Tourette syndrome: a new look at an old condition. (Tourette Syndrome).

During the past 15-20 years there has been a great deal written about Tourette syndrome (TS). By far the greatest amount of literature relates to the medical aspects of the condition. Establishment of the Tourette Syndrome Association (TSA) in 1972 has influenced the growth and areas of the literature. TSA has funded (and continues to fund) a considerable amount of research related to the causes and treatment of TS. Probably the second greatest amount of literature regarding TS relates to accommodating young children in school and family environments.

Review of the literature

An extensive review of the literature has revealed almost nothing about rehabilitation services for people who have TS. Symptoms manifested by some children will be very mild and will require few, if any, accommodations or rehabilitation services. Other children diagnosed with this condition will outgrow it or see a substantial reduction in their symptoms by early adulthood (Bernstein, 1997). However, approximately one third of the children who have been diagnosed with TS will have and continue to have major symptoms for the remainder of their lives. (Murray, 1997) There are also some people who experience an adult onset of TS (Chouinard & Ford, 2000; Marneros, 1983). Those who provide vocational rehabilitation services can reasonably anticipate that many of those adults who have symptoms of TS might seek vocational rehabilitation services to help them find and secure their place in the work force. They may also need help in achieving independent living.

Description of TS

Tourette's syndrome is described in much of the literature as an inherited tic disorder. (Merck, 2001; Murray, 1997; National Institute of Neurological Disorders and Stroke [NINDS], 2000; Willis, 1993). However, other recent literature indicates that only 50 to 70% of the diagnosed cases are hereditary (Meyers, 1998). Research suggests the remainder of the cases, acquired Tourette's syndrome (or Tourettism), might be related to several things including environmental, infectious, and psychosocial factors (Zinner, 2000). Stell, Thickbroom, and Mastaglia (1995, p.729) relate Tourettism to "inflammatory, toxic, metabolic, and structural lesions of the central gray matter of the brainstem." Meyers (1998) reports "a growing number of children have been identified with stimulant induced TS. That is, stimulant medications commonly prescribed to hyperactive children (e.g., Ritalin, Cylert, Dexedrine, etc.) can sometimes precipitate TS in predisposed children, according to the Food and Drug Administration."(p.4) This condition is neither degenerative nor terminal (Meyers, 1998).

Diagnosis of TS

To be diagnosed with TS, a person must present with both motor tics and vocal (phonic) tics. The motor and vocal tics do not necessarily occur concurrently. Table 1 lists some of the tics commonly associated with TS. The person might have simple and/or complex tics. The table provides examples of both simple and complex tics. For a diagnosis of TS to be made, the tics must have occurred for at least 1 year with no "tic-free" period greater than 3 months (American Psychiatric Association, 1994). Symptoms usually occur before the age of 18, although there are reported cases of adult onset of TS. (Chouinard & Ford, 2000; Marneros, 1983).

Tourette's syndrome symptoms fall along a continuum from barely noticeable to blatantly noticeable and debilitating. Frequency of tics may range from those who tic only when they are anxious or fatigued to those who might tic as much as 30 to 100 times per minute (Murray, 1997). For many years clinical interventions and research focused almost exclusively on people with the most severe cases of TS. This focus may have created a distorted perception of the TS population, i.e., TS was thought to be a very rare condition that a practicing physician might see only once throughout his or her practice of medicine (Zinner, 2000). However, It is now recognized that most cases of TS are in the mild to moderate category. Many people affected by TS may never seek medical intervention (Peterson, 1996).

