1. Tourette Syndrome
Psychopathology
Winter 2013

2. Tourette Syndrome: Introduction
Neurological disorder characterized
by repetitive, involuntary
movements and vocalizations called
tics
Typical onset in early childhood or
adolescence between the ages of 2
and 15

3. Neurological Component to TS
 TS thought to be a developmental disorder
of synaptic neurotransmission
 Involves basal ganglia and
related neural pathways
 Failure in filtering (disinhibition)
along striatal-thalamic-cortical
circuit, resulting in ineffective removal of
unwanted, interfering information
 Same circuits and structures involved in
OCD, ADHD

4. Neurological Component to TS
Tourette’s is
thought to
involve both
dopamine and
serotonin where
dopamine is too
high and
serotonin too
low.

5. What are tics?
 Repetitive, sudden, involuntary or semi-
voluntary movements or sounds
 Non-rhythmic
 Classification
 Motor or Phonic (vocal)
 Simple or complex

6. Motor Tics
Simple motor tics
 Involve single muscle or functionally
related group of muscles
 Fast and brief, lasting <1 sec
 May occur in bouts of rapid
succession
Complex motor tics
 Involve more muscle groups
 Sequentially and/or simultaneously
produced movements
 May appear purposeful

7. Phonic/Vocal Tics
 Simple phonic tics
 Single, meaningless sound or noise
 Complex phonic tics
 Linguistically meaningful utterances and
verbalizations

8. Simple Complex
Motor tics Eye blinking Hand gestures
Nose wrinkling Facial contortions
Jaw thrusting Jumping
Shoulder shrugging Touching
Wrist snapping Repeatedly smelling object
Neck jerking Squatting
Limb jerking Copropraxia
Abdominal tensing Echopraxia
Phonic tics Sniffing Single words or phrases
Barking Partial words or syllables
Grunting Repeated use of word or
Throat clearing words out of context
Coughing Palilalia
Chirping Echolalia
Screaming Coprolalia

9. Tic Characteristics
 Premonitory feelings or
sensations
 Sensory discomfort in muscle
or muscle groups preceding
tic
 Similar to having to sneeze
 Described as physical tension,
pressure, tickle, itch, or other
sensory experience
 Some described as “psychic”
phenomenon such as anxiety
rather than physical sensation
 Performing tic results in relief
of sensation
 Some patients describe
needing to perform tic “just
right” in order to relieve
sensation

10. Tic Characteristics
 May be temporarily suppressed
 Rebound phenomenon
 Suggestibility in some individuals
 When tics brought to child’s attention, they increase
 Usually increase with heightened emotion
 Stress
 Anger
 Excitement

11. Tic Characteristics
 Often occur while relaxing, and may
increase during relaxation after stress
 May diminish during either concentration
or distraction or during physical activity
 May persist during all sleep stages, but
not common during sleep

12. Tourette Syndrome: Clinical
Presentation
 Clinical manifestation diverse: ”no two
patients the same”
 Majority have minor tics
 Tics can change daily
 Coprolalia (swearing) RARE (6-12%)
 Misconception that coprolalia a core
symptom may impede diagnosis

13. Life with Tourette Syndrome

14. Tourette Syndrome:DSM-IV-TR
Criteria
 Both multiple motor and one or more vocal tics
present at some time during illness, although not
necessarily concurrently
 Tics occur many times a day (usually in bouts) nearly
every day or intermittently throughout a period of
more than one year, and during this period there was
never a tic-free period of >3 consecutive months
 Onset before age 18 years (thought TS is considered
a lifelong illness)
 Disturbance not due to direct physiological effects of a
substance or general medical condition

15. Other DSM-IV-TR Tic Disorders
Tic disorders differ on basis of
duration of disorder and presence of
motor and/or phonic tics
Chronic Motor or Vocal Tic Disorder
 Only motor or only vocal tics
Transient Tic Disorder
 May have both or only one tic form
 Duration: 4 weeks to 12 months

16. TS: Diagnosis
 No definitive diagnostic test
 Diagnosis based on thorough clinical
evaluation and history of symptoms
 Observation for assessment of symptoms
aids differential diagnosis
 May not present tics during evaluation
 Lab work or imaging can rule out other
disorders

17. TS: Differential Diagnosis
 Tics and TS may resemble other disorders or
conditions
 Myoclonus
 Dystonia
 Hyperkinetic disorders
 Extreme ADHD
 Seizure disorder
 Developmental stuttering
 Tics may also be symptom of neurologic insult such
as CO poisoning, medication-induced insult, or
head trauma

18. Prevalence and Incidence
 Originally thought to be rare,
but now recognized to be
more prevalent
 20% of children experience
tics, mostly transient
 Prevalence estimates 0.3%-3%
of all children
 750,000* children in US,
although many undiagnosed
 Occurs in all races and
ethnicities
 Males 3-4x > females
*Tourette Syndrome Association, www.tsa-usa.org

19. TS: Course
 Tics typically appear in early childhood (most
often by age 6 or 7)
 In 96% of patients, disorder manifested by
age 11
 Simple motor tics often initial symptom
 eye blinking and neck movements common
 Phonic tics and more complex motor tics
follow in next two years, but may appear later
in adolescence
 Motor tics tend to progress top-to-bottom and
central-to-peripheral
 Phonic tics also progress in complexity

