1. Tourette Syndrome PsychopathologyWinter 2013

2. Tourette Syndrome: IntroductionNeurological disorder characterized by repetitive, involuntary movements and vocalizations called ticsTypical onset in early childhood or adolescence between the ages of 2 and 15 3. Neurological Component to TS TS thought to be a developmental disorderof synaptic neurotransmission Involves basal ganglia andrelated neural pathways Failure in filtering (disinhibition)along striatal-thalamic-corticalcircuit, resulting in ineffective removal ofunwanted, interfering information Same circuits and structures involved inOCD, ADHD 4. Neurological Component to TSTourettes isthought toinvolve bothdopamine andserotonin wheredopamine is toohigh andserotonin toolow. 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 TicsSimple motor tics Involve single muscle or functionallyrelated group of muscles Fast and brief, lasting 3 consecutive months Onset before age 18 years (thought TS is considereda lifelong illness) Disturbance not due to direct physiological effects of asubstance or general medical condition 15. Other DSM-IV-TR Tic DisordersTic disorders differ on basis of duration of disorder and presence of motor and/or phonic ticsChronic Motor or Vocal Tic Disorder Only motor or only vocal ticsTransient 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 clinicalevaluation and history of symptoms Observation for assessment of symptomsaids differential diagnosis May not present tics during evaluation Lab work or imaging can rule out otherdisorders 17. TS: Differential Diagnosis Tics and TS may resemble other disorders orconditions Myoclonus Dystonia Hyperkinetic disorders Extreme ADHD Seizure disorder Developmental stuttering Tics may also be symptom of neurologic insult suchas CO poisoning, medication-induced insult, orhead trauma 18. Prevalence and Incidence Originally thought to be rare,but now recognized to bemore prevalent 20% of children experiencetics, mostly transient Prevalence estimates 0.3%-3%of all children 750,000* children in US,although many undiagnosed Occurs in all races andethnicities Males 3-4x > females *Tourette Syndrome Association, www.tsa-usa.org 19. TS: Course Tics typically appear in early childhood (mostoften by age 6 or 7) In 96% of patients, disorder manifested byage 11 Simple motor tics often initial symptom eye blinking and neck movements common Phonic tics and more complex motor ticsfollow in next two years, but may appear laterin 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 waxand wane in frequency and intensity Type, location, and severity may changeover time By age 18 years, half of patients are freeof tics For those whose tics persist, severitytypically 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 persons appearance, corpropraxia, corprolalia, spinning Associated with greater social difficulties,academic problems, and disruptivebehavior 23. Comorbidity: TS and OCD Obsessive or compulsive symptomsand/or behaviors suggested to occur innearly all patients Clinical OCD occurs in ~25% of TSpatients Can be difficult to differentiate complextics from compulsive behaviors e.g., touching something repeatedly until it feels just right 24. Course with ComorbiditiesJancovic, 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 socialsituations/Isolation Difficulties in school orworkplace Comorbid ADHD or otherdisorders increaseslikelihood 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 themto 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 payingattention 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 theenvironment distracting for classmates Classmates and sensory inputs can be distracting for childwith TS 28. Management and Treatment Multi-component management approachrecommended 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 anxietyreduction Habit Reversal Therapy based on the fact that tics arereduced during periods of great focus Hypnosis/Self Hypnosis Narrative Therapy to externalize tics 32. Treatment: Medication Simply having tics not indicator formedication Medication usually considered whensymptoms interfere with peerrelationships, social interactions,academic or job performance, oractivities of daily living No drug will entirely eliminate tics Unwanted side-effects may beworse than symptoms Goals is to relieve tic-relatedphysical discomfort orembarrassment and to achieve adegree of control of tics that allowsthe child to function as normally aspossible 33. Treatment: Medication Medication may be prescribed for tics,comorbid disorders or both Monotherapy ideal, but polypharmacycommon Most med use is off-label or notspecifically approved for children Controversy regarding whether ADHDtreatment with psychostimulantsexacerbates tics 34. Management: AcademicAccommodations Classroom accommodations Tic breaks Untimed tests Private room for test-taking Can make accommodationsunder 504 plan for anIndividual Education Plan (IEP) Semiformal classroompresentations or videos on TSto educate teacher andstudents 35. Management: Psychosocial and psychologicalsupports Provide information and assistance inaccessing support networks Address potential social impact (reducedself-esteem, self-consciousness) viapsychotherapy May benefit from social skill building 36. Treatment: Other Approaches Alternative approachessuch as fish oil/vitaminsupplements are beinginvestigated Dietary modification andallergy testing have beenexplored for ticmanagement but notsupported (allergies are astress to the body) High frequency Deep BrainStimulation (DBS) shownto be effective in smallnumber of cases (nochildren) 37. Prognosis In 50% of TS cases,symptoms will reduceor resolve by age 18. With proper educationand behavior changes,adults with TS canabsolutely live normallives.