Comorbid conditions

A number of conditions commonly occur in association with TS. These are referred to as comorbid conditions. Common comorbid conditions include Attention Deficit Hyperactivity disorder (ADHD), Obsessive Compulsive disorder (OCD), anxiety and phobia disorders, and learning disabilities. Less frequent occurring comorbid conditions include mood disorders, bipolar disorders, oppositional defiant disorder, self-injurious behaviors, bedwetting, conduct disorders, sleep disorders, speech and language disorders, executive function difficulties, personality disorders, lack of impulse control and rage attacks (Bagheri, Kerbeshian, & Burd, 1999a; Bruun, Rickler, & Kelman-Bravo, 1999; Zinner, 2000). The cumulative effect of TS and even one or two comorbid conditions can have a tremendous impact upon the academic, employment and social functioning of an individual. Vocational rehabilitation personnel should recognize that TS involves a complexity of behavioral disorders. Even though a person's TS may be labeled mild, it could represent an obstacle to success in the academic and work environments. The cumulative effect of several mild conditions may be greater than the sum of the parts e.g., mild dyslexia, mild attention deficit, mild hyperactivity, and mild phonic and motor tics might create more than a mild disruption of the person's ability to achieve his or her maximum potential. Rehabilitation personnel and the consumer must work together to determine which symptoms most need to be attenuated or accommodated.

Evaluation implications

It is possible that the Rehabilitation Counselors and Vocational Evaluators serving the person that has TS might never see the person tic. Several factors may account for the absence of tics in the presence of the counselor or evaluator. Tics can wax and wane and may be absent for periods of up to 3 months. A person's tics may occur only in bouts. Those bouts may occur outside the hours during which the person has contact with the counselor or evaluator. Moreover, the person may suppress or disguise the tics when in the presence of others. Rehabilitation Counselors and Vocational Evaluators must be careful to not minimize the functional limitations associated with TS just because they have not seen the consumer have tics.

TS is a neurological condition that is often unrecognized, misdiagnosed, misunderstood, and mistreated (Kushner, 1999). The person being evaluated may have been identified as having some disability other than TS. The identified disability may well be a condition commonly found in association with TS but not the primary cause of disability. Tourette's syndrome may simply have been overlooked or misdiagnosed. TS has been misdiagnosed as asthma (Hogan & Wilson, 1999), acute anxiety, a gynecologic disorder (Templeman & Hertweck, 2000), vision problems, schizophrenia, and a variety of other conditions (Kushner, 1999). The person seeking rehabilitation services may never have heard of TS. However, if the evaluator suspects the person being evaluated might have TS, the evaluator should question previous evaluation reports, diagnostic information, or both in the consumer's files. If it is suspected a consumer may have undiagnosed or misdiagnosed TS, the evaluator should consider discussing the issue with a medical consultant and the consumer.

Results from evaluation instruments might provide a reason to suspect Tourette's syndrome as a disability. Cohen, Bruun and Leckman (1988) reported that most investigators have documented a more frequent occurrence of "significant disparity" between verbal and performance IQ scores of TS patients when compared to control populations.

Problems with visual-motor integration are frequent among people who are diagnosed with TS (Bronheim, 1991; Willis, 1993). Willis (1993) indicated that administration of the Bender-Gestalt test to people with TS often reveals impaired performance. Willis also indicated that, when performing tests of general intelligence, people with TS often have their lowest scaled scores on sub-tests like the block design and object assembly that measure visuospatial organization.

Evaluators should be alert to the possibility that a person being evaluated might have an undiagnosed case of TS. Is there evidence of any of the common comorbid conditions? If so, the evaluator should ask about tics. Is there any history of tics? Does any other family member have tics or a diagnosis of TS, OCD, or ADHD? Affirmative answers to these questions would correlate highly with a diagnosis of TS (Wand, Matazow, Shady, Furer, & Staley 1993; Willis, 1993).

The course of TS is impossible to predict. About a third of those people diagnosed with TS in their childhood will show few, if any, symptoms as adults; another a third will experience a substantial reduction in symptoms as adults; and the remaining third will continue to exhibit symptoms throughout their adult years (Murray, 1997). There is no way to predict who will outgrow their symptoms, who will stabilize, and who will get worse. Therefore, rehabilitation plans should be developed with the assumption that the person will continue to experience the full effect of TS throughout the adult years.

Considerations with TS

Wand et al. (1993) surveyed 177 people over the age of 18 who had TS. They asked them to indicate the extent to which they experienced any of 13 conditions. The results are presented in Table 2.

