Do not let your happiness depend on something you may lose…only (upon) the Beloved who will never pass away.
C.S. Lewis

Synonymes terminology

Synonymes terminology

Synonyms

Tourette Disorder
Gilles del la Tourette’s Syndrome also known as Gilles de la Tourette’s Disease Chronic Multiple Tics
TS
Habit Spasms
Tics
Maladie de Tics
Passing Tics of Childhood also known as Transient Tics of Childhood Brissaud’s II
Coprolalia-generalized tic
Guinon’s myospasia impulsiva

General Discussion

If you wish to obtain more information about this disorder, then you should contact your personal physician and/or the agencies listed in the “Resources” section of this report.

Tourette Syndrome is a neurological movement disorder which begins in childhood between the ages of 2 and 16. The disease is characterized by involuntary muscular movements, tics, and uncontrollable vocal sounds. Sometimes inappropriate words may occur. Tourette Syndrome is not a degenerative disorder and those affected can expect to live a normal life span.

Symptomatology

Tourette Syndrome is not a progressive disease. Over 3 – 4 month periods some symptoms may disappear only to be replaced by new tics, or new symptoms may be added to old ones.

  1. Tourette Syndrome generally begins with a generalized tic, usually of a facial muscle producing excessive blinking of eyes, excessive twitching of the nose or grimacing.
  2. Inarticulate and incoherent sounds such as grunts, barks or repeated sniffing.
  3. Involuntary head shaking, shoulder jerking, arm flapping, foot stamping, etc.
  4. Patient may use inappropriate or obscene words. This occurs in 30% of all cases.
  5. The involuntary repetition of a word or a sentence spoken by oneself or another person (palilalia or echolalia).
  6. The involuntary imitation of other people’s movements.
  7. Tics are intensified by stress, and may disappear during concentration or when doing particular tasks.
  8. Some patients may have self-mutilating symptoms

A 16% remission rate during puberty has been reported. For most, Tourette Syndrome maintains a chronic, waxing and waning course, always exacerbated under stress.

TRANSIENT TICS OF CHILDHOOD are common among elementary school children. These motor tics usually disappear within one year.

CHRONIC TICS begin in childhood or after age 40. These tics do not wax and wane, and symptoms remain the same permanently.

Etiology

The cause of Tourette Syndrome is unknown. Research suggests that there may be a biochemical dysfunction which affects neurotransmitter systems in the brain. Approximately 70% of Tourette cases appear to be genetic, but the mode of transmission is still unknown. There is a high prevalence of first degree relatives with milder tic conditions in families with Tourette Syndrome, indicating the effect of gene penetrance.

It has been noted that Tourette Syndrome (TS) often runs in families, but relatives do not often appear to have all symptoms of TS. Some relatives may have only chronic tics while others may not display any tics, but may exhibit obsessive-compulsive behaviors. Recent research indicates that these behaviors are related to TS and appear to be milder forms of this disorder.

Geneticists believe that TS is inherited through an autosomal dominant gene. (In autosomal dominant disorders, a single abnormal gene, contributed by either parent, “overrides” the normal gene contributed by the other parent causing disease. Individuals with one affected parent have a 50% chance of inheriting the disorder. Males and females have an equal chance of inheriting the abnormal gene.

A child with a parent who has Tourette Syndrome has a 50% chance of inheriting the TS gene. However, when the gene is inherited, the probability of expression of symptoms depends upon the sex of the child. If a son receives the gene from his parent, there is a 100% chance that he will exhibit some form of the disorder (either TS, chronic tics or obsessive- compulsive behaviors). If a daughter receives the gene, her chance is 70% that she will have some form of the disorder. The data indicate the chance that a TS patient will have an affected son is 50% and the chance that a daughter will be affected is 35%.

In most cases, an affected child will have a very mild case, although at the present time, there is no way to predict the severity of symptoms. Genetic studies suggest that only about 10% of affected relatives have symptoms severe enough for their lives to be significantly affected. While the chance of an affected parent having a child with TS symptoms ranges between 40% and 50%, the chance of having a severely affected child is considerably smaller.

Affected Population

Tics usually begin when the patient is between the ages of 2 and 16, though there are rarely cases with later onset. Males and females have an equal chance of inheriting the disorder. Tourette Syndrome occurs in all nationalities and all economic groups.

Therapies: Standard

DRUGS

Haloperidol (Haldol) – Low doses help suppress the symptoms in many patients

Clonidine (Catapres) – This drug, approved by the FDA for treatment of hypertension, appears to be effective on motor, vocal, and behavioral symptoms. Controlled studies are underway at Yale University and Rush Presbyterian Hospital.

Pimozide (Orap) is a newly approved orphan drug with Dopamine D-2 blocking action. Pimozide is reported to be as effective as Haldol with fewer side effects in the majority of Tourette patients. Other dopamine blocking drugs (i.e., prolixin) are also used to reduce Tourette symptoms.

OTHER – Supportive psychotherapy may be indicated to foster the patient’s adjustment to this chronic, socially crippling disorder.

Therapies: Investigational

Research is ongoing in the areas of neurotransmitters, drugs, and genetics.

The Food and Drug Administration (FDA) has awarded a research grant to Paul Sandor, M.D., FRCPC, Toronto Western Hospital, Toronto, Ontario, Canada, for obsessive-compulsive symptoms of Tourette Syndrome.

For information on additional therapies that have been designated as Orphan Drugs in the last few months, please return to the main menu of NORD Services and access the Orphan Drug Database.

This disease entry is based upon medical information available through October 1987. Since NORD’s resources are limited it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the resources section for the most current information about this disorder.

Resources

For more information on Tourette Syndrome, please contact:

National Organization for Rare Disorders P.O. Box 8923
New Fairfield, CT 06812
(203) 746-6518

NIH/National Institute of Neurological and Communicative Disorders and Stroke 9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751

Tourette Syndrome Association
42-40 Bell Blvd.
Bayside, NY 11361
(718) 224-2999
(800)237-0717
24HJPress <CR> for more !9 
HJCompuServe;73H RDB-203

For information on genetics and genetic counseling referrals, please contact:

March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100

National Center for Education in Maternal and Child Health 38th & R Streets
Washington, DC 20057
(202) 625-8400

For information about Brain Tissue Banks west of the Mississippi River, please contact:
Wallace W. Tourtelotte, M.D.
Director, National Neurological Research Bank Neurology Research (W127A)
Veterans Administration
Wadsworth Medical Center
24HJPress <CR> for more !
HJCompuServe;73H RDB-204

Los Angeles, CA 90073
(213) 824-4307

For information about Brain Tissue Banks east of the Mississippi River, please contact:
Brain Tissue Resource Center
Edward D. Bird, M.D., Director
McLean Hospital
115 Mill Street
Belmont, MA 02178-9983
(617) 855-2400