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What Is Tourette Syndrome?

What Is Tourette Syndrome?. A neurological syndrome characterized by multiple motor and vocal tics with onset before age 21 years

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What Is Tourette Syndrome?

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  1. What Is Tourette Syndrome? • A neurological syndrome characterized by multiple motor and vocal tics with onset before age 21 years • Tics are involuntary, repetitive, stereotyped movements that occur many times a day, nearly every day. Tics typically change anatomic location, frequency, type, complexity, and severity over time • Tics can be simple or complex • Behavioral features of TS often include OCD, ADHD, or both

  2. History of Tourette Syndrome • First report in 1825 by Itard who described a French noblewoman with body tics, barking sounds and uncontrollable utterance of obscenities • Itard also described 7 men and 3 women with a variety of sudden, bizarre behaviors that we would now call tics

  3. History of Tourette Syndrome • George Gilles de la Tourette described 8 patients in 1885 who all had motor tics • 6 made noises (vocal tics) • 5 shouted obscenities (coprolalia) • 5 repeated words of others (echolalia) • 2 mimicked others gestures (echopraxia) • Itard’s patient became the standard example and formed the basis for initial conceptualization that tics were progressive and degenerative

  4. History of Tourette Syndrome • In early 1900s psychoanalytic school become dominant and attributed tics to repressed sexual impulses and/or conflict between parent and child resulting in deficits of will and character • Alternative views continued to be that tics were post-rheumatic and a variant of chorea or were hysterical

  5. History of Tourette Syndrome • In the 1930s, developmental psychologists described tics as common among school-age children, but this was largely ignored by the neurological and psychoanalytic communities • Observations of heritability and response to dopamine receptor antagonists began to suggest a biological basis

  6. Modern View of TS • Tic disorders are biological and likely involve the basal ganglia • Tourette syndrome is familial with incomplete penetrance and possibly variable expression • Once thought to be a simple autosomal inheritance, it now appears that the genetics are complex and there are significant environmental factors

  7. Clinical Features of Tics • Median age of onset is 7 years • Most common initial symptom is eye blinking (36-48%) • Most common misdiagnoses are eye problems and allergies • Vocal tics are presenting symptom in 12 - 37% • Severity in childhood does not predict severity in adulthood • Severity is rarely greater in adulthood than in childhood

  8. Incidence of TS and Tics • Determination of incidence and prevalence is complicated • 33% of patients with tics do not recognize some or all of their tics • Parents and teachers may not recognize tics • Recent studies indicate that 5 – 6 % of school children have tics at some time and that up 0.1 - 3% have chronic tics (> 1 year)

  9. Natural History of TS and Tics • Tics tend to wax and wane throughout childhood and adolescence • Tics change in type, frequency and intensity • Long-term prognosis (e.g., early adulthood) quite favorable: • 1/3 of patients experience resolution of tics • 1/3 of patients have very mild tics • 1/3 of patients continue to have persistent tics

  10. Clinical Features of Tics • Tics are categorized as motor or vocal • Any tic that produces a sounds from the nose of mouth is a vocal tic • Distinction between motor and vocal is based more on history than on physiology. Muscle contraction underlies both types • Motor tics may be abrupt onset and fast ("clonic") or slow and sustained ("dystonic" or "tonic")

  11. Clinical Features of Tics • Tics are also categorized as simple or complex • Simple tics are individual movements that typically look or sounds purposeless • Complex tics resemble purposeful movements or may be ensembles of more simple movements • The tics are normal movements occurring in an abnormal context and pattern

  12. Simple Tics • Simple Motor Tics • blinking, nose twitching, head jerking, blepharospasm, oculogyric movements, bruxism, torticollis, sustained mouth opening, abdominal tensing • Simple Vocal Tics • sniffing, throat clearing, grunting, squeaking, screaming, coughing, blowing and sucking sounds

  13. Complex Tics • Complex Motor Tics • head shaking, trunk flexion, scratching, touching, throwing, hitting, jumping, kicking, obscene gestures (copropraxia) or imitating gestures (echopraxia) • Complex Vocal Tics • shouting of obscenities or profanities (coprolalia), repetition of the words of others (echolalia), repetition of final syllable, word, or phrase of own words (palilalia)

  14. Clinical Features of Tics • Premonitory feelings or sensory tics occur in 80% of patients with TS: • Specific sensation (e.g. "itch", "dry throat") • Nonspecific urge or feeling such as anxiety

  15. Influencing Factors • Tics may change with emotional or cognitive state • Decrease with distraction • Increase with stress • Increase during relaxation after a period of stress • May be suppressible with effort; frequent "rebound" increase afterward • May persist in all stages of sleep

  16. Classification of Tic Disorders - DSM-IV • Tourette Syndrome • Multiple motor tics and at least one vocal tic • Intermittently present for > 1 yr • Onset before age 18 yrs • Chronic Motor or Vocal Tic Disorder (presence of only motor or vocal tics for greater than 12 months) • Transient Tic Disorder (presence of tics for more than four weeks but less than 12 months) • Tic Disorder Not Otherwise Specified

