Tourette Syndrome and Child Development
Tourette Syndrome and Child Development: School-Based Treatment
Special to ADVANCE
Some people still think of it as "The Cursing Disease," but Tourette Syndrome (TS) is actually a complex and baffling disorder that can create havoc with a child's development.
Over 70 percent of children and adolescents diagnosed with the condition may require special education services. These students are clearly a high-risk population for developing special education needs, yet only 25 percent of them are even identified and referred to the committees on special education in their school district for evaluation.
To Date, there has been no research addressing interventions for the motoric and organizational deficits that are often observed in this population. Recent research suggests that most cases of TS are genetically transmitted.
TS is a neurobiological disorder characterized by involuntary movements and sounds. While their exact neurological basis is not clear, these tics are thought to be caused by dysfunction in the dopamine system.
Tourette's is one of a number of tic disorders. Before a physician can make the differential diagnosis of TS, the following criteria from the Fourth Diagnostic Statistical Manual (DSM-IV) must be met:
* Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently.
* The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than one year. During this period, there is never a tic-free period of more than three consecutive months.
* The disturbance causes marked distress or significant impairment in social, occupational or other important areas of functioning.
* The onset is before age 18.
* The disturbance is not due to the direct physiological effects of a substance (e.g., stimulants) or a general medical condition (e.g., Huntington's disease or postviral encephalitis).
There Are No neurological or hematological tests that can confirm the diagnosis of TS. Diagnosis is made on the basis of the physician's observation of the patient and/or on the reported history by a reliable observer.
While TS was once thought to be extremely rare, there is now growing evidence that it may be a fairly common pediatric-onset disorder that
goes undiagnosed or misdiagnosed. Tourette's appears to be more prevalent in children than in adults and may be present in approximately 1.6 percent of a pediatric population.
The symptoms generally emerge between the ages of 5 and 10 years, with a mean age of onset of 7 years. In more than 60 percent of clinic patients, the first tics are usually simple motor tics of the head, neck or face, such as eye blinking or rolling, squinting, shoulder shrugging, facial grimacing and tossing hair out of eyes.
Approximately 13 percent of clinic patients report simple phonic tics such as throat-clearing sounds, grunting, sniffing, spitting and coughing as their first symptoms. Almost every clinical study on TS has found that the disorder is more prevalent in males than females. The ratio in adults is approximately 3:1; in children, 9:1.
The first tic usually appears suddenly, lasts a few weeks and then disappears, only to be replaced a few weeks or months later by a different tic. Apart from the variability in the anatomic location of the tic(s), there is variability in the frequency and severity.
This inherent variability is referred to as the "waxing and waning" of TS.
Early tics usually are misdiagnosed, thereby delaying referral to pediatric neurologists. For example, children with eye-blinking tics often are referred for vision examinations, while children with sniffing or coughing tics are referred to pediatricians or allergists for treatment of colds or suspected allergies. It often takes five years from the onset of symptoms before a child finally is diagnosed correctly.
As the symptoms of TS emerge, simple motor tics of the head and neck region may be replaced by more complex tics of the extremities and trunk. These movements involve the coordinated, but involuntary, sequence or activation of two or more muscle groups.
Some common complex tics include touching people or objects, smelling fingers or objects, jumping or skipping, poking or jabbing, punching, kicking, hopping, flapping arms, twirling around, tensing muscle groups, thrusting movements of the groin or torso, twirling hair and imitating the movements of others.
Complex phonic tics are linguistically meaningful but involuntary utterances. These may include animal-like sounds, unusual changes in pitch or volume of the voice, stuttering and echo phenomena such as palilalia or echolalia. In a child who is diagnosed as having TS, almost any unusual and repetitive movement or utterance may be a complex tic.
Complex tics frequently are misinterpreted as voluntary misbehavior. It is not uncommon for school districts to misclassify children with TS as emotionally disturbed.
If Behaviors are complex tics, these children are appropriately classified as "other health impaired" under the Individuals with Disabilities Education Act (IDEA). Because tic disorders and TS are not well-known to most educators, health care professionals evaluating a referred child should be particularly alert to the presence of tics. If they are observed or suspected, further neurological study may be needed.
Although the symptoms of coprolalia (involuntary obscenities) or copropraxia (involuntary obscene gestures) have attracted the most media interest, they are generally misunderstood by school personnel. Practitioners working with children who exhibit these symptoms need to remember that the utterances or movements do not reflect the child's true thoughts or feelings. Less than 30 percent of all patients with TS will develop either of these symptoms.
Even if the symptoms do appear, they are unlikely to persist for long. As with all tics, they will come and go. Practitioners should communicate to the child or adolescent that they understand the involuntary nature of the symptoms, thereby validating the child's experience of being out of control of his or her body and helping to foster a positive therapeutic relationship.
