Tourette's Syndrome: Psychopathology In Adolescents

The symptoms were first described more than a hundred years ago by the French neurologist Gilles de la Tourette (Harvard Mental Health) , and his name has been given to the disorder now regarded as the most common cause of tics — brief intermittent involuntary or semi-voluntary movements or sounds. A diagnosis of Tourette’s syndrome (TS) requires many motor tics (movements) and at least one vocal or phonic tic (sound), with symptoms beginning before age 18 and lasting more than a year. Often the earliest signs are rapid blinking and twitching of the mouth and nose. Other motor tics are head jerking, tooth grinding, neck twisting, abnormal postures and muscle contractions. More complex movement tics include facial contortions, squatting, deep
knee bends, bowing, jumping, obscene gestures and imitation of other people’s gestures. Vocal or phonic tics include sniffing, throat clearing, squeaking, barking, grunting, humming, blowing or sucking sounds, repeating one’s own or someone else’s words, and spewing obscenities which is know as coprolalia. Obscene gestures and speech are the most conspicuous, no more than 10% of people with the disorder ever have these symptoms. Yet, severity of motor and phonic tics peaks in the second decade of life, and adolescence is a period with strong emphasis on the importance of physical attractiveness. TS may thus result in greater psychopathology during adolescence than in any other stage in life. Tics are not entirely automatic. The need to perform a tic may resemble the need to sneeze or scratch an itch. There may also be a feeling of tension or tightness or a less specific urge or anxiety. Many people with TS say they have to repeat their tics until it “feels right.” Often they try to disguise tics as normal movements and sounds, or control themselves in company and then “release” the tics when they are alone. The symptoms become worse under stress, although they may also occur during sleep. Tics tend to go away during intense concentration. TS is mainly a disorder of childhood and adolescence, with the first symptoms appearing at an average age of six. About 10% of children have some tics and 1% have TS . Most tics fade by age 18, and even when they persist in adulthood, they are likely to become less severe. The is three times more common in boys than in girls, and it has a strong genetic component. The vhance that a parent, brother, sister, or child of a person with the disorder will have at least some chronic tics is about 25%. The concordance (matching) rate for TS itself is 90% in identical twins. A study done on an understanding of the clinical characteristics (Chang , 2003), in terms of general psychopathology, of TS, as it manifests in adolescent patients, and to compare these results with those of previous studies conducted in Western countries. In total, 38 male and 5 female adolescent patients who visited a pediatric neurological specialty clinic for TS between 1 January and 1 March 2003, and who met the World Health Organization criteria for TS, were studied. These patients were clinically interviewed and assessed with the Symptom-Checklist-90-R, Family APGAR and Tic Symptoms questionnaire, a self-report questionnaire based on DSM-IV TS diagnostic criteria. Among this population, the mean age of onset of motor tics was 9.65 ± 3.7 years, and motor tics of the head and eyes were usually the first symptoms to manifest; coprolalia occurred in 44.1% ( n = 19) of our patients. Many of their findings were similar to those reported in trials conducted in other regions of the world. TS symptoms showed stronger correlation with emotional distress in older patients. Individuals with TS often experience social difficulties( Marcks, 2007) which may be caused or compounded by others’ negative perceptions of persons with the disorder. As a result, researchers and clinicians have called for the development of attitude change strategies. One such strategy is preventative disclosure, in which one informs others about his or her condition. To date, no known research exists exploring the effects of this type of disclosure with TS. In an attempt to examine the effects of TS disclosure, adults (N =369) read vignettes that varied in a 2 (male vs. female character) × 2 (preventative disclosure of disorder vs. nondisclosure) design. Respondents answered several questions regarding the character presented in the vignette, which when factor-analyzed, resulted in four factors (social rejection, attributions of a drug/alcohol problem, perceptions of psychological, medical problems, and general concern). The results of this preliminary study are promising, in that the data suggest that preventative disclosure of TS may reduce social rejection, minimize concern, and decrease perceptions of drug and alcohol problems. No effect of character gender was found. Implications of these findings, limitations to the