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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 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 • Rubbing eyes and blinking • Wiping/blowing nose until bleeds • Allergy medications not helping
Case 3 • 8-year-old boy • Deteriorating school performance • Disruptive in classroom • Recruits kids in noise-making antics • Moves about classroom
Case 4 • 7-year-old boy with possible otitis media • Severe lip chapping • Licking lips
Overview • Signs and symptoms • Associated problems • Management
Take Home Points: • TS not rare • Tics usually mild • Tics usually 1 of many related problems • Address main problems
Charcot & Tourette
Georges Albert Edouard BrutusGilles de la Tourette(1857-1904)
Georges Albert Edouard BrutusGilles de la Tourette(1857-1904) Childhood onset Premonitory sensation Heritable Motor & Vocal Coprolalila Echolalia Wax & Wane
Tic Disorders: Historical context • Psychological • Neurological • Neuropsychiatric • Neurology • Genetics & Environment • Behavioral & Functional
Tic Disorders: Characteristics • Tic Definition • motor or phonic • involuntary (unvoluntary?) • sudden and rapid • recurrent • non-rhythmic and stereotyped
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
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
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
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
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
Tics: Characteristics Anatomic evolution of tics top → bottom midline → peripheral simple → complex
Epidemiology • Prevalence • 1% males (or more) • Male > Female (3-to-10 times)
“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
Tics: Pathophysiology • Cortical & Subcortical network • Sensory • Affective • Motor
Tic Disorders: Characteristics • Premonitory urge • Tics can usually be suppressed
Etiology URGE → TIC → RELIEF
Tics:Pathophysiology • Dis-inhibition • “sensori-motor gating” • “filtering” • Motor programs • “fixed action patterns” • “muscle memory”
Brain Regions in TS With permission, NIMH
Basal Ganglia Striatum Striatum cortex GP / SN Thalamus brainstem
PANDAScontroversial Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections
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
Genetics • TS is genetic in origin • TS is inherited • family, twin and adoption studies • Non-genetic factors also present • Gestational exposure? • Perinatal? • Hormonal?