Post on 28-Jan-2017
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
Tics: CharacteristicsSimple Complex
Motor
•“Meaningless”/isolated •Facial and neck•Abdomen•Extremities
Phonic
Tics: CharacteristicsSimple Complex
Motor
•“Meaningless”/isolated •Facial and neck•Abdomen•Extremities
•“Purposeful”•Gestures•Dystonic postures•Self-abusive or vulgar
Phonic
Tics: CharacteristicsSimple Complex
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: CharacteristicsSimple Complex
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-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