Although the information contained in Table 2 is not an exhaustive list of problems associated with TS, the table does reflect the complexity of problems that evaluators should consider when trying to determine the potential and the functional limitations of a person who has TS.

Several other conditions often associated with TS might influence the employability of those who have TS and should be considered during the process of vocational evaluation. Some of those conditions are (a) a lack of inhibition control, (b) speech problems (palalalia, coprolalia, echolalia and stuttering), (c) copropraxia (use of obscene gestures), (d) echopraxia (imitating the behaviors of others), (e) poor handwriting (Davidovicz, 1994; Meyers, 1998), and (f) limitations associated with any comorbid conditions the person may have.

Medical considerations

A pharmacological intervention may not always be appropriate. Medications currently used to attenuate the symptoms of TS are drugs approved for the treatment of other conditions. Some of the medications prescribed to decrease the frequency and intensity of the tics may cause the person to gain weight, be lethargic, sleepy, cognitively blunted or even show signs of Parkinsonism. Many people who have mild to moderate cases of TS choose to tic rather than experience the side effects of the medication (Mikkelson, Detlor, & Cohen, 1981). Medications prescribed for a comorbid condition may aggravate the Tourette's syndrome. For example, administering Ritalin to a child who has TS may make the tics much worse (Knoblauch, 1998). The use of nitrous oxide (laughing gas) in association with dental care might cause a temporary exacerbation of the tics (Friedlander & Cummings, 1992).

If a consumer with TS is taking medication, the rehabilitation personnel involved with that person should know why the person is taking the medication. Some questions to ask are: (a) Is the medication intended to reduce or eliminate tics? (b) Is it being prescribed at the request of another person, e.g., a parent? (c) Is it being administered to control something that might be controlled by another means, e.g., behavioral intervention, work site modification, etc.? and (d) Might the medication be compounding the problems of TS rather than alleviating them (side effects)? The answers to these questions might prove important in helping the consumer and the rehabilitation counselor design a rehabilitation program that would best meet the consumer's needs.

It is very important that health care personnel working with the person who has TS are well trained with current knowledge regarding TS. Psychiatrists may be quick to medicate the problem, neurologists may not be up to date on movement disorders and the appropriate interventions. Counselors and psychologists that serve people who have TS should be experienced with cognitive behavioral therapies (TS - now what?, 2000).

Medication may negatively affect the person's performance on evaluation activities. Some medications prescribed for TS (e.g., haloperidol) may cause cognitive blunting, lethargy, and parkinsonism. Medications have also been linked to "work phobia" (Mikkelson et al., 1981). Because the symptoms wax and wane, it is difficult to get a level of medication that will control the symptoms at an acceptable level.

Tics might be aggravated by the use of medications taken for other purposes. Stimulants and products containing stimulants, (e.g., caffeinated beverages, cough syrup, diet medication, etc.), psychoactive drugs and recreational drugs (e.g., cocaine) may worsen the tics (Bagheri et al., 1999a).

Counseling considerations when working with people who have TS

Counseling interventions may be beneficial to people with TS for various reasons. Focal issues in a counseling relationship with a person who has TS might include managing (a) depression, (b) feelings of being different from others, (c) fear of having tics at awkward times, and (d) a variety of other adjustment issues (Murray, 1997). A counselor might help the person with TS learn effective stress management techniques. Reducing stress often reduces the incidence of tics. Other goals of a counseling relationship might include helping the person be more sensitive to premonitory sensations for improved tic management (Robertson, 2000), learning behavior management techniques (e.g., habit reversal; Oliver & deLange, 1999), developing a positive self-concept, and working with family dynamics (Meyers, 1998). Of course, there might also be issues not related to the Tourette syndrome that the counselee would like to examine within the counseling relationship.