  17. Associated Symptoms in TS • Majority of patients with TS have symptoms of ADHD or OCD at some point during the illness • 50% incidence of both ADHD and OCD in TS (compared to 3-5% in gen. pop.) • These symptoms are often more bothersome or interfering than tics

  18. Common Obsessive Symptoms In TS • Frequent and repetitive worrying (e.g., harm coming to self, family). • Preoccupation with need for order and routine (e.g., difficulty accepting change). • Repetitive thoughts, words, and phrases. • Urges to perform forbidden or dangerous activities (e.g., stick finger in fan, hot stove, etc.). • Indecision, tendency to be unsure of self. • Preoccupation with dirt/contamination

  19. Common Compulsive Symptoms In TS • Need for order, routine, symmetry (“evening-up”). • Repetitive checking and re-checking (e.g., doors, appliances, belongings). • Need for perfection, tendency to repetitively perform same activity to ensure correctness. • Repetitive touching of objects, persons (may be a complex motor tic). • Cleaning, washing, dressing rituals. • Inability to tolerate certain types of clothing, foods touch one another on the plate.

  20. Neuropsychology of TS • Intellectual Ability/IQ Testing • Learning Disabilities - Fact or Fiction? • Specific Neuropsychological Deficits • Potential Confounding Factors Influencing Neuropsychological Function in TS

  21. Intellectual Ability In TS • IQ Scores Normally Distributed in Epidemiological Studies (Apter et al, 1993) • Below Average IQ Reported in TS Clinic Samples (Parraga & McDonald, 1996) • Verbal IQ > Performance IQ • Most studies failed to control for presence of ADHD or LD (Bornstein, 1990) • PIQ Subtests Primarily Assess Visuospatial Function and Psychomotor Speed

  22. Learning Disabilities in TS • No Long-Term Outcome Studies of the Learning Patterns in TS (Walkup et al., 1999) • LD in TS Highly Correlated with Presence of ADHD (Similar to that reported in ADHD children) • Prevalence of LD in TS Estimated to be 22% (Erenberg et al., 1986; Abwender et al., 1996) • Math and Written Language Skills Most Common Areas of Weakness (Burd et al., 1992; Brookshire et al., 1994; Schuerholz et al., 1996)

  23. School Problems in TS • ADHD Significant Predictor of School Problems in TS (Abwender et al., 1996) • Higher Rate of Special Education Placement in TS (Comings et al., 1990; Kurlan et al., 1994) • Kurlan et al., Neurology, 2001: • Epidemiological study of tic prevalence in Monroe County, NY • 1596 students directly evaluated (N=341, Spec. Ed, N=1255, Reg. Ed) • Spec. Ed - 23.4% weighted prevalence of tics • Reg. Ed – 19.7% weighted prevalence of tics

  24. Neuropsychological Deficits in TS • Visuomotor Deficits • Consistent deficits noted on copying tasks (e.g., geometric designs) • 10/12 Studies (N=308 TS patients, mean age of ~10 yrs) revealed individual deficits or group differences on various copying tasks (Schultz et al., 1999) • TS individuals perform about 1.0 SD below age norm • Visuomotor Integration Deficits also Common in ADHD Children

  25. Neuropsychological Deficits in TS • Gross/Fine Motor Skill • Literature equally compelling and similar to that reported for visuomotor deficits • Preponderance of studies suggest greater fine motor skill deficits in TS • Deficits in both TS adults and children about 0.5-1.0 SD below unaffected controls (Schultz et al., 1998)

  26. Neuropsychological Deficits in TS • Spatial/Perceptual Deficits • Reported VIQ > PIQ suggest spatial/perceptual problems in TS (? confounding factors) • Deficits largely due to difficulty with pure motor function and/or impaired visuoperceptual integration (Schultz et al., 1998) • Confounding Factor - Lack of studies employing motor-free spatial tasks

  27. Neuropsychological Deficits in TS • “Executive” Function (EF) • Loosely defined construct: • mental tracking, sustained attention, working memory • planning and organization • goal-directed behavior • cognitive flexibility • impulse control, self-regulation • EF Deficits also Common in ADHD (Barkley et al., 1992) and in OCD (Hollander & Wong, 1996; Rosenberg et al., 1997)

  28. Neuropsychological Deficits in TS • “Executive” Function (EF) • No consistent EF deficit has emerged in TS • Tasks of mental flexibility, planning and sequencing most commonly cited • Consistent findings of slowed response time on CPT tasks • Cirino et al., 2000: • No differences in card sorting performance between TS alone and TS+ADHD • Results suggest that executive dysfunction may occur in TS independent of co-morbid ADHD

  29. Confounding Factors • Tic Disorder Itself: • Natural history of waxing and waning tics • Active (or passive) tic suppression • Tic suppressing medications (e.g., neuroleptics, clonidine) • Co-Morbid Conditions (ADHD, OCD) • Methodological Issues (sample sizes, use of clinic samples)

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