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Tourette Syndrome: The Whole Tic and Kaboodle. Tourette Syndrome Association, Inc. & CDC Samuel H. Zinner, M.D. Associate Professor of Pediatrics University of Washington, Seattle depts.washington.edu/dbpeds December 15, 2012. Case 1. 10-year-old boy “Not himself” past year

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Tourette Syndrome: The Whole Tic and Kaboodle


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    1. Tourette Syndrome:The Whole Tic and Kaboodle Tourette Syndrome Association, Inc. & CDC Samuel H. Zinner, M.D. Associate Professor of Pediatrics University of Washington, Seattle depts.washington.edu/dbpeds December 15, 2012

    2. Case 1 • 10-year-old boy • “Not himself” past year • Rubbing eyes and blinking • Wiping/blowing nose until bleeds • Allergy medications not helping

    3. Case 3 • 8-year-old boy • Deteriorating school performance • Disruptive in classroom • Recruits kids in noise-making antics • Moves about classroom

    4. Case 4 • 7-year-old boy with possible otitis media • Severe lip chapping • Licking lips

    5. Overview • Signs and symptoms • Associated problems • Management

    6. Take Home Points: • TS not rare • Tics usually mild • Tics usually 1 of many related problems • Address main problems

    7. Historical timeline of Tourette syndrome events

    8. Charcot & Tourette

    9. Georges Albert Edouard BrutusGilles de la Tourette(1857-1904)

    10. Georges Albert Edouard BrutusGilles de la Tourette(1857-1904) Childhood onset Premonitory sensation Heritable Motor & Vocal Coprolalila Echolalia Wax & Wane

    11. Eiffel Tower erected in Paris1889

    12. Tic Disorders: Historical context • Psychological • Neurological • Neuropsychiatric • Neurology • Genetics & Environment • Behavioral & Functional

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

    14. Tics: Characteristics

    15. Tics: Characteristics

    16. Tics: Characteristics

    17. Tics: Characteristics

    18. Tics: Characteristics

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

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

    21. Chronic Tic Disorder (M or V) • DSM-IV-TRTM Criteria • Multiple (or single) motor or vocal • Many times/day and at least 1 year • Onset before 18 years • Not due to substance or medical condition

    22. Transient Tic Disorder • DSM-IV-TRTM Criteria • Multiple(&/orsingle) M.&/orV. • Many times/day(4 weeks – 1 year) • Onset before 18 years • Not due to substance or medical condition

    23. Tourette’s Disorder • DSM-V • Duration criterion for chronic tics • Tics persist for > 1 yr since first tic onset • Changes from DSM-IV-TR. Removed: • More than 9/12 months of any year • Tic-free period of no more than 3 months • Transient Tic Disorder • Provisional tic disorder

    24. Tourette’s Disorder • DSM-V • Duration criterion for chronic tics • Tics persist for > 1 yr since first tic onset • Changes from DSM-IV-TR. Removed: • More than 9/12 months of any year • Tic-free period of no more than 3 months • Transient Tic Disorder • Provisional tic disorder

    25. PREMONITORY URGE

    26. Tics: Characteristics Anatomic evolution of tics top → bottom midline → peripheral simple → complex

    27. Anatomic evolution of tics

    28. Anatomic evolution of tics

    29. Anatomic evolution of tics

    30. Anatomic evolution of tics

    31. Anatomic evolution of tics

    32. Anatomic evolution of tics

    33. Anatomic evolution of tics

    34. Epidemiology • Prevalence • 1% males (or more) • Male > Female (3-to-10 times)

    35. “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

    36. Tics: Pathophysiology • Cortical & Subcortical network • Sensory • Affective • Motor

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

    38. Etiology URGE → TIC → RELIEF

    39. Tics:Pathophysiology • Dis-inhibition • “sensori-motor gating” • “filtering” • Motor programs • “fixed action patterns” • “muscle memory”

    40. Brain Regions in TS With permission, NIMH

    41. Basal Ganglia Striatum Striatum cortex GP / SN Thalamus brainstem

    42. PANDAScontroversial Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections

    43. PANDAS5 identifying criteriadeveloped for research by clinical observation • Dramatic emergence or exacerbation of OCD and/or tics • Pre-pubertal symptom onset • Other neurological signs • Association with GABHS • Episodic or sawtooth symptom course

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