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Tourette Syndrome_ History and Clinical Aspects of Tics

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Tourette Syndrome_ History and Clinical Aspects of Tics Powered By Docstoc
					     What Is Tourette Syndrome?

w A neurological syndrome characterized by
  multiple motor and vocal tics with onset before
  age 21 years
w 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
w Tics can be simple or complex
w Behavioral features of TS often include OCD,
  ADHD, or both
    History of Tourette Syndrome

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

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

w 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
w Alternative views continued to be that tics
  were post-rheumatic and a variant of chorea
  or were hysterical
   History of Tourette Syndrome

w In the 1930s, developmental psychologists
  described tics as common among school-
  age children, but this was largely ignored by
  the neurological and psychoanalytic
  communities
w Observations of heritability and response to
  dopamine receptor antagonists began to
  suggest a biological basis
              Modern View of TS

w Tic disorders are biological and likely
  involve the basal ganglia
w Tourette syndrome is familial with
  incomplete penetrance and possibly variable
  expression
  n   Once thought to be a simple autosomal
      inheritance, it now appears that the genetics are
      complex and there are significant environmental
      factors
         Clinical Features of Tics
w Median age of onset is 7 years
w Most common initial symptom is eye blinking
  (36-48%)
   n   Most common misdiagnoses are eye problems and
       allergies
w Vocal tics are presenting symptom in 12 - 37%
w Severity in childhood does not predict severity in
  adulthood
w Severity is rarely greater in adulthood than in
  childhood
         Incidence of TS and Tics

w Determination of incidence and prevalence is
  complicated
w 33% of patients with tics do not recognize some or
  all of their tics
w Parents and teachers may not recognize tics
w 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)
       Natural History of TS and Tics

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

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

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

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

w Complex Motor Tics
  n head shaking, trunk flexion, scratching,

    touching, throwing, hitting, jumping, kicking,
    obscene gestures (copropraxia) or imitating
    gestures (echopraxia)
w Complex Vocal Tics
  n shouting of obscenities or profanities

    (coprolalia), repetition of the words of others
    (echolalia), repetition of final syllable, word, or
    phrase of own words (palilalia)
       Clinical Features of Tics

w Premonitory feelings or sensory tics occur
  in 80% of patients with TS:
   n Specific sensation (e.g. "itch", "dry
     throat")
   n Nonspecific urge or feeling such as
     anxiety
              Influencing Factors

w Tics may change with emotional or cognitive
  state
  n   Decrease with distraction
  n   Increase with stress
  n   Increase during relaxation after a period of stress
  n   May be suppressible with effort; frequent
      "rebound" increase afterward
  n   May persist in all stages of sleep
       Classification of Tic Disorders -
                   DSM-IV
w Tourette Syndrome
   n   Multiple motor tics and at least one vocal tic
   n   Intermittently present for > 1 yr
   n   Onset before age 18 yrs
w Chronic Motor or Vocal Tic Disorder (presence
  of only motor or vocal tics for greater than 12
  months)
w Transient Tic Disorder (presence of tics for more
  than four weeks but less than 12 months)
w Tic Disorder Not Otherwise Specified
     Associated Symptoms in TS

w Majority of patients with TS have symptoms
  of ADHD or OCD at some point during the
  illness
w 50% incidence of both ADHD and OCD in
  TS (compared to 3-5% in gen. pop.)
w These symptoms are often more bothersome
  or interfering than tics
            Common Obsessive
             Symptoms In TS
w Frequent and repetitive worrying (e.g., harm
  coming to self, family).
w Preoccupation with need for order and routine
  (e.g., difficulty accepting change).
w Repetitive thoughts, words, and phrases.
w Urges to perform forbidden or dangerous
  activities (e.g., stick finger in fan, hot stove,
  etc.).
w Indecision, tendency to be unsure of self.
w Preoccupation with dirt/contamination
            Common Compulsive
              Symptoms In TS
w Need for order, routine, symmetry (“evening-up”).
w Repetitive checking and re-checking (e.g., doors,
  appliances, belongings).
w Need for perfection, tendency to repetitively
  perform same activity to ensure correctness.
w Repetitive touching of objects, persons (may be a
  complex motor tic).
w Cleaning, washing, dressing rituals.
w Inability to tolerate certain types of clothing, foods
  touch one another on the plate.
         Neuropsychology of TS


w Intellectual Ability/IQ Testing
w Learning Disabilities - Fact or Fiction?
w Specific Neuropsychological Deficits
w Potential Confounding Factors Influencing
  Neuropsychological Function in TS
          Intellectual Ability In TS

w IQ Scores Normally Distributed in
  Epidemiological Studies (Apter et al, 1993)
w Below Average IQ Reported in TS Clinic
  Samples (Parraga & McDonald, 1996)
   n Verbal IQ > Performance IQ

   – Most studies failed to control for presence of

     ADHD or LD (Bornstein, 1990)
w PIQ Subtests Primarily Assess Visuospatial
  Function and Psychomotor Speed
         Learning Disabilities in TS

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

w ADHD Significant Predictor of School Problems in TS
  (Abwender et al., 1996)
w Higher Rate of Special Education Placement in TS
  (Comings et al., 1990; Kurlan et al., 1994)
w Kurlan et al., Neurology, 2001:
  n Epidemiological study of tic prevalence in Monroe

    County, NY
  n 1596 students directly evaluated (N=341, Spec. Ed,

    N=1255, Reg. Ed)
      l   Spec. Ed - 23.4% weighted prevalence of tics
      l   Reg. Ed – 19.7% weighted prevalence of tics
    Neuropsychological Deficits in TS

w Visuomotor Deficits
   n Consistent deficits noted on copying tasks (e.g.,

     geometric designs)
   n 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)
   n TS individuals perform about 1.0 SD below age norm

w Visuomotor Integration Deficits also Common in ADHD
  Children
  Neuropsychological Deficits in TS

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

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

w “Executive” Function (EF)
   n Loosely defined construct:

      l   mental tracking, sustained attention, working memory
      l   planning and organization
      l   goal-directed behavior
      l   cognitive flexibility
      l   impulse control, self-regulation
w EF Deficits also Common in ADHD (Barkley et
  al., 1992) and in OCD (Hollander & Wong, 1996;
  Rosenberg et al., 1997)
        Neuropsychological Deficits in TS

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

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

				
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