Tourette Syndrome: Tackling a noisy tic disorder (with just a whisper about medication)

1. Tourette Syndrome:Tackling a noisy tic disorder(with just a whisper about medication) Samuel H. Zinner, M.D. Assistant Professor of Pediatrics & Developmental-Behavioral Pediatrician University of Washington, Seattle http://depts.washington.edu/dbpeds Conference on Early Learning Sept 24, 2007

2. Tourette Syndrome:Tackling a noisy tic disorder(with just a whisper about medication) Samuel H. Zinner, M.D. discloses no relevant financial relationships with any commercial interests. This presentation will reference unlabeled/unapproved uses of medications and products, and will be identified as such.

4. Overview • Tics & associated problems • Assessment • Tic management (non-Rx) • Conventional • Experimental

5. Take Home Points: • TS is not rare • Tics are usually mild, not catastrophic • In most people with TS, tics are one of many related complications • Address main problems, often not tics

6. Who cares about Tourette syndrome? • TS is: • common • under-diagnosed • misunderstood • ripe with opportunity for management (and mismanagement) & research

7. Tic Disorders: Characteristics • Tic Definition • motor or phonic • involuntary (unvoluntary?) • sudden and rapid • recurrent • non-rhythmic and stereotyped

8. Tics: Characteristics

9. Tics: Characteristics

10. Tics: Characteristics

11. Tics: Characteristics

12. Tics: Characteristics

13. Tics: Characteristics • Fractal quality • Tics occur in bouts over: • seconds • minutes • weeks • months • years

15. Tics: Characteristics Anatomic evolution of tics rostral → caudal midline → peripheral simple → complex

16. Tic Disorders: Characteristics • Premonitory urge • Tics can usually be suppressed

18. . . . . . . . W A X E SW A N E S . . . . . . .

21. Tourette’s Disorder • DSM-IV-TRTM Criteria • Multiple motor + 1 or more vocal • Many times/day & at least 1 year • Onset before 18 years • Not due to substance or medical condition

22. Epidemiology • “Official” prevalence • 1 in 1,000 boys • 1 in 5,000 girls • Actual prevalence • 1 in 100 boys (or even higher)

23. Etiology • Neuro-anatomy and function • Neurotransmitters • Genetics

24. “If the brain were simple enough that we could understand it, we’d be so simple that we couldn’t” Paul Greengard, Ph.D. Nobel Prize in Physiology or Medicine 2000

25. Brain Regions in TS With permission, NIMH

26. Differential Diagnosis of tics • Compulsions • Habits • Stereotypies • Allergies • Sydenham chorea • Various involuntary neuromuscular

27. PANDAScontroversial Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections

28. Genetics • TS is genetic in origin • TS is inherited • family, twin and adoption studies • Non-genetic factors also present • Gestational exposure? • Perinatal? • Hormonal?

29. Geneticsbarriers to identifying genes • Diagnosis based on behaviors • Defining the TS phenotypic spectrum • “endophenotypes” • Family pedigree problems • Environmental influences • Combinations of genes may be involved • Symptoms decrease with age • Transient tics

30. Differential Diagnosis of tics • Sydenham’s chorea • Compulsions • Blepharospasm • Other hyperkinetic disorders • Stereotypies • Allergies

31. Diagnostic Pitfalls 101 • Subject or clinician unaware of tics • Waxing and waning nature of tics • Tics are suppressible

32. Diagnostic Pitfalls 102 • T.S. is not rare • T.S. is usually not catastrophic • Few have coprolalia • You may not see the tics

33. Assessment:co-morbid conditions • ADHD • Obsessions/Compulsions • Learning interferences • Behavioral disorders • Developmental disorders • Mood disorders • Anxiety • Social difficulties (including PDDs)

34. Assessment:co-morbid conditions and tics Lumpers vs. Splitters

35. Clinical Course • Hyperactivity often precedes tics • Head and neck tic onset age 6 to 7 • Vocal tics age 8 to 9 • Obsessive-Compulsive symptoms 11-12 • Peak tic severity age 10 to 11 • Often see decrease in tics • Tics lifelong in 50% to 90%

36. Quality of Life?

37. Quality of Life? “Tourette differs from other neuropsychiatric disorders in one simple way: It is largely the disease of the onlooker. When I tic, I am usually not the problem. You are.” Peter Hollenbeck, Ph.D. (a neuroscientist with TS) -Cerebrum (2003)

38. Management • General Guidelines • Education • Monitoring (tics and non-tics) • Containment

39. Identification • Clinical aspects of tics • Comorbid conditions • Emotion and behavior

40. Identification – comorbid conditions KEY POINT! Always assess for non-tic comorbidity * 90% occurrence if tics mild * 100% occurrence if tics severe *in clinically-referred samples

41. Identification – comorbid conditions • Anxiety Disorders • ADHD • Learning Disorders • Behavioral Disorders • Developmental Disorders • Mood Disorders

43. TRICHOTILLOMANIA: moth-eaten appearance to hair and scalp excoriations

44. David Sedaris a plague of tics from “Naked” Little, Brown and Company, 1997

45. Clinical Course • Hyperactivity often precedes tics • Head and neck tic onset age 6 to 7 • Vocal tics age 8 to 9 • Obsessive-Compulsive symptoms 11-12 • Peak tic severity age 10 to 11 • Often see decrease in tics • Tics lifelong in 50% to 90%

46. Management • Is additional treatment needed: • for tics? • for co-morbid conditions?

47. Management • Perspectives: • The child • The parent • The school • You

48. Managementparent perspective • Most Important • Episodic rage • Attention deficit • Learning difficulties • Least Important • Motor tics • Vocal tics

49. Management:“co-morbid” conditions • OCD & other anxiety disorders • ADHD • Learning difficulties • Behavioral Disorders • Sleep disturbances • Other self-injurious behaviors • Family dysfunction

50. Management: tics • Education & Accommodation • Medications • Experimental • Behavioral • Integrative • Surgical • Advocacy