20. TS: Course, cont.
 Tics generally occur daily, but tend to wax
and wane in frequency and intensity
 Type, location, and severity may change
over time
 By age 18 years, half of patients are free
of tics
 For those whose tics persist, severity
typically diminishes in adulthood

21. Comorbidity
 Approx 90% of patients have comorbid condition
 ADHD
 Obsessive compulsive symptoms/disorder
 Learning difficulties/Learning disorder
 Anxiety disorders, including phobias
 Mood disorders (depression, dysthymia)
 Sleep disturbance
 Oppositional defiant disorder
 Executive dysfunction
 Self-injurious behaviors (may be tics)

22. Comorbidity: TS and ADHD
 At least 50% of TS patients have ADHD
 ADHD typically presents prior to tics
 Impulsive behaviors may be complex tics
 e.g., pointing out a flaw in another person’s
appearance, corpropraxia, corprolalia, spinning
 Associated with greater social difficulties,
academic problems, and disruptive
behavior

23. Comorbidity: TS and OCD
 Obsessive or compulsive symptoms
and/or behaviors suggested to occur in
nearly all patients
 Clinical OCD occurs in ~25% of TS
patients
 Can be difficult to differentiate complex
tics from compulsive behaviors
 e.g., touching something repeatedly until it
feels “just right”

24. Course with Comorbidities
Jancovic, 2001

25. Social, Emotional, Familial Effects

26. Social Impact of TS
 Increased self-
consciousness and poor self-
esteem
 Often targets for mocking,
bullying
 Withdrawal from social
situations/Isolation
 Difficulties in school or
workplace
 Comorbid ADHD or other
disorders increases
likelihood of social problems

27. Academic Issues
 Children with TS are typical at or above normal intelligence,
many may even be gifted
 Lack of education of Teachers and Administration causes them
to see tic behavior as misbehavior
 Bullying and social issues with peers
 Dysgraphia makes writing difficult
 Motor tics make writing, reading, copying very difficult
 Eye Rolling tics etc. can look as though the child is not paying
attention
 Loss of focus due to tics worsened by anxiety surrounding tic-
ing in school
 Stress of testing and social issues worsen tic-ing
 Impulsivity and Distracting behavior can make the
environment distracting for classmates
 Classmates and sensory inputs can be distracting for child
with TS

28. Management and Treatment
 Multi-component management approach
recommended
 Therapeutic Interventions
 Education for child, family, teachers, peers
 Behavioral approaches used to reduce tics
 Medication
 Academic accommodations
 Psychosocial and psychological supports

29. Therapeutic Interventions
 Child
 Social Functioning
 Self-Esteem
 Anxiety
 Depression and feelings of Isolation
 Family
 Parenting skills, techniques, education
 Stress/anxiety
 Depression/guilt

30. Psychoeducation
 Child
 Helps normalize
 Reduces guilt
 Increases Self-Esteem
 Improves Behavioral Interventions
 Family
 Parenting Skills
 Reduced frustration
 Reduced anxiety
 Increased number of techniques
 School
 Administration
 Teachers
 Peers (Informal presentations, sensitivity training)

31. Behavioral Therapy
 Cognitive Behavioral Therapy for tic reduction
 Assertiveness Training for increase in Self-Esteem
 Relaxation Therapy for tic reduction and anxiety
reduction
 Habit Reversal Therapy based on the fact that tics are
reduced during periods of great focus
 Hypnosis/Self Hypnosis
 Narrative Therapy to externalize tics

32. Treatment: Medication
 Simply having tics not indicator for
medication
 Medication usually considered when
symptoms interfere with peer
relationships, social interactions,
academic or job performance, or
activities of daily living
 No drug will entirely eliminate tics
 Unwanted side-effects may be
worse than symptoms
 Goals is to relieve tic-related
physical discomfort or
embarrassment and to achieve a
degree of control of tics that allows
the child to function as normally as
possible

33. Treatment: Medication
 Medication may be prescribed for tics,
comorbid disorders or both
 Monotherapy ideal, but polypharmacy
common
 Most med use is off-label or not
specifically approved for children
 Controversy regarding whether ADHD
treatment with psychostimulants
exacerbates tics

34. Management: Academic
Accommodations
 Classroom accommodations
 Tic breaks
 Untimed tests
 Private room for test-taking
 Can make accommodations
under 504 plan for an
Individual Education Plan (IEP)
 Semiformal classroom
presentations or videos on TS
to educate teacher and
students

35. Management: Psychosocial and psychological
supports
 Provide information and assistance in
accessing support networks
 Address potential social impact (reduced
self-esteem, self-consciousness) via
psychotherapy
 May benefit from social skill building

36. Treatment: Other Approaches
 Alternative approaches
such as fish oil/vitamin
supplements are being
investigated
 Dietary modification and
allergy testing have been
explored for tic
management but not
supported (allergies are a
stress to the body)
 High frequency Deep Brain
Stimulation (DBS) shown
to be effective in small
number of cases (no
children)

37. Prognosis
 In 50% of TS cases,
symptoms will reduce
or resolve by age 18.
 With proper education
and behavior changes,
adults with TS can
absolutely live normal
lives.