One of the greatest sources of frustration and unhappiness for children and adolescents with TS is that their peers and significant adults in their life don't understand what it feels like to live in a body that does unpredictable and undesirable things. Children with TS frequently are frustrated that tasks that should be easy for them require tremendous effort, yet their almost heroic struggle often goes unrecognized.
Most People with TS can suppress tics for varying amounts of time, from a few seconds to hours. Additionally, many individuals automatically, or unconsciously, suppress tics when they are in the presence of authority figures such as teachers or doctors. This ability to suppress the symptoms often misleads school personnel into believing that the symptoms are voluntary.
Suppressing tics is generally undesirable because it may lead to a more explosive release of them later. People who suppress tics throughout the day often come home and appear to explode in tics until they can get them out of their system.
The severity or frequency of tics may vary across settings or activities within school. Children generally are less likely to tic in the classroom than they are in one-on-one situations. For that reason, health care professionals conducting individual treatment sessions are more likely to observe tics than the classroom teacher.
In the natural progression of the disorder, symptom severity tends to increase over the first decade from onset. The severity or frequency of the tics then will diminish significantly in the late teen years for about 70 percent of clinic patients with TS.
For others, however, tic symptoms may become more complex or unpredictable. In fact, coprolalia and copropraxia typically emerge between the ages of 10 and 13.
Stress Is the single most potent factor that exacerbates the symptoms of TS. Some stressors that have been identified in clinical literature include time pressure, environments or programs that do not provide adequate opportunity to discharge symptoms or dissipate excessive motor activity, anticipation of exciting events, starting school in the fall, environmental factors such as allergy seasons or hot weather, settings such as the library or church where children are expected to sit quietly, and fatigue.
These observations are based on controlled research, but it is important to keep in mind that any child may or may not display these patterns.
Furthermore, while tics tend to worsen under conditions of stress or fatigue, relaxation does not necessarily decrease tics. Children observed relaxing and watching TV often display a significant number of tics. One possible explanation is that there is a decreased effort to suppress tics as the child relaxes. Many patients report that symptom severity abates under conditions where they are nonanxiously engrossed or constructively engaged in a novel or challenging task. That may be of particular relevance for health care professionals in selecting treatment activities for the child with TS.
In clinic samples, approximately 40 percent to 50 percent of young patients who have TS also have obsessive-compulsive disorder (OCD). The symptoms of OCD tend to emerge after the tics.
In young children the most frequent compulsive-type behaviors relate to "evening up," such as tugging on one ear and then tugging on the other; symmetry; touching; arranging things in a particular order; and smelling things.
Other Common compulsive behaviors that may be exhibited by children with TS are similar to those observed in children who have OCD but no tics: checking or re-checking a fixed number of times, counting and rituals involving hygiene. Children with either disorder also may engage in repetitive questioning or reassurance-seeking.
The second most commonly observed comorbid disorder is attention deficit disorder with hyperactivity (ADHD). The symptoms of ADHD may precede the emergence of tics by two to three years. Children who have ADHD may appear inattentive, impulsive, distractible and hyperactive. These children tend to have significant difficulty in a regular classroom and in peer relationships.
Children with TS may be more likely than their peers to experience problems from oppositional defiant disorder; depression; anxiety disorders, including phobias and panic attacks; explosive outbursts of aggressive behavior; and self-injurious behaviors such as hitting and biting. Children with TS also appear to be more likely to have learning disabilities.
Because tics are of neurologic origin, the first line of treatment usually will be pharmacological. The most commonly prescribed medications for tic management are clonidine, neuroleptics such as haloperidol, pimozide and fluphenazine hydrochloride. There are a variety of possible side effects associated with neuroleptic use of these agents.
Side Effects may include extrapyramidal or Parkinsonian-type symptoms such as slowed movements, tremor or muscle rigidity. Other motoric side effects may be persistent involuntary movements, or dyskinesias; impaired coordination, including handwriting; akinesia, the partial or complete loss of movement; akathisia, or motor restlessness; and acute dystonic reactions.
Visual disturbances such as blurred vision or decreased visual accommodation also may occur. Neuroleptics may produce behavioral and mood changes including drowsiness, lethargy or sedation, cognitive dulling, weepiness, irritability, dysphoria and/or depression, school avoidance or school phobia, and increased aggressivity.
For Children who have ADHD in the presence of TS, physicians may prescribe the anti-hypertensive medication clonidine. Clonidine may have beneficial effects on the ADHD and tics. Some children who have TS-ADHD experience a worsening of tics when placed on stimulant medications such as dextroamphetamine sulfate or methylphenidate hydrochloride (Ritalin). The most commonly observed side effects of clonidine are drowsiness, lethargy and sedation.
A number of anti-depressants may be prescribed for children with obsessive-compulsive symptoms or full-blown OCD. They include fluoxetine (Prozac®), sertraline (Zoloft®), and paroxetine (Paxil®).
