Juvenile-HD

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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
Surgery-Movement Disorders

INDEX Page

 
 
Orthopedic procedures

Orthopedic procedures may be very helpful at fixing particular mechanical impairments associated with spasticity. Tendon lengthening procedures, rotation of bone segments, or cutting tendons or muscles may reduce the mechanical effects of severe contraction and lead to significantly improved performance.

Orthopedic procedures may be helpful for children with spasticity; however, they are rarely beneficial for children with dystonia.

As with all treatments for spasticity, it is important to assess whether the spasticity is the cause of the child's functional problems or whether it is in fact helping to compensate for muscle weakness. Gait analysis may be particularly helpful in determining whether an orthopedic procedure is likely to be helpful or worsen the child's function. Before or as an adjunct to surgery, many orthopedists attempt serial casting. During casting, sequences of casts are applied to progressively lengthen a particular muscle by providing increasing stretch.

If the primary problem is dystonia or another movement disorder, surgery and casting are only rarely benefical.

Dorsal Rhizotomy

Because spasticity is probably due to increased activity in the stretch reflex, dorsal rhizotomy was developed as a procedure to cut the sensory nerve fibers from the muscles where they enter the spinal cord. This procedure was performed to lessen the "loop" from sensory fibers to excessive muscle activity. Experienced surgeons have reported excellent results with this procedure in some children; it is important to carefully select appropriate candidates for this surgery.

In particular, rhizotomy is not helpful for children with dystonia or parkinsonian rigidity. Dystonia, rigidity, and spasticity must be correctly diagnosed to determine which children are likely to benefit from this procedure.

Deep Brain Lesion

There is a long history of making lesions in the thalamus and basal ganglia as a treatment for tremor, parkinsonism, chorea, and dystonia. Modern, advanced neurosurgical techniques with precise localization of the lesions have led to a significant improvement in the efficacy of these procedures.

The results of deep brain lesions are often remarkable, with almost complete resolution of symptoms.

  • Tremor may respond to lesions in the thalamus
  • Dystonia may respond to lesions in the thalamus or sensorimotor region of the internal globus pallidus
  • Parkinsonism may respond to lesions in the subthalamic nucleus or internal globus pallidus

In some cases, there has been almost complete resolution of symptoms.

In children, this surgery has been used to treat severe generalized dystonia. Unfortunately, many children do not achieve benefit with these procedures or are not candidates for the procedures due to medical issues or concern over side effects. When used to treat dystonia, the benefits of a lesion in the globus pallidus may not be evident for several months.

Children with dystonia due to an inherited genetic cause seem to be more likely to have good results than children with dystonia due to a brain injury. Results with treatment of chorea have been disappointing; however, continued efforts are underway. Currently, the success with deep brain stimulation (DBS) has led to a decrease in the use of lesions; there are still situations in which a lesion is more appropriate.

Deep Brain Stimulation (DBS)

Deep brain stimulation (DBS) is a modern technique that is based on the results of deep brain lesions. In DBS, a pacemaker is implanted in the chest wall; a wire from the pacemaker connects to a terminal within the basal ganglia. The procedure to implant the stimulator is lengthy, since the precise location must be found, using a combination of MRI and electrical measurement of basal ganglia activity. Older children may remain awake during most of the procedure, but younger children will require sedation or anesthesia. When the pacemaker is turned on at a rapid rate, it may block activity in the region of the basal ganglia near the end of the wire. In dystonia, the effect of treatment may not be evident until after several months, but some children have shown remarkable improvement.

The effects of DBS are thus similar to those of a deep brain lesion. The advantages of DBS are that the strength and frequency of the stimulation may be adjusted and the location may be adjusted since there are usually four possible contacts on the wire that may be alternately selected (using an external magnetic programmer). In addition, should the effects of DBS not be as desired, the wire and pacemaker may be removed.

The disadvantages of DBS are the need for occasional reprogramming of the pacemaker; the need for a surgical procedure to replace the batteries every few years; and the potential for infection at the pacemaker or the point where the wire enters the skull. DBS must be used with caution in children; it is difficult to predict who will benefit. The long-term side effects of DBS are not yet known. Nevertheless, this is a promising technique and active research programs continue throughout the world.

Baclofen Pump

Baclofen acts by stimulating GABA type B receptors in the spinal cord and is often helpful in the treatment of spasticity. In order to avoid the side effects of high oral doses of baclofen, the medicine may be continuously administered by implanting pump mechanism in the abdomen. The pump, containing a small reservoir filled with baclofen, is directly connected by tubing into the lower part of the spine. Very small doses are given directly to the spinal cord. The pump is refilled by injecting directly through the skin into its reservoir. This technique is extremely effective for some children. If it is not effective, the pump and tubing may be removed without significant permanent effects.

Unfortunately, there is a high rate of complications. These include infections as well as rapid and serious withdrawal reactions, if the medication in the reservoir is depleted.

Source:  We Move.  Visit their website at www.wemove.org/kidsmove/
Kids Move is WE MOVE's  Web site devoted to pediatric movement disorders. Healthcare professionals and parents  may access up-to-date information about the recognition, assessment, treatment, and avenues of support that are  available for individuals concerned with childhood movement disorders
 
Vagus Nerve Stimulation
This treatment involves the implantation of a generator that stimulates the vagus nerve and thus reduces seizure activity.  To read all about the Vagus Nerve Stimulation, read  this Neurology Channel information. Click on title to be taken to their website: