Juvenile-HD

HOME

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
Epilepsy-Seizures~PG

INDEX Page

 

Chapter 3-The Physicians Guide To The Management of HD

Movemement Disorder

Myodonus, Tics and Epilepsy

Myoclonus, sudden brief jerks involving groups of muscles, is more common in juvenile-onset HD, where it may be mistaken for a seizure. Like chorea, myoclonus may not be disabling or particularly distressing, but may respond to treatment with clonazepam or divalproex sodium if treatment is necessary.

Tics are brief, intermittent stereotyped movements such as blinking, nose twitching, head jerking, or transient abnormal postures. Ties which involve the respiratory and vocal apparatus may result in sounds including sniffs, snorts, grunts, coughs, and sucking sounds.

Patients may be unaware of vocal tics, but family members may find the incessant noises grating. They should be helped to understand that the tics are not under voluntary control. Tics generally do not by themselves require treatment, but may respond to neuroleptics, benzodiazepines, or SSRIs.

Epilepsy is uncommon, though not unheard of, in adults with HD, but is said to be present in 30% of individuals with juvenile-onset HD.

A first seizure in an HD patient should not be attributed to HD without further evaluation as it may be indicative of  an additional neurologic problem, such as a subdural hematoma sustained in a fall.

The workup of a first seizure should include a complete exam, laboratory studies to rule out an infection or metabolic disturbance, an EEG, and a brain imaging study. The treatment of a seizure disorder in a person with HD depends on the nature of the seizures.

In the juvenile HD patient, myoclonic epilepsy or other generalized seizures may suggest divalproex sodium as a first treatment choice.

Although seizure management in HD is not usually difficult, for the occasional patient seizure control is quite difficult to achieve, requiring multiple medications or specialized referral. ======================================================

In the following it is interesting to see the types of seizures from dehydration/electrolytes which is critical in someone not consuming sufficient amounts of clear liquids each day!

Seizure- Convulsion. Epilepsy
http://www.fpnotebook.com/NEU183.htm
(See links for causes, signs & symtpoms and treatments)

See Also
    Single Seizure Evaluation
http://www.fpnotebook.com/NEU184.htm
    Status Epilepticus http://www.fpnotebook.com/NEU185.htm
    Febrile Seizure http://www.fpnotebook.com/NEU188.htm
    Newborn Seizure  http://www.fpnotebook.com/NEU143.htm

Primary Seizure Types
    Absence Seizure (Petit Mal Seizure)
http://www.fpnotebook.com/NEU187.htm
    Generalized Tonic Clonic Seizure
         (Grand Mal Seizure) http://www.fpnotebook.com/NEU189.htm
    Simple Partial Seizure http://www.fpnotebook.com/NEU190.htm
    Complex Partial Seizure http://www.fpnotebook.com/NEU191.htm
    Rolandic Epilepsy http://www.fpnotebook.com/NEU192.htm

New Onset Secondary Seizure Causes by age
    Age under 10 years
        Idiopathic
        Congenital
        Birth Injury
        Metabolic disorder
    Age 10 to 40 years
        Idiopathic
        Head Trauma
http://www.fpnotebook.com/ER40.htm
        Pre-existing focal brain disease
        Drug Withdrawal
http://www.fpnotebook.com/PSY29.htm
    Age over 40 years
        Brain tumor
        Old Cerebrovascular Accident
http://www.fpnotebook.com/NEU25.htm
        Head Trauma

Non-Epileptic Causes of Seizure
    Cardiogenic
        Syncope
http://www.fpnotebook.com/CV209.htm
        Transient Ischemic Attack http://www.fpnotebook.com/NEU26.htm
        Arrythmia
        Sick Sinus Syndrome
    Electrolyte
        Hypocalcemia/Low Serum Calcium/
http://www.fpnotebook.com/REN10.htm
        Hyponatremia (Water intoxication)
            Low Serum Sodium http://www.fpnotebook.com/REN116.htm
        Hypomagnesemia Low Serum
                Magnesium http://www.fpnotebook.com/REN87.htm

