Tics, Tourettes Rationale and DSM V Tics are the most common - - PowerPoint PPT Presentation

Tics, Tourette’s

and DSM V

Thomas K. Koch, MD

Credit Unions for Kids Professor of Pediatric Neurology

Tics, Tourette’s

and DSM V

Presented by

Faculty Disclosure Information

• A. I do not have any financial relationships with the manufactures of

any commercial product and/or provider of commercial services discussed in this CME activity: Thomas K. Koch, MD

• B. I do intend to discuss an unapproved / investigative use of a

commercial product / device in my presentation.

Tics in Childhood

Rationale

Tics are the most common movement disorder seen

in childhood.

Primary care providers are often reluctant to

diagnose Tourette’s without neurologic consultation.

Proper evaluation and recognition of the true tic

burden and any associated co-morbidities is essential for proper treatment.

Counseling and treatment discussions are critical in

the management of these patients and families.

Tics in Childhood

Objectives

Recognize and classify the common variety of tics

seen in children

Be comfortable with making the diagnosis of

Tourette Syndrome

Be able to properly discuss both the natural

history of tics and Tourette as well as pharmacologic and non-pharmocologic treatment

• ptions.

Tics in Childhood

Features

Common: 4% to 24% of all children

Most common movement disorder

Involuntary stereotypic repetitive

movements or vocalizations

May be transient or chronic

Tics in Childhood

Characteristics

Wax and Wane Exacerbated by stress, excitement, anxiety,

fatigue

Improve with rest, relaxation, concentration Usually absent during sleep but may be

present on polysomnograms

Briefly suppressible; build up of “inner

tension”

Often preceded by a premonitory urge or

“sensory tic”

Tic Classification

Simple Complex

Eye blinking Head twitching Facial grimacing Touching

Motor

Head thrusting Shoulder shrugging Smelling Jumping Mouth opening Echokinesis Copropraxia Sniffing Echolalia

Vocal

Snorting Coughing Palilalia Coprolalia Throat clearing Grunting Barking

Tic Syndromes in Children Provisional Tic Disorder (DSM V) Chronic Tic Disorder

Chronic Motor or Vocal Tics Tourette Syndrome

Nonspecific Tic Disorder

Secondary to Drugs (Stimulants) Assoc with Autistic Spectrum

PANDAS ?

Tic Syndromes in Children Provisional Tic Disorder

Most common

Duration < 1 year Single motor or vocal tic Rx usually not necessary

Tic Syndromes in Children

Chronic Tic Disorders (>1yr)

Chronic Multiple Motor or Vocal Tics Tourette Syndrome In 1885, George Gilles de la Tourette reported nine patients with chronic tic disorders characterised by involuntary motor and phonic tics.

Tourette Syndrome

Criteria (DSM V)

Onset < 18 yrs of age Multiple motor tics One or more vocal tic A waxing and waning course Duration > 1 yr Absence of medical explanation for tics

DMS V, 2013

Diagnostic Criteria

Tourette syndrome1 Tourette disorder 2

Onset : By age 21 By age 18 Motor tics:

multiple

Vocal tic

at least one

Course:

gradual; wax & wane

Duration:

> 1 year

Medications:

no tic provoking medications

Other:

not due to other disease

Witnessed: Observed

1 TS Classification Group 1993 2 DSM V

Tourette Syndrome

Clinical Facts

Worldwide distribution Prevalence: 1 per 1000 up to 3.5% of school age Inherited but probably more than one gene 3:1 male > female Onset: 6-7 yrs (mean)

Usually before adolescence

Usually begins with simple motor tic Increase with stress and anxiety Examination

Tics “Soft signs”

Tourette Syndrome

Clinical Course of Tics

Wax and Wane Maximum severity between 8-12 yrs Early severity = Later severity Prognosis: Most improve

26% resolved 46% diminished 14% stable 14% increased

Erenberg et al. Ann Neurol, 1987

72% Improve

Leckman JF et al. Pediatrics, 1998

Tourette Syndrome

Comorbidity

Obsessive Compulsive Disorders – 20-89% Attention Deficit Hyperactive Disorder – 50% Anxiety – 19-80% Mood Disorders - Depression – 30-40% Learning Difficulties – 20-30% Other

