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 Conference on Early Learning Sept 24, 2007

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.

Overview Tics & associated problems Assessment Tic management (non-Rx) – Conventional – Experimental

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

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

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

Tics: Characteristics SimpleComplex Motor Phonic

Tics: Characteristics SimpleComplex Motor “Meaningless”/isolated Facial and neck Abdomen Extremities Phonic

Tics: Characteristics SimpleComplex Motor “Meaningless”/isolated Facial and neck Abdomen Extremities “Purposeful” Gestures Dystonic postures Self-abusive or vulgar Phonic

Tics: Characteristics SimpleComplex Motor “Meaningless”/isolated Facial and neck Abdomen Extremities “Purposeful” Gestures Dystonic postures Self-abusive or vulgar Phonic “Meaningless” “Allergy”-like Grunting Tongue-clicking Animal noises

Tics: Characteristics SimpleComplex Motor “Meaningless”/isolated Facial and neck Abdomen Extremities “Purposeful” Gestures Dystonic postures Self-abusive or vulgar Phonic “Meaningless” “Allergy”-like Grunting Tongue-clicking Animal noises “Linguistic” Syllables Words, obscenities Imitative (“echoic”) Speech atypicalities

Tics: Characteristics Fractal quality – Tics occur in bouts over: seconds minutes weeks months years

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

Tic Disorders: Characteristics Premonitory urge Tics can usually be suppressed

W A X E S W A N E S

Tourette’s Disorder DSM-IV-TR TM 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

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

Etiology Neuro-anatomy and function Neurotransmitters Genetics

“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

Brain Regions in TS With permission, NIMH

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

PANDAS controversial Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections

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

Genetics barriers 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

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

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

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

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

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

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

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)

Management General Guidelines – Education – Monitoring (tics and non-tics) – Containment

Identification Clinical aspects of tics Comorbid conditions Emotion and behavior

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

Identification – comorbid conditions Anxiety Disorders ADHD Learning Disorders Behavioral Disorders Developmental Disorders Mood Disorders

TRICHOTILLOMANIA: moth-eaten appearance to hair and scalp excoriations

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

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

Management Is additional treatment needed: – for tics? – for co-morbid conditions?

Management Perspectives: – The child – The parent – The school – You

Management parent perspective Most Important –Episodic rage –Attention deficit –Learning difficulties Least Important –Motor tics –Vocal tics

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

Management: tics Education & Accommodation Medications Experimental – Behavioral – Integrative – Surgical Advocacy

Management: tics Education & Accommodation – Teacher in-service – Classroom education – Teacher as role model – Tic breaks/sanctuaries

Management: tics Education & Accommodation – cont. – Testing accommodations – Opportunities for movement – Scribes – Bullying

Bullying Stop Bullying Now – HRSA

Management: tics Experimental: Behavioral –CBIT (Comprehensive Behavioral Intervention - Tics) HRT (Habit Reversal Training) »Awareness Training »Competing Response »Relaxation »Social Support FA (Functional Analysis) »Social situations that influence behaviors

Management: tics Experimental: Integrative – Complementary – Alternative – Holistic

Management: tics Experimental: Integrative – cont. – Six categories Medical Nutritional Foreign substances Behavioral and cognitive Manual and energy medicine Mind-Body

A common sense guide to complementary/alternative medicine Safe? YES NO YES RecommendTolerate NO Monitor closely or discourage Discourage Effective? Source: Cohen MH & Eisenberg DM, Ann Intern Med (2002)

Integrative Medicine websites National Center for Complementary and Alternative Medicine Consortium of Academic Health Centers for Integrative Medicine

Management: tics Experimental: Surgical – Deep Brain Stimulation (DBS)

Deep Brain Stimulation Printed with permission, Medtronic DBS lead Extension adjust settings Neuro- stimulator

Management: Advocacy and Legal Rights Tourette Syndrome Association Protection and Advocacy office IDEA Section 504

Pharmacotherapy for Comorbid Conditions KEY POINT! Target the most troubling symptoms

Pharmacotherapy KEY POINTS! Do not assume medication is necessary Address comorbid condition(s) Complete tic remission is rare Stimulants are generally safe

Pharmacotherapy  International Psychopharmacology Algorithm Project  Category A  Good supportive evidence (short-term safety and efficacy)  Category B  Fair supportive evidence (short-term safety and efficacy)  Category C  Minimal supportive evidence (short-term safety and efficacy)

Take Home Points: Clarifying Common Misconceptions 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

For further information, including Rx discussion: Tourette Syndrome Association, Inc. Medical Education: “Diagnosing and treating Tourette syndrome” John Walkup, M.D.

Tourette Syndrome Association, Inc.