Clomipramine hydrochloride (Anafranil®) frequently is prescribed for OCD. Anafranil can produce tremor and sweating, among other possible side effects. Health care professionals may need to obtain information from the child's treating physician on side effect profiles.
Although TS is not correlated with intellectual potential, children with Tourette's may be more likely to have deficits in the areas of fine motor skills, handwriting and visual-motor integration. Studies have indicated that in addition to motor impairment, these children may display deficits in academic areas such as written mathematics, passage comprehension, spelling and written expressive language.
Many Children with TS-ADHD also have significantly impaired organizational skills and might be described as "terminally disorganized" in terms of time, materials and space management. They may lose track of papers, assignments and possessions and experience significant difficulty in completing projects.
Health care professionals evaluating or working with younger children with TS may find impairments in fine motor speed and dexterity. Children with these problems may have difficulty manipulating objects, using scissors and tying shoelaces. Many parents report significant difficulties in activities of daily living such as using eating utensils and dressing.
In children and adolescents, dysgraphia is the most commonly observed problem. Letters may be poorly formed, there may be inconsistency in size of the letters, and there may be a lack of fluidity to the writing, secondary to increased upper extremity tension. Some children appear to use excessive pressure on the writing instrument, and a number of them may employ an improper pencil or pen grip.
Many children leave almost no space between words and/or run the line off the edge of the page. They may start out at the left margin and then shift the margin successively to the right over the course of the page. Other children appear to totally disregard the ruled horizontal lines as they write.
Because children with TS often have low frustration tolerance, practitioners should make every effort to pace their sessions appropriately and to introduce enjoyable tasks.
Slow handwriting speed—observed in a significant percentage of children with TS—may reflect interference from several sources in addition to impairment in fine motor speed or dexterity. Also, a child who has OCD or obsessive-compulsive symptoms may have compulsive rituals that interfere with writing, such as reworking his or her writing until it looks "just right" or perseverating on a particular letter. Imagine if you had to mentally count how many times the letter "e" occurred as you were writing a sentence or paragraph.
In addition, there may be independent visual processing deficits, such as decreased ocular motility, that reduce graphomotor speed.
Some of the problems that can be addressed are improper pencil or pen grip, increased muscle tone and visual-spatial organizational deficits.
Other Problems, like the compulsive reworking of letters or counting rituals, are unlikely to be successfully remediated through therapy alone. Health care professionals will find it helpful to consult with the child's treating psychologist or physician, as well as the school psychologist.
Children whose obsessive-compulsive writing rituals are so severe as to preclude functional handwriting may require alternative means of expressions, such as tape recording, dictating to a scribe, or word processing.
Dysgraphia and poor visual-motor integration probably both contribute to the common observation that children with TS tend to have problems with written arithmetic, but not necessarily mental math or applied problems. Teachers may be advised to have the child turn lined paper sideways so that the lines serve as column guides.
Health care professionals also may encourage teachers to allow the child a larger workspace for performing their computations. Enlarging the ditto or work page by using the enlargement feature on the office copier will provide extra space for the student who needs it.
The child also may require a reduced amount of copying from the board or text to paper. Many of these children also will need testing accommodations that permit them to record their answers directly in test booklets.
For The Child with TS and ADD, practitioners should consider providing services in the classroom. Children who have ADD typically have a problem with carryover of skills. If health care professionals cannot provide some services in the classroom, they should allocate time to serve as an ongoing consultant to classroom teachers.
The practitioner's advice with respect to the following questions can make a significant difference to the child:
* How much handwritten work should the child be expected to do at one time?
* Should the teacher grade for neatness or ignore it?
* Should the teacher reduce copying activities from the board for the child with TS?
* Does the child need assistive technology to complete his or her assignments?
Many children with TS require word processors in the classroom and at home to enable them to produce written work that is commensurate with their knowledge and potential. Those who are academically on grade level but need a computer as assistive technology because of their disability qualify to receive assistance under Section 504 of the Rehabilitation Act and under the Americans with Disabilities Act (ADA), even if they do not qualify for an individualized education plan (IEP) under the Individuals with Disabilities Education Act (IDEA).
If the child is classified under IDEA, modifications, accommodations and assistive technology become part of the IEP. In both situations the child's parents would meet with members of the special education committee or 504 Committee to discuss the matter and come to a joint decision.
* About the authors: Dr. Packer is a psychologist who specializes in motor retraining of adults with motor control dysfunction due to neurological trauma or disease. Ms. Gentile worked with children in a specialized TS program at the Captree Learning Center in West Islip, Long Island. The authors acknowledge the assistance of Rosa A. Hagin, PhD, who has published several articles on school-based problems in children with TS. Some of her articles are available from the Tourette Syndrome Association (TSA). For more information, contact Dr. Packer at 940 Lincoln Pl., North Bellmore, NY 11710-1016 or TSA at 800-237-0717.