Impulsivity and aggression Substance Abuse

Tourette Syndrome

Comorbidity

Obsessive-Compulsive Behaviors

20-89% of TS patients Usually emerge after tics Usually obsessions or compulsions Associated with:

Impulsivity / Aggression Depression / Anxiety

Tourette Syndrome

Comorbidity

Attention Deficit-Hyperactive Disorder

50-60% of TS patients (21-90%) Generally begins before tics by 2-3 yrs Not associated with the tic severity Characterized by:

Impulsivity / Hyperactivity Poor attention

Tourette Syndrome

Genetic Epidemiology

Overall risk of TS in relatives is 10.7%

Male relatives – 17.7% Female relatives – 5.2%

Concordance rate for TS

MZ twins – 86% DZ twins - 20%

Tourette’s and the SLITRK1 gene

SLITRK1 is a Tourette gene

Only accounts for < 2% of TS patients

Other candidate genes:

Chromosome 17 Chromosome 8 Chromosome 2 Chromosome 11

Abelson JF, et al. Sequence variants in SLITRK1 are associated with Tourette’s syndrome. Science 2005;310:1-9.

What is the risk to my children if I have Tourette ?

Risk for TS – 10% Risk for a tic disorder – 30% Risk for OCD – 30% Risk for ADHD – 40% Risk for any of the three – 60% Higher risk if both parents have TS 75% for a tic disorder 50% for Tourette 95% for any of above

Tourette’s Syndromes

Neurobiology

Cortico-striatal-thalamocortical pathway Neurotransmitters

Dopamine GABA Glutamate Noradrenergic Serotonin Cholinergic Opioid

Tourette’s Syndromes

How to Rx – What to Rx

Tics ADHD OCD

Tic Treatments

Options

Education *** Behavioral approaches Pharmacotherapy Deep brain stimulation

Tic Treatment

Non-pharmacologic Therapy

Relaxation therapy Habit reversal training Acupuncture Biofeedback Hypnosis

Habit Reversal Therapy

Habit reversal training consists of two main

• components. These are:

Tic-awareness training, which teaches patients to

recognize early signs that precede the onset of a tic

Competing-response training, which teaches patients to

perform a voluntary movement that is incompatible with the particular type of tic

Behavior therapy for children with Tourette disorder: a randomized

controlled trial. Piacentini J, Woods DW, Scahill L, Wilhelm S, Peterson AL, Chang S, Ginsburg GS, Deckersbach T, Dziura J, Levi-Pearl S, Walkup JT. Results: Behavioral intervention led to a significantly greater decrease on the Yale Global Tic Severity Scale (24.7 [95% confidence interval, 23.1-26.3] to 17.1 [95% CI, 15.1-19.1]) from baseline to end point compared with the control treatment (24.6 [95% CI, 23.2-26.0] to 21.1 [95% CI, 19.2-23.0]) (P < .001; difference between groups, 4.1; 95% CI, 2.0-6.2). Significantly more children receiving behavioral intervention compared with those in the control group were rated as being very much improved or much improved on the Clinical Global Impressions–Improvement scale (52.5% vs 18.5%, respectively; P < .001; number needed to treat = 3). Attrition was low (12/126, or 9.5%); tic worsening was reported by 4% of children (5/126). Treatment gains were durable, with 87% of available responders to behavior therapy exhibiting continued benefit 6 months following treatment. Design, Setting, and Participants: Randomized, observer-blind, controlled trial of 126 children recruited from December 2004 through May 2007 and aged 9 through 17 years, with impairing Tourette

• r chronic tic disorder as a primary diagnosis, randomly assigned to 8 sessions during 10 weeks of

behavior therapy (n = 61) or a control treatment consisting of supportive therapy and education (n = 65). Responders received 3 monthly booster treatment sessions and were reassessed at 3 and 6

months following treatment.