Job placement implications

Meyers (1999) cites a study conducted by the University of Cincinnati in 1982 which involved a questionnaire mailed to all people in Ohio diagnosed with TS and known to the Tourette Syndrome Association in Ohio. One hundred-fourteen of the respondents were over the age of 19 and were not in school. Of those 114 respondents, 36% had full-time employment, 14% had part-time employment, and 48% were unemployed. That rate of unemployment was almost four times the rate of unemployment for the general population of Ohio at the time of the study. Many of the respondents were considered to be under-employed at the time of the study, i.e., college graduates working in manual labor or blue collar situations. While acknowledging that this study is somewhat dated, this author has been unable to find any more current studies that might suggest any favorable change in employment data for people who have TS. Only 12% of the 114 respondents reported receiving any vocational rehabilitation services. Of those who received vocational rehabilitation services, only half indicated that they thought the services were beneficial.

Perhaps short-term training programs designed for vocational rehabilitation personnel to help them gain more information about Tourette syndrome would help the counselors and evaluators have a better understanding of the employment and social needs of people who have TS. Such training might help rehabilitation personnel improve the employment rate of people who have TS and their level of satisfaction with vocational rehabilitation services. Training might help rehabilitation personnel learn more about the TS and accommodations that could make appropriate and sustained employment a reality for people who have TS.

Suggestions for accommodations

It may be necessary, or at least beneficial, to provide an acoustically insulated work site for the person who has TS. This arrangement would benefit both the person with TS and his or her coworkers. Tic-related noises and the likelihood they would bother coworkers would be lessened and the person with TS would have a quieter work environment (Meyers, 1998). It has been estimated that as many as 20% of people who have TS have an exaggerated or hyperactive startle response (Sachdev, Chee, & Aniss, 1997; Stell et al., 1995). When doing job development with a person that has a hyperactive startle response, rehabilitation counselors should try to create a work environment that will be free of sudden loud noises (e.g., slamming doors, buzzers, loud speakers, loud telephone bells, etc.). Such noises might precipitate a bout of tics and detract from work performance.

An alternate form of accommodation for phonic tics might be to employ the person in a work setting that is routinely noisy (Meyers, 1998). In such a setting the person's "noises" (phonic tics) might be less likely to bother coworkers. Both motor and vocal tics might be accommodated in employment settings that would allow frequent escapes from coworkers during which time the tics could be released, e.g., truck driver, letter carrier, messenger service, etc. (Bernstein, 1997). Some people with TS may simply need an opportunity or a place to release tics as needed (Meyers, 1998). This accommodation might involve allowing the person to switch occasionally from mental to physical tasks or to briefly escape to a place where tics can be discharged (e.g., a break room, a restroom, etc.).

Stress and physical exhaustion will increase the likelihood of tics occurring (Meyers, 1998). An appropriate accommodation would be to help the individual find employment that involves relatively low levels of stress and is not routinely exhausting. The person who has TS might benefit from being taught effective stress management techniques.

Tics may be worse at certain times of the day or with certain tasks. They often come in bouts. The absence of noticeable tics during working hours (or during evaluation) cannot be interpreted to mean that the TS is not affecting the individual's work performance. The person might be exerting great energy to suppress the tics and to contain the effects of obsessive thoughts and compulsive behaviors.

When contemplating job placement with a person with TS or developing an individualized plan for employment, rehabilitation counselors should remember that there may be some benefits of TS. Oliver Sacks (1992), a noted neurologist, suggests that people who have TS may have a number of qualities that could be an asset to them in an appropriate employment setting. He cites qualities like inventiveness, a vivid imagination, a good memory, and creativity. Sacks cites examples of athletes, writers, creative artists and performing artists who have TS and who believe their TS has contributed to their success in their respective professional activities. Sacks suggests some people that have TS may manage their symptoms by deferring some (e.g., tics, coprolalia, copropraxia, etc.) while channeling others (e.g., quick wit, mimicry, creativity, etc.) into a positive part of their job performance. He gives an example of a disc jockey whose language, off the air, is full of profanities but, when the man is on the air, he does not use profanities. Sacks indicates that the disc jockey is able to use to his advantage other TS related qualities like his quick wit, mimicry skills and creative thinking.