current study, and directions for future research where discussed. The available anatomic and electrophysiological findings in TS are reviewed in the context of an emerging picture of the crucial role that neural oscillations play in maintaining normal central nervous system (CNS) function ( Leckman, Vaccarino, Kalanthini & Rothenberger, 2007 ). Neurons form behavior-dependent oscillating networks of various sizes and frequencies that bias input selection and facilitate synaptic plasticity, mechanisms that cooperatively support temporal representation as well as the transfer and long-term consolidation of information. Coherent network activity is likely to modulate sensorimotor gating as well as focused motor actions. When these networks are dysrhythmic, there may be a loss of control of sensory information and motor action. The known electrophysiological effects of medications and surgical interventions used to treat TS likely have an ameliorative effect on these aberrant oscillations. Similarly, a strong case can be made that successful behavioral treatments involve the willful training regions of the prefrontal cortex to engage in tic suppression and the performance of competing motor responses to unwanted sensory urges such that these prefrontal regions become effective modulators of aberrant thalamocortical rhythms. In this they saw a deeper understanding of neural oscillations may illuminate the complex, challenging, enigmatic, internal world that is TS. Thalamic abnormality has been implicated in the pathophysiology of TS(Lee, Yoo, Cho Ock, Lim & Panych 2006). They examined the presence of aberrant thalamic volume from the treatment-naive boys with TS using MRI Volumetric MRI was performed on 18 treatment-naive boys with TS, aged 7–14 years, and 16 healthy comparison subjects. The anatomical boundaries were then manually parcellated to measure the thalamic volume. They found that TS subjects had a significantly larger left thalamus in comparison with those of healthy subjects. On the contrary, no group difference was observed from the right thalamic volume. TS subjects also showed a significant reduction in rightward asymmetry in thalamic volume compared with the healthy subjects. Their findings provide new evidence of abnormal thalamic volume in pediatric TS. In Leckman, Vaccarino, Kalanithi & Rothenberger’s case study, they studied the comorbid behavioral and mood problems in children with non psychiatric TS (TS) and their relationship with severity of tic disorder. Sixty-nine TS children and 69 healthy controls were assessed by Child Behavior Checklist (CBCL) and Yale Global Tic Severity Scale (YGTSS). The relationships between behavioral problems and severity of tic symptoms were analyzed statistically by comparison, correlation and multiple linear regression. TS patients scored significantly lower on the CBCL competency subscales and total score, and higher on all behavioral problem subscales and total score. Expectedly, the TS children had lower social competence than normal children. Among the TS children, the severity of tic symptoms is positively correlated with the severity of overall impairment in school and social competence. When the behavioral and mood problems commonly associated with TS were studied in detail, we found that delinquent behavior, thought problems, attention problems, aggressive behavior and externalizing are positively correlated with severity of tic symptoms. The findings indicated that children with TS-only also had a broad range of behavioral problems, and some of these were related to the severity of tic symptoms. Brain imaging studies have revealed anatomical anomalies in the brains of individuals with TS( Plessen et al, 2006). Prefrontal regions have been found to be larger and the corpus callosum (CC) area smaller in children and young adults with TS compared with healthy control subjects, and these anatomical features have been understood to reflect neural plasticity that helps to attenuate the severity of tics. CC white matter connectivity, as measured by the Fractional Anisotropy (FA) index from diffusion tensor images, was assessed in 20 clinically well-defined boys with TS and 20 age- and gender-matched controls. The hypothesis that children with TS would show reduced measures of connectivity in CC fibers was confirmed for all subregions of the CC There was no significant interaction of TS and region. Reductions in FA in CC regions may reflect either fewer interhemispheric fibers or reduced axonal myelination. FA values did not correlate significantly with the severity of tic symptoms. Group differences in measures of connectivity did not seem to be attributable to the presence of comorbid ADHD or OCD, to medication exposure, or group differences in IQ. Their findings of a reduced interhemispheral white matter connectivity add to the understanding of neural connectivity and plasticity in the brains of children who have TS.
