the transmission of impulses.   Lecithin, the substance that helps to make choline, can be found in soybeans, peanuts, egg yolks, butter and liver.
 
Proteins contain amino acids such as phenylaianine and tyrosine, which help form such neurotransmitters as  epinephrine, norepinephrine and dopamine.  These influence our emotions, and too much epinephrine can cause  excitability, irritability and anxiety.
 
Tryptophan, the amino acid responsible for the production of serotonin, is abundant in turkey, as well as in  other meats and fish.  Serotonin is thought to be the neurotransmitter responsible for perception and sleep
function.  In order to increase the amount of tryptophan received by the brain, one must combine carbohydrates  and proteins.  This is because carbohydrates increase the body's release of insulin, which transports other  amino acids from the blood into cells, leaving a higher concentration of tryptophan in the blood for the brain.
 
Voluntary & Involuntary Brain Functions
 
Medulla
In addition to allowing us to make deliberate decisions, the brain controls those body functions over which we  have no control.  The lower area of the brain known as the medulla controls heart rate, breathing, vomiting,
salivation, coughing and other automatic functions
 
These automatic functions are a great advantage.  Imagine  having to tell yourself to breathe, or tell your heart to beat!  Because of the importance of these functions,  damage to this area of the brain and spinal chord is very dangerous.
 
Cerebral Cortex
Contains about 85% of the nerve cells in the brain and the human cortex has become increasingly complex in  comparison to other species.  It is divided into Temporal, Frontal, Parietal and Occipital lobes. 
 
The cerebral cortex contains the gray matter of the brain and the prefrontal area of the cerebral cortex comprises a larger portion of the human brain then the brains of other species.  Yet, the prefrontal cortex develops more slowly  in humans than it does in monkeys.  Large numbers of neuronal connections develop in the human brain
between 7 and 12 months.
 
Frontal Lobe
The frontal lobe controls planning of movements and some aspects of memory. 
 
Parietal Lobe
Controls body sensations like the sense of touch.
 
Occipital Lobe
Controls visual functions.
 
Temporal Lobe
Controls hearing and some advanced visual processing.

"suggests that the right frontal lobe "is the glue holding together a sense of self."Hence damages to that area of the brain in HD could contribute to personality changes?
============================================

Brain area holding 'sense of self' found

NEW YORK, Sep 24 2001 (Reuters Health) - "I think, therefore I am" may be a sound philosophy. But if a certain part of the brain isn't working right, you might not be sure who you are, scientists have found.

They say the right frontal lobe appears to be key in holding on to a sense of self--from political persuasions to fashion sense. In a study of men and women with damage to that brain area, they found that six out of seven went through profound personality and lifestyle changes.

University of California researchers studied 72 patients with frontotemporal dementia (FTD), a type of mental decline that affects the brain's frontal and temporal lobes. When the left side of the brain is more affected than the right, patients have trouble with language. When the
problem is predominantly on the right, patients' behavior is often disturbed.

"Remarkably," the authors write in the September 11th issue of Neurology, "we have observed that patients with predominantly right frontal FTD often evolve dramatic changes in well-established patterns of religion, political philosophy, dress and style."

In this study, seven of the 72 patients had predominantly right frontal FTD. Six of these patients--but only one of the remaining patients--showed a strong "change in self."

Religious conversions, shifts in morals, wardrobe makeovers and turnarounds in temperament were among the changes, according to Dr. Bruce L. Miller, of the University of California, San Francisco, and his colleagues.

One woman in her 50s developed an alternate personality she called "Jenny"--a person she described as "irritable, aggressive and domineering." Another woman shifted her political inclinations in her 60s, adopting an animal-rights, anti-conservative stance.

Another woman who had always been "meticulously health conscious" took to sodas and junk food and starting smoking. And a conservative businessman sold his business at age 40, took odd jobs from which he was repeatedly fired, and shed his "puritanical" views on sex for more "experimental" ones.

All of this, according to Miller's team, suggests that the right frontal lobe "is the glue holding together a sense of self."

They note that patients with FTD on the left side tend to lose their comprehension of words like"Catholic" or "Republican," but they do not shed such identities.

While knowledge of who we are is stored throughout the brain, the report indicates, the right frontal lobe appears to contain the "activator" for using and maintaining that knowledge.

SOURCE: Neurology 2001 September.
http://www.reutershealth.com/archive/2001/09/24/eline/links/20010924elin008.html