tourette's disorder and comorbidity

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 LearningSept 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.

"I Have Tourette's but Tourette's Doesn't

Have Me"premieres Saturday, November 12, 2005at 7:30 p.m. ET/PT

on HBO

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: CharacteristicsSimple Complex

Motor

Phonic


Motor

•“Meaningless”/isolated •Facial and neck•Abdomen•Extremities

Phonic


Motor


•“Purposeful”•Gestures•Dystonic postures•Self-abusive or vulgar

Phonic


Motor



Phonic

•“Meaningless”•“Allergy”-like•Grunting•Tongue-clicking•Animal noises


Motor



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-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

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

BrainRegionsinTS

With permission, NIMH

Differential Diagnosis of tics

• Compulsions• Habits• Stereotypies• Allergies• Sydenham chorea• Various involuntary neuromuscular

PANDAScontroversial

PediatricAutoimmuneNeuropsychiatricDisordersAssociated withStreptococcal infections

Genetics

• TS is genetic in origin• TS is inherited

– family, twin and adoption studies• Non-genetic factors also present

– Gestational exposure?– Perinatal?– Hormonal?

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

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 11-12• Peak tic severity age 10 to 11• Often see decrease in tics• Tics lifelong in 50% to 90%

Quality of Life?

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 11-12• 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

Managementparent 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

www.stopbullyingnow.hrsa.gov

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 Recommend Tolerate

NOMonitor closely or discourage Discourage

Effective?

Source: Cohen MH & Eisenberg DM, Ann Intern Med (2002)

Integrative Medicinewebsites

National Center for Complementary and Alternative Medicine

http://nccam.nih.gov

Consortium of Academic Health Centers for Integrative Medicine

www.imconsortium.org

Management:tics

• Experimental: Surgical– Deep Brain Stimulation (DBS)

DeepBrainStimulation

Printed with permission, Medtronic

DBS lead Extension

adjustsettings

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 AGood supportive evidence (short-term safety and efficacy)

Category BFair supportive evidence (short-term safety and efficacy)

Category CMinimal 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.

www.tsa-usa.org

Medical Education:“Diagnosing and treating Tourette syndrome”

John Walkup, M.D.

Tourette Syndrome Association, Inc.

www.tsa-usa.org

tourette's disorder and comorbidity

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