Transcript 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 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.
"I Have Tourette's but
Tourette's Doesn't
Have Me"
premieres Saturday,
November 12, 2005
at 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: Characteristics
Simple
Motor
Phonic
Complex
Tics: Characteristics
Simple
Motor
Phonic
•“Meaningless”/isolated
•Facial and neck
•Abdomen
•Extremities
Complex
Tics: Characteristics
Simple
Motor
Phonic
•“Meaningless”/isolated
•Facial and neck
•Abdomen
•Extremities
Complex
•“Purposeful”
•Gestures
•Dystonic postures
•Self-abusive or
vulgar
Tics: Characteristics
Simple
Motor
Phonic
•“Meaningless”/isolated
•Facial and neck
•Abdomen
•Extremities
•“Meaningless”
•“Allergy”-like
•Grunting
•Tongue-clicking
•Animal noises
Complex
•“Purposeful”
•Gestures
•Dystonic postures
•Self-abusive or
vulgar
Tics: Characteristics
Simple
Motor
Phonic
•“Meaningless”/isolated
•Facial and neck
•Abdomen
•Extremities
•“Meaningless”
•“Allergy”-like
•Grunting
•Tongue-clicking
•Animal noises
Complex
•“Purposeful”
•Gestures
•Dystonic postures
•Self-abusive or
vulgar
•“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
midline
simple
→
→
→
caudal
peripheral
complex
Tic Disorders: Characteristics
• Premonitory urge
• Tics can usually be suppressed
.......W
W
A
A
X
E
N
E
S
S .......
Tourette’s Disorder
TM
• DSM-IV-TR 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 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
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
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
Effective?
YES
YES
NO
Recommend
Tolerate
Safe?
NO
Monitor closely
Discourage
or discourage
Source: Cohen MH & Eisenberg DM, Ann Intern Med (2002)
Integrative Medicine
websites
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)
DBS lead
Deep
Brain
Stimulation
Printed with permission, Medtronic
Extension
adjust
settings
Neurostimulator
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.
www.tsa-usa.org
Medical Education:
“Diagnosing and treating Tourette syndrome”
John Walkup, M.D.
Tourette Syndrome Association, Inc.
www.tsa-usa.org