SYMPTOMATOLOGY


Motor and Phonic Tic Manifestations

The varied symptoms of TS can be divided into motor, vocal, and behavioral manifestations. (Table 1) Simple motor tics are fast, darting, meaningless muscular events. They can be embarrassing or even painful (such as jaw snapping). They are easily distinguished from simple muscular twitches or rapid fasciculation (e.g. of the eyelid or lip). Complex motor tics often are slower, more purposeful in appearance, and more easily described with terms used for deliberate actions (Table 2).

Complex motor tics can be virtually any type of movement that the body can produce, including gyrating, hopping, clapping, tensing arm or neck muscles, touching people or things, and obscene gesturing.

At some point in the continuum of complex motor tics, the term "compulsion" seems appropriate for capturing the organized, ritualistic character of the actions. The need to do and then redo or undo the same action a certain number of times (e.g. to stretch out an arm ten times before writing, to even up, or to stand up and push a chair into "just the right position") is compulsive in quality and accompanied by considerable internal discomfort. Complex motor tics may greatly impair school work (e.g., when a child must stab at a workbook with a pencil or must go over the same letter so many times that the paper is worn thin. Self-destructive behaviors, such as head banging, eye poking, and lip biting, also may occur.

Vocal tics extend over a similar spectrum of complexity and disruption as motor tics
(Table 3). With simple vocal tics, individuals emit linguistically meaningless sounds or noises, such as hissing, coughing, or barking. Complex vocal tics involve linguistically meaningful words, phrases, sentences (e.g., "wow," "Oh boy, now you've said it," "Yup, that's it," "but, but...."). Vocal symptoms may interfere with the smooth flow of speech and resemble a stammer, stutter or other speech irregularities. Often, but not always, vocal symptoms occur at points of linguistic transition, such as at the beginning of a sentence where there may be blocking or difficulties in the initiation of speech, or at phrase transitions. Individuals suddenly may alter speech volume, slur a phrase, emphasize a word, or assume an accent.

The most socially distressing complex vocal symptom is coprolalia, the explosive utterance of foul or "dirty" words or more elaborate sexual and aggressive statements (i.g., racial slurs). Coprolalia is not simply obscene speech spoken in anger or to offend. Rather it is often sudden speech (typically just the first syllable of an inappropriate word) that interrupts an otherwise appropriate flow of words. While coprolalia occurs in only a minority of TS individuals (from 5-30%, depending on the clinical series), it remains the most well known symptom of TS. A diagnosis of TS does not require that coprolalia be present and the majority of individuals do not ever exhibit this symptom.

Some TS individuals may have a tendency to imitate what they have just seen (echopraxia), heard (echolalia), or said (palilalia). For example, the individual may feel an impulse to imitate another's body movements, to speak with an odd inflection, or to accent a syllable just the way it has been pronounced by another person. Such modeling or repetition may lead to the onset of new specific symptoms that will wax and wane in the same way as other TS symptoms. Some individuals also describe "triggers" that almost invariably prompt a tic, e.g., another person coughing in a certain way.

Table 2. Examples of Motor Symptoms

Simple motor tics
Eye blinking, grimacing, nose twitching, lip pouting, shoulder shrugging, arm jerking, head jerking, abdominal tensing, kicking, finger movements, jaw snapping tooth clicking, frowning, tensing parts of the body, and rapid jerking of any part of the body.
Complex motor tics
Hopping, clapping, touching objects (or others or self), throwing, arranging, gyrating, bending, "dystonic" postures, biting the mouth, the lip, or the arm, head banging, arm thrusting, striking out, picking scabs, writhing movements, rolling eyes upwards or side-to-side, making funny expressions, sticking out the tongue, kissing, pinching, writing the same letter or word over-and-over, pulling back on a pencil while writing, and tearing paper or books.

Copropraxia
"Giving the finger" and other obscene gestures.
Echopraxia
Imitating gestures or movements of other people.