Various behavioral therapeutic strategies are used in the treatment of TS (Hoogduin, Verdellen, Cath, 1996). Some success has been reported following treatment involving massed negative practice, contingency management, relaxation training, self-monitoring and habit reversal Only a few controlled studies have; however, been carried out. new treatment strategy involving exposure and response prevention has recently been developed, based on the ideas of J. Bliss. Bliss, himself a TS sufferer, described his own experiences of the disorder. He expounded the notion that TS patients produced the movements and noises voluntarily in order to rid themselves of unpleasant sensations. Over recent years, an increasing amount of research has focused on the extent to which tics can be regarded as voluntary and the existence of such sensations. Hoogduin et al, describes a new treatment method for TS, consisting of 10 2-hour sessions of exposure and response prevention. This method is based on the notion expounded by Bliss (1980) that patients with this syndrome often produce the tics voluntarily in order to rid themselves of unpleasant sensations The hypothesis was that, if the patient was aware of premonitory sensations, it should be possible to intervene at the level of these sensations; by preventing the tics, the patient might habituate to the sensations. As a result, it was hypothesized, the urge to produce the movements and sounds would eventually diminish, leading to an extinction of the motor and vocal tics. This article discusses four Gilles de la Tourette patients for whom this method of exposure and response prevention was reasonably effective. Within-session habituation to the premonitory sensations took place in three of the four patients. These case studies of four patients with a severe form of GTS would seem to show that treatment using exposure and response prevention can bring about a decrease in the number of motor and vocal responses. There was a reduction in measured tiquing behaviour (at the end of treatment) ranging from 68% (case IV) to 83% (case II). At follow-up after 2 months, the improvement in tiquing behaviour ranged from 50% (case IV) to 100% (case II). In cases I and IV, the improvement, as measured at the end of treatment, had decreased by follow-up 2 months later. In cases II and III, there was a further improvement at follow-up measurement. All subjects reported a subjective improvement at the end of treatment, ranging from 45% (case III) to 60% (case I). At follow-up after 2 months, however, the subjectively rated improvement had decreased in three patients (case I: 0%,case III: 30%, case IV: 40%). One patient (case II) reported a further improvement at follow-up; after 2 months she considered herself to be 90% improved. Multimodal treatment for Tourette’s syndrome is usually indicated (J. Leckman, 2002) . this approach includes educational and supportive interventions appropriate for any chronic disease. Because acute and chronic stress can exacerbate tics, psychotherapeutic attention to difficulties of self-esteem, social coping, family issues, and school adjustment could have non-specific ameliorative effects on tic severity and on attendant anxiety and depression. Local chapters of patients’ advocacy organizations, such as the Tourette’s Syndrome Association, can have a very supportive role, by putting families of newly diagnosed children in contact with more experienced families. Parents should be encouraged to build on their child’s strengths. Many cases of uncomplicated Tourette’s syndrome can be successfully managed with just these interventions, and do not need anti-tic drugs. When patients present with coexisting hyperkinetic disorder, obsessive-compulsive disorder, depression, or two or three of these disorders, it is usually better to treat these comorbid disorders first, since successful treatment of them will often diminish tic severity. Ideal anti-tic treatments are not presently available. None of the drugs or techniques can be used effectively just when tics are at their worst. Most of the available pharmacological drugs need long-term treatment, and many have potentially serious side-effects. Indeed, for some drugs, it is much easier to begin their use than to stop them. The natural waxing and waning pattern of tics often confounds the results of drug trials. Even without intervention, periods of severe tics will be followed by one of spontaneous waning. Because tic-suppressant drugs generally need several weeks to have their full effect, it is often difficult to distinguish response to a drug from spontaneous waning of symptoms. Thus, it is usually best to avoid beginning or increasing drugs as soon as an exacerbation begins. There is only one way to fully prevent TS and that is if one parent has the disorder not to reproduce.
References
Chang, H., Tu, M., & Wang, H.
(2004, August). Tourette's syndrome: Psychopathology in adolescents. Psychiatry & Clinical Neurosciences, 58(4), 353-358. Retrieved April 1, 2008, Hoogduin, K., Verdellen, C., & Cath, D. (1997, June). Exposure and Response Prevention in the Treatment of Gilles de la Tourette's Syndrome: Four Case Studies. Clinical Psychology & Psychotherapy, 4(2), 125-135. Retrieved March 23, 2008, Leckman, J., Vaccarino, F., Kalanithi, P., & Rothenberger, A. (2006, June). Annotation: Tourette syndrome: a relentless drumbeat – driven by misguided brain oscillations. Journal of Child Psychology & Psychiatry, 47(6), 537-550. Retrieved April 8, 2008, Leckman, J. (2002, November 16). Tourette's syndrome. Lancet, 360(9345), 1577. Retrieved March 20, 2008, from Psychology and Behavioral Sciences Collection database. Lee, J., Yoo, S., Cho, S., Ock, S., Lim, M., & Panych, L. (2006, January). Abnormal thalamic volume in treatment-naïve boys with Tourette syndrome. . Retrieved April 14, 2008 Marcks, B., Berlin, K., Woods, D., & Davies, W. (2007, Spring). Impact of Tourette Syndrome: A Preliminary Investigation of the Effects of Disclosure on Peer Perceptions and Social Functioning. Psychiatry: Interpersonal & Biological Processes, 70(1), 59-67. Retrieved April 3, 2008, from Psychology and Behavioral Sciences Collection
database Plessen, K., Grüner, R., Lundervold, A., Hirsch, J., Xu, D., Bansal, R., et al. (2006, October). Reduced white matter connectivity in the corpus callosum of children with Tourette syndrome. Journal of Child Psychology & Psychiatry, 47(10), 1013-1022. Retrieved April 13, 2008,
Tourette's disorder. (2005, June). Harvard Mental Health Letter, Retrieved March 10, 2008, .