Make your own free website on Tripod.com
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
Chorea

INDEX Page

 

Definition

Chorea is an irregular, rapid, uncontrolled, involuntary, excessive movement that seems to move randomly from one part of the body to another.

The movements of chorea, athetosis, choreoathetosis, and ballism occur by themselves, without conscious attempts at movement.

The affected child often appears fidgety or restless and unable to sit still. The word "chorea" comes from the Greek word for dance. The jerky movements of the feet or hands are often similar to dancing or piano playing. When chorea is severe, the movements may cause motion of the arms or legs that results in throwing whatever is in the hand or falling to the ground. Walking may become bizarre, with inserted excessive postures and leg movements. Unlike parkinsonism, which is an inability to make voluntary movements, or ataxia and dystonia, which affect the quality of voluntary movements, the movements of chorea, athetosis, choreoathetosis, and ballism occur by themselves, without conscious attempts at movement. In some cases, attempts to move may make the symptoms worse.

Athetosis is a slower writhing and twisting movement. Choreoathetosis is a movement of intermediate speed, between the quick, flitting movements of chorea and the slower, writhing movements of athetosis. Ballism is a violent flinging of one or more limbs out from the body. Choreoathetosis is the most common form in children.

These disorders may affect the hands, feet, trunk, neck, and face. In the face, they often lead to nose wrinkling, continual flitting eye movements, and mouth or tongue movements. These disorders may be distinguished from tics, as tics tend to repeat the same set of movements. In addition, the child often describes a "build-up" in the need to make the tic, with a sense of release afterwards. There is no such sense of release following chorea; the movements are continually changing and flowing from one body part to another.

Examination

Mild chorea may be difficult to distinguish from normal restlessness. Therefore, it is important to assess whether the movements are controllable by the child and whether these movements are sustained or occur only in the doctor's office. It is important to note which parts of the body are involved. The speed and size of the movements determine whether this is most appropriately called chorea, athetosis, choreoathetosis, or ballism. For practical purposes, the distinction is often difficult to make; this distinction is not usually helpful in diagnosis and treatment.

The effect of purposeful movements, such as reaching, speaking, or walking, must be assessed. Dystonia may cause excessive and apparently random movements. These movements are usually worsened with intentional, attempted movements and improve when the child is at rest. There is no image of restlessness, although, in some cases of chorea, the symptoms may appear to be similar. Ataxia may lead to an abnormal gait, with extra movements needed to compensate; however, the movements disappear when the child is steadied or seated.

The history of other affected family members is critically important.

The timing of the chorea is important. There are paroxysmal types that only occur with sudden movement, exercise, or when the child is under stress. The age of onset is also important. In particular, mild chorea may be normal during the first few years of life, as long as it is not worsening over time. It is important to determine whether there are particular foods that trigger the symptoms, or whether there are clues to a metabolic disorder. These clues include intolerance of certain foods or episodes of unexplained vomiting and lethargy.

Genetics of Chorea
There are several genetic causes of chorea. The history of other affected family members is critically important to determining the need for special testing. Since chorea may be caused by exposure to medications or toxins, it is also important to determine whether any such exposure is possible. One more common cause of chorea is Sydenham's chorea, which often follows a streptococcal infection. For this reason, a history of sore throat or flu symptoms that preceded the onset of chorea is important. This history is not necessarily diagnostic as sore throats, flu symptoms, and even positive throat swabs for strep are common in children.

Mechanism

Chorea is thought to be caused by damage to the basal ganglia. As described in the section on anatomy and physiology, the basal ganglia can be divided into two fundamental pathways: the direct and the indirect pathways. In some cases, chorea seems to result from damage to the indirect pathway.

Chorea is thought to be caused by damage to the basal ganglia.

For example, chorea or ballism may be caused by injury to the subthalamic nucleus. Huntington's chorea is caused by selective loss of the indirect pathway cells in the striatum. Current theories suggest that the indirect pathway helps to inhibit unwanted movements, so it is possible that damage to this pathway allows the "escape" of movements that would ordinarily be suppressed. The random initiation of movements suggests that the indirect pathway serves not only to suppress unwanted movements but also to prevent the initiation of movement. It is not known why some children with diffuse injury to the basal ganglia (such as occurs in cerebral palsy) develop choreoathetosis, while others develop dystonia.