Table 3. Examples of Vocal Symptoms

Simple vocal tics
Coughing, spitting, screeching, barking, grunting, gurgling, throat clearing, clacking, whistling, hissing, sucking sounds, and syllable sounds such as "uh, uh," "eee," and "bu."
Complex vocal tics
"Oh boy," "you know," "shut up," "you're fat," "all right," and "what's that."
Rituals
Repeating a phrase until it sounds "just right" and saying something over 3 times.
Speech atypicalities
Unusual rhythms, tone, accents, loudness, and very rapid speech.
Coprolalia
Obscene, aggressive, or otherwise socially unacceptable words or phrases.
Palilalia
Repeating one's own words or parts of words.
Echolalia
Repeating sounds, words, or parts of words of others.

The symptoms of TS can be characterized as mild, moderate, or severe by their frequency, their complexity, and the degree to which they cause impairment or disruption of the individual's ongoing activities and daily life. For example, extremely frequent tics that occur 20-30 times a minute, such as blinking, nodding, or arm flexion, may be less disruptive than an infrequent tic that occurs several times an hour, such as loud barking, coprolalic utterances, or touching tics. The premonitory sensory urges tend to be present by 9 to 10 years of age. They are most commonly reported in the shoulder girdle, hands, throat and abdomen.

There may be tremendous variability over short and long periods of time in symptomatology, frequency, and severity. Individuals may be able to inhibit or not feel a great need to emit their symptoms while at school or work. When they arrive home, however, the tics may erupt with violence and remain at a distressing level throughout the remainder of the day.
It is not unusual for individuals to "lose" their tics as they enter the doctor's office. Parents may plead with a child to "show the doctor what you do at home," only to be told that the youngster "just doesn't feel like doing them" or "can't do them" on command. Adults will say "I only wish you could see me outside of your office," and family members will heartily agree.

Often an individual with minimal symptoms may display more usual severe tics when the examination is over. Thus, for example, the doctor often sees a nearly symptom-free individual leave the office who begins to hop, flail, or bark as soon as the street or even the bathroom is reached.

In addition to the moment-to-moment or short-term changes in symptom intensity, many individuals have oscillations in severity over the course of weeks and months. The waxing and waning of severity may be triggered by changes in the individual's life; for example, around the time of holidays, children may develop exacerbations that take weeks to subside. Other individuals report that their symptoms show seasonal fluctuation. However, there are no rigorous data on whether life events, stresses, or seasons, in fact, do influence the onset or offset of a period of exacerbation. Once a individual enters a phase of waxing symptomatology, a process seems to be triggered that will run its course for weeks or months.

In its most severe forms, individuals may have uncountable motor and vocal tics during all their waking hours with paroxysms of full-body movements, shouting, or self-mutilation. At times the tics seem organized in orchestrated patterns that are characteristic of that individual. Despite that, many individuals with severe tics achieve adequate social adjustment in adult life, although usually with considerable emotional pain. More than the severity of motor and vocal tics, the factors that appear to be of importance with regard to social adaptation include the seriousness of attentional problems, obsesive-compulsive symptoms, the degree of family acceptance and support, intelligence and ego strength.

In adolescence and early adulthood, TS individuals frequently come to feel that their social isolation, vocational and academic failure, and painful and disfiguring symptoms are more than they can bear. At times, a small number may consider and attempt suicide. Conversely, some individuals with the most bizarre and disruptive symptomatology may achieve excelling social, academic, and vocational adjustments.Fortunately, in many cases, tics diminish during the course of adolescence. However, in other cases (10%), the tic symptoms can become even more severe in adulthood.

Associated Behaviors and Cognitive Difficulties

Many, though not all individuals with Ts experience a variety of behavioral and psychological difficulties in addition to tics. These behavioral features have placed TS on the border between neurology and psychiatry and require an understanding of both disciplines to comprehend the complex problems faced by many individuals.

The most frequently reported behavioral problems are attentional deficits, obsessions, compulsions, impulsivity, irritability, aggressivity, immaturity, self-injurious behaviors and depression. Some of the behaviors (e.g., obsessive-compulsive behavior and certain forms of ADHD) may be an integral part of Ts, while others may be common in individuals with Ts because of certain biological vulnerabilities. Still others may represent responses to the social and psychological reactions.