An important part of living with TS is social acceptance. To promote social acceptance of the worker that has TS, rehabilitation counselors might provide to employers and coworkers of people who have TS general information regarding TS. Such information might be presented in print (or other appropriate medium) or via formal training. The information might be presented as part of a general overview of several disabilities thus keeping the focus off of any particular individual. A good example of such material is a booklet written by Judy Cohen (1998). The booklet is titled Disability etiquette: Tips on interacting with people with disabilities. The booklet is published by the Eastern Paralyzed Veterans Association (EPVA). One can get a copy of the booklet by telephoning the EPVA at 1-800-444-0120.

It is important for rehabilitation counselors and evaluators to remember that approximately 60% of the people diagnosed with TS also have comorbid conditions. Of course, those conditions must also be considered and accommodated throughout the rehabilitation process.

Conclusion

"Tourette syndrome represents one of the fastest growing diagnoses in North America" (Kushner, 1999, p.4). Increased awareness of TS and the more frequent diagnosis of TS increase the likelihood that rehabilitation personnel will be asked to provide services to people who have TS.

Tourette syndrome is a very complex condition. It often involves comorbid conditions. There is no known cure for TS at this time. However, continuing research is providing a wealth of new information about this condition. It is important for rehabilitation personnel to stay abreast of the research findings so that they can better understand TS and, thus, provide a high quality of service for those people with TS who might seek their help.

Table 1. Representative tics associated with Tourette
syndrome (not an exhaustive list).

SIMPLE               COMPLEX

MOTOR

Arm flapping         Body jerking
Arm jerking          Copropraxia
Clapping             Echopraxia
Eye blinking         Hitting
Eye rolling          Hopping
Facial contortions   Jumping
Foot tapping         Kicking
Grimacing            Kissing
Hair tossing         Pinching
Knee knocking        Scratching
Leg jerking          Skipping
Lip smacking         Smelling things
Nose twitching       Stepping backwards
Shoulder shrugging   Throwing things
Squinting            Twirling in circles
Tongue thrusting     Walking on toes

PHONIC

Belching             Amplitude variations
Clicking             (voice)
Coughing             Animal sounds
Grunting             Coprolalia
Hiccupping           Echolalia
Hissing              "Hello," "Hey," "Wow," etc.
Honking              Hollering
Moaning              Humming
Puffing              Laughing (inappropriately)
Screaming            Palilalia
Shouting             Repeating parts of words
Snorting             Repeating phrases
Squeaking            Spitting
Throat clearing      Stuttering
"Tsk," Pft," etc.    Talking to oneself
Yelping              Whistling
                     Yelling

Source: The educators' in-service program on understanding
Tourette syndrome (2001)
Table 2. Reported frequency of tics and TS associated conditions in
adults with TS (adapted from Wand et.al., 1993).

                                Often   Sometimes   Never

Motor tics                      69.3%     24.5%      6.2%
Vocal tics                      49.2%     42.0%      8.8%
Obsessive/compulsive behavior   31.2%     37.0%     31.7%
Extreme anxiety                 31.2%     41.4%     27.4%
Problems getting to sleep       29.7%     38.5%     31.8%
Hyperactive behavior            23.5%     32.1%     44.4%
Mood swings                     22.8%     41.3%     36.0%
Temper control                  19.0%     32.8%     48.2%
Aggression                      15.0%     33.2%     51.9%
Coprolalia                      19.3%     23.5%     57.2%
Problems staying asleep         12.9%     29.6%     57.5%
Self-abusive behavior            8.1%     25.4%     66.5%
Sleepwalking                     0.0%      8.5%     91.5%

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Glen Hendren, Ph.D., CRC, LPC, Professor and Coordinator of Rehabilitation Programs, Department of Counselor Education and Education Psychology, Mississippi State University, Mail Stop 9727, Mississippi State, MS 39762. Email: [e-mail]