Etiology

Static (fixed) injury:
Cerebral palsy (often with delayed onset), encephalitis/post-encephalitis, trauma, tumors, kernicterus

Hereditary/Degenerative:
Ataxia-telangiectasia (AT), Fahr's disease, neurodegeneration with brain iron accumulation type I (NBIA-I, formerly called "Hallervorden-Spatz disease"), Huntington's disease (the dystonic-parkinsonian Westphal variant is more common in children), Rett syndrome, neuroacanthocytosis, HARP syndrome (hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration), benign hereditary chorea

Chemical/Metabolic:
Acyl-CoA dehydrogenase deficiency, mitochondrial disorders (e.g., Leigh's syndrome, etc.), Wilson's disease, GM1 gangliosidosis, metachromatic leukodystrophy, Lesch-Nyhan disease, Niemann-Pick disease type C, methylmalonic aciduria, nonketotic hyperglycinemia, Pelizaeus-Merzbacher disease, vitamin E deficiency or malabsorption (Bassen-Kornzweig disease), hypoparathyroidism, hyperthyroidism, propionic acidemia, hypernatremia, hypomagnesemia, hypocalcemia, hypoglycemia, hyperglycemia, post-cardiac bypass

Immune-mediated:
Lupus erythematosis, Henoch-Schonlein purpura, anticardiolipin or antiphospholipid antibody syndrome, Sydenham's chorea (associated with previous [one to six months] streptococcal infection), chorea gravidarum (during or immediately after pregnancy)

Vascular:
Basal ganglia stroke, cerebral vasculitis, Moya-Moya disease, complex migraine, alternating hemiplegia

Malformations:
Holoprosencephaly, Joubert syndrome, agenesis of the corpus callosum

Drug-induced:
Neuroleptic medications including anti-emetics (e.g., haloperidol, thorazine, clomipramine, pimozide, pemoline, compazine, metoclopramide, etc.), calcium channel blockers (e.g., flunarizine, cinarizine, etc.), amphetamines (e.g., ritalin and dexedrine, etc.), anti-seizure medications (e.g., phenytoin, carbamazepine, valproate, phenobarbital, etc.), anti-cholinergic medications (e.g., trihexphenydil, benztropine, etc.), anti-histamines, tricyclic antidepressants, benzodiazepines, stimulants (e.g., bronchodilators), clonidine, L-dopa, amantadine, cocaine, bismuth, lithium, manganese toxicity, ethanol, carbon monoxide, oral contraceptives, general anesthesia

Acute/Paroxysmal:
PKC: paroxysmal kinesogenic choreoathetosis (triggered by movement and responds to treatment with carbamazepine), PNKC: paroxysmal nonkinesogenic choreoathetosis (also known as paroxysmal dystonic choreoathetosis [PDC or Mount & Reback disease], often worsened by stress, caffeine, or alcohol, improved by clonazepam or acetazolamide, localized to a gene near 2q33-q35, also called DYT8), PEC: paroxysmal exercise-induced choreoathetosis (occurs with fatigue, rather than at the onset of movement).

Disorders that mimic chorea:
Spasmus nutans, tics, dystonia, shaking/shuddering spells, proprioceptive loss (e.g., spinal cord injury, peripheral neuropathy, etc.), self-stimulation, psychogenic, normal development (less than 1 year of age)

Workup

If the chorea is a new or acute symptom, it may be helpful to determine whether there has been a prior streptococcal infection (such as "strep throat"). It is known that Sydenham's chorea often follows such an infection. The importance of determining this is due to the fact that untreated streptococcal infections may lead to rheumatic fever. Up to 63% of children with Sydenham's chorea develop heart abnormalities. Sore throats and flu symptoms are very common and laboratory testing for streptococcus is not always reliable. Therefore, it may be difficult to determine if a previous streptococcal infection is the exact cause of a child's symptoms.

Laboratory tests may include a throat culture and streptococcal blood antigen test (ASLO), electrolytes (i.e., sodium, potassium, chloride, bicarbonate), magnesium, calcium, amino and organic acid studies, thyroid function, glucose, ammonia, antinuclear antigen (ANA), antiphospholipid antibodies (APLA), and a complete blood count (CBC). An additional test may be performed for the presence of acanthocytes.

In some cases, an MRI of the head may be important in order to look for structural abnormalities, such as those related to a tumor, stroke, metabolic or degenerative disorders, or a remote injury due to low oxygen.

Treatment

Chorea may be difficult to treat. If the child is taking any medications that can cause or worsen chorea, these should be tapered and discontinued, if possible. The mainstay of treatment in adults is the class of neuroleptics, including haloperidol and pimozide.

According to reports, certain anti-epileptic medications may help to improve the symptoms of chorea.

These drugs selectively enhance the function of the indirect pathway by blocking the inhibitory effect of dopamine on this pathway. However, the incidence of side effects in children from neuroleptics has been reported to be as high as 20%. Therefore, it is often safer to start with an alternative medication, such as a benzodiazepine, particularly clonazepam, diazepam, or clobazam. According to reports, certain anti-epileptic medications, such as valproate and piracetam, may improve the symptoms of chorea. In particular, valproate may be very helpful in children with Sydenham's chorea.

Last Update: January 3, 2002

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.