Obsessions and Compulsions

Although TS may present itself purely as a disorder of multiple motor and vocal tics, many TS individuals have obsessive-compulsive (OC) symptoms that may be as disruptive to their lives as the tics -- sometimes even more so. There is recent evidence that obsessive-compulsive symptomatology may actually be another expression of the TS gene and, therefore, an integral part of the disorder. That is to say, the gene may express itself in a particular individual either by tics or by obsessive-compulsive behaviors -- or by both. Whether this is true or not, it has been well documented that a high percentage of TS individuals have OC symptoms.

The nature of OC symptoms in TS individuals is quite variable. Conventionally, obsessions are defined as thoughts, images, or impulses that intrude on consciousness, are involuntary and distressful, and while perceived as silly or excessive, cannot be abolished. Compulsions consist of the actual behaviors carried out in response to the obsessions or in an effort to ward them off. Typical OC behaviors include rituals of counting, checking things over and over, and washing or cleaning excessively. While many TS individuals do have such behaviors, there are other symptoms typical of TS individuals that seem to straddle the border between tics and OC symptoms. Examples are the need to "even things up," to touch things a certain number of times, to perform tasks over and over until they "feel right," as well as self-injurious behaviors. Although individuals with TS can have the full range of OC symptoms, some symptoms such as contamination worries occur less frequently than among OCD individuals without tics or TS.

Attention Deficit Hyperactivity Disorder (ADHD)

Up to 50% of all children with TS who come to the attention of a physician also have attention deficit hyperactivity disorder (ADHD), which is manifested by problems with attention span, concentration, distractibility, impulsivity and motoric hyperactivity. Attentional problems often precede the onset of TS symptoms and may worsen as the tics develop. The increasing difficulty with attention may reflect an underlying biological dysfunction involving inhibition, and may be exacerbated by the strain of attending to the outer world while working hard to ramain quiet and still. Attentional problems and hyperactivity can profoundly affect school achievement. At least 30-40% of children with TS have serious school performance handicaps that require special intervention and children with both TS and ADHD are especially vulnerable to serious, long term educational impairment.

Attention deficits may persist into adulthood and together with compulsions and obsessions, can seriously impair job performance.

Emotional Lability, Impulsivity and Aggressivity

Some individuals with TS (percentages vary greatly in different studies) have significant problems with labile emotions, impulsivity and aggression directed at others. Fits of temper that include screaming, punching holes in walls, threatening others, hitting, biting and kicking are common in such individuals. Often they will be the individuals who also have ADHA, making impulse control a considerable problem. At times, the temper outbursts can be seen as reactions to the internal and external pressures of having TS. A specific etiology for such behavioral problems is, at present, not well understoood. Nevertheless, they create much consternation in teachers and great anguish for the individuals and their families. The treating physician or courselor is often asked whether these behaviors are as involuntary as the tics, or whether they can be controlled. Rather than trying to make such a distinction, it is perhaps more helpful to think of such individuals as having a "thin barrier" between aggressive thoughts and the expression of those thoughts through actions. These individuals may think of themselves as being out of control, a concept that is as frightening to them as it is to others.

Management of these behaviors is often difficult and may involve adjustment of medications, individual therapy, family therapy or behavioral retraining. The intensity of these behaviors sometimes increases as the tics wax and decrease as the tics wane. Along with the tics and ADHD symptoms, these additional disruptive symptoms can cause major social difficulties.

Self-Injurious Behaviors

These may consist of complex tics (e.g., hitting or biting oneself) or may be compulsions (e.g., moving a sore joint over and over in order to achieve a certain painful sensation or the need to touch hot objects).

Learning Difficulties

There are many reasons why children with TS have difficulties in school and may require special educational assistance. Tics may interfere with writing, listening (as trying to control tics may require all of the student's concentration), or may be disruptive in the classroom. Associated symptoms such as ADD, ADHD or obsessions and compulsions may impair attention. Medications may also impair attention and concentration. Finally, there are a number of specific learning disabilities which have been found to be frequently associated with TS. These include impairments in visual-perceptual and visual-motor skills, wide discrepancies between verbal and performance IQs and other specific learning disabilities.

References
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REPRINTED WITH PERMISSION FROM:

Wang, C., & Curry, L. (Eds.) Tourette Syndrome A Continuing Education Course for Registered Nurses, Tourette Syndrome Association - Southern California Chapter. TSA-SC Reseda, CA 1993.

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