Main Outcome Measures: Yale Global Tic Severity Scale (range 0-50, score >15 indicating clinically significant tics) and Clinical Global Impressions–Improvement Scale (range 1 [very much improved] to 8 [very much worse]).

• JAMA. 2010;303(19):1929-1937.

Tic Treatment

Pharmacotherapy – Non-neuroleptics

Generic Brand Dose Starting (mg/d) Dose Usual (mg/d) Clonidine Catapres 0.025-0.05 0.1-0.3 Guanfacine Tenex 0.25-0.5 0.5-3.0 Baclofen Lioresal 10-15 20-60 Clonazepam Topiramate Klonopin Topamax 0.025-0.5 15-25 0.5-3.0 100

Tic Treatment

Pharmacotherapy – Neuroleptics

Generic Brand Dose Starting (mg/d) Dose Usual (mg/d) Pimozide Orap 0.5-1 1-10 Risperidone Risperdal 0.25-0.5 0.5-3.0 Fluphenazine Prolixin 0.25-1.0 0.5-6 Olanzapine Zyprexa 2.5 2.5-10 Haloperidol Haldol 0.25-0.5 1-5

Tic Treatment

Pharmacotherapy – Others

Botulinum toxin delta-9-tetrahydrocannabinol Nicotine patch Tetrabenazine Ropinirole

Practical Points

■ Chronic tics are common ■ Do not assume tics are cause of disability ■ Ascertain comorbidities, and identify

impairment/disability

■ Pharmacologic therapy for tics should not be the default ■ Relatively low impact strategies are often sufficient ■ Education, stress reduction ■ Cognitive/behavioral intervention ■ Beware the early institution of neuroleptics ■ Therapy for comorbidities may help to ameliorate tics

Tics in Childhood

Suggested Changes: As a result of participating in this conference, I plan to apply the following changes in my practice:

Recognize and diagnose common motor and

vocal tics in children

Be comfortable in diagnosing Tourette Syndrome Be able to properly discuss both the natural

history of tics and Tourette as well as pharmacologic and non-pharmocologic treatment

• ptions.

References

• 1. Singer H. Tourette’s Syndrome: from behavior to biology.

Lancet Neurol 2005;4:149-159.

• 2. Shavitt RG, Hounie AG, et al. Tourette’s Syndrome.

Psychiatr Clin N Am 2006;29:471-486.

• 3. Abelson JF, Kwan KY, O’Roak BJ, et al. Sequence variants

in SLITRK1 are associated with Tourette’s syndrome. Science 2005;310:317-320.

• 4. Kadesjo B, Gillberg C. Tourette’s disorder: epidemiology

and comorbidity in primary school children. J Amer Acad Child Adolesc Psychiatry 2000;39:548-555.

• 5. http://tsa-usa.org
• 6. www.tourettesyndrome.net

Addendum

Supplemental Material

Tourette Syndrome - DSM-V

■ Both multiple motor and one or more vocal tics ■ Tics occur for over one year ■ Onset before 18 years ■ No other medical / neurologic condition

■ Changes with DSM-V ■ Tic-free intervals ■ Distress / Impairment ■ No more Transient Tic Disorder - now Provisional Tic

Disorder

Tourette’s Syndromes

How to Rx – What to Rx

Tics ADHD OCD

Clonidine Pimozide Risperdal Clonidine Guanfacine Stimulants Strattera SSRIs Clomipramine Clomipramine

Singer H. Tourette’s Syndrome: from behavior to biology. Lancet Neurol 2005;4:149-159.

Tourette’s Syndromes

Treatment

Attention Deficit-Hyperactive Disorder

Behavioral and educational approaches Pharmacologic Treatment

alpha-Adrenergic agonists

Central Stimulants (Ritalin, Dexedrine, Adderall) Atomoxetine (Strattera) Tricyclics

OCD Treatment

Pharmacotherapy

Generic Brand Dose Starting (mg/d) Dose Usual (mg/d) Fluoxetine Prozac 5-10 20-40 Sertraline Zoloft 25 75-200 Citalopram Celexa 10-20 20-60 Clomipramine Anafranil 25 50-200