    Metabolic disorders
        Abnormal Glucose (especially Hypoglycemia)
        Hyperthyroidism
http://www.fpnotebook.com/END147.htm
        Thyroid Storm http://www.fpnotebook.com/END150.htm
        Vitamin B6 deficiency http://www.fpnotebook.com/PHA84.htm

    Drug Withdrawal http://www.fpnotebook.com/PSY29.htm
        Alcohol Withdrawal http://www.fpnotebook.com/PSY31.htm
        Benzodiazepine Withdrawal http://www.fpnotebook.com/PSY43.htm
        Cocaine withdrawal http://www.fpnotebook.com/PSY42.htm
        Barbiturate withdrawal http://www.fpnotebook.com/PSY42.htm
        Meperidine withdrawal Demerol http://www.fpnotebook.com/PHA29.htm
    Drug intoxication or overdose
        Cocaine
        D-Amphetamine
        Theophylline
        Isoniazid (INH)
        Lithium
        Nitrous Oxide
        Acethylcholinesterase inhibitor
    Metal toxicity
        Mercury poisoning
        Lead Poisoning
http://www.fpnotebook.com/HEM135.htm
    Infectious disease
        Gram Negative Sepsis
http://www.fpnotebook.com/ID46.htm
        Viral Meningitis http://www.fpnotebook.com/NEU117.htm
        Bacterial Meningitis http://www.fpnotebook.com/NEU115.htm
    Miscellaneous
        Hyperthermia
        PseudoSeizure
        Malignancy
        Idiopathic
        Isolated unprovoked, non-epileptic Seizure
=================================================
Seizure studies to watch   October 16, 2001

Cleveland Clinics Clinical Trials
http://www.clevelandclinic.org/health/research/
CAPS my input/Jean Miller

TOPIRAMATE FOR COGNITION?
New Topiramate Study The Cleveland Clinic Foundation is beginning a study investigating the effects of the antiepileptic drug topiramate on cognition and vigilance using self-administered computer neuropsychological batteries, polysomnography and the multiple sleep latency test. Patients with epilepsy who have incompletely controlled seizures may qualify for treatment with the new antiepileptic drug. The study requires five visits to The Cleveland Clinic Foundation.

CARBATROL FOR SLEEP & ALERTNESS
Opportunity for Patients with Newly Diagnosed/Untreated Epilepsy The Cleveland Clinic Foundation is beginning a study investigating the effects of the antiepileptic drug Carbatrol (carbamazepine) on nighttime sleep and daytime alertness. Carbamazepine is a commonly used, FDA-approved medication used in the treatment of partial (focal) seizures. Untreated
patients interested in participating would undergo sleep evaluation including sleep questionnaires and a baseline overnight sleep study followed by a "daytime nap" test at no charge. Subjects would be treated with carbamazepine for several weeks and then undergo a second sleep
evaluation.

HARMONAL AFFECT ON SEIZURES?
For Women Only Women and adolescent girls recently diagnosed with a seizure disorder who are not yet taking anti-epileptic medications may be eligible to participate in a new study being conducted at the Cleveland Clinic. Hormonal, metabolic and ovarian functions will be evaluated in females using either lamotrigine or valproic acid.


NEW DRUG HARKOSERIDE
For Refractory Patients Adults who continue to have difficulty controlling their partial seizures may be eligible to participate in a study to begin soon at the Cleveland Clinic. This five month study will investigate the efficacy of harkoseride as an add-on treatment in the control of partial seizures with or without generalization.

NOTE: Kelly's seizures were typically only during her menstrual cycle when her choretic movements were accelerated.  Earlier discussions with doctors on the relationship to her mentstrual cycle and chorea were dismissed as a whacko mother trying to the determine NON HD causes when of course they all knew everything was HD related (era late 1980's).  In the early 90's I heard there was a study on female HD patients to determine relationship between menstrual cycles and chorea.  I haven't been able to find the outcome of that study.