Dr. Alan Carroll MB, BCh, MRC Psych. FRCP(C) Psychiatrist, Clinical Professor of Psychiatry, University of Alberta Glenrose Rehabilitation Hospital

Dr. Alan Carroll MB, BCh, MRC Psych. FRCP(C) Psychiatrist, Clinical Professor of Psychiatry, University of Alberta Glenrose Rehabilitation Hospital

Introduction! Tics Description and Variation! Tourette Syndrome! Diagnosis DSM-5! Diagnostic Process! Clinical interview! Associated Symptoms! Differential Diagnosis! Treatment! Discussion/Critique! The future

Everything is extraordinary in this disease; the name is ridiculous, its symptoms peculiar, its character equivocal, its causes unknown, its treatment problematical.

Diagnosis of Tourette Syndrome DSM -5 Criteria (a) Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently. (b) The tics may wax and wane in frequency but have persisted for more than one year since first tic onset. (c) Onset is before age of 18 years. (d) The disturbance is not attributable to the physiological effects of a substance (e.g., cocaine) or another medical condition (e.g., Huntington s disease, post-viral encephalitis)

Motor Tics (Video Clip 1)

Video Clip: Student in Class

Vocal Tics! Sniffing! Throat clearing! Squealing! Barking! Swearing

Tics! Wax and Wane! Often made worse by :! stress! anxiety! excitement! fatigue! Illness! inflammation

Tourette Disorder

The Clinical Spectrum of Tourette Syndrome Symptomatic Developmental Physiologic TS Mild-Moderate TS with School & Social/Emotional Problems Severe TS with resistant tics

Developmental Disorder NERVOUS SYSTEM Balance

Pathogenesis of Tic Disorder Genetic & Environmental Factors Epi-Genetic Factors Neurobiologic al Substrates Clinical Phenotype

Epigene*cs

Environmental Factors! Stress in Pregnancy/Childbirth! Medications/Drugs! PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus)! PANS (Pediatric Autoimmune Neuropsychiatric Syndrome)! Toxins! Diet

Cortico-Striatal-Thalamic Cortical Circuits! Integrates Subsystems! a. Attention! b. Perception! c. Emotional! d. Motor control

Neurochemical Systems

Differential Diagnosis! Tics can occur in other neurological conditions and it is sometimes difficult to differentiate: Disorder Shared Symptoms Lab Tests Huntington's Disease Neuroacanthocytes Chorea, clonic, dystonic tics Mouth movement, lip biting, motor & phonic tics DNA Analysis >15% blood cells, creatine kinase Wilson s Disease Dystonia & Dystonic tics ceruloplasmin, copper in urine Kayser Fleischer Rings Sydenham's chorea Chorea tic-like movements Group A B haemolytic strep Drug Induced Motor and phonic tics Stimulant drug use cocaine Developmental Disorders Stereotypic movements, mannerisms Global developmental delay

TS Associated Disorders! Attention Deficit Hyperactivity Disorder! Executive Function! Obsessive-Compulsive Disorder (OCD)! Behavior Problems! Speech and Language Disorders! Learning Disability (LD)! Sleep Disorders

Frequency of Associated Symptoms in Tourette Disorder Frequency of Symptoms 70 60 50 40 30 20 10 0 Sleep Speech OC Disorder ODD Behvaior OC Behavior Executive Function ADHD Comorbid Condition

Obsessive-Compulsive Behavior

What are Executive Functions Executive Functions Definitions Example a. Response Inhibition Think before act Evaluate & Respond b. Emotional Control Manage emotions Disproportionate reaction Extreme Response Interrupt in class/reflex opposition Outbursts/Catastrophic c. Task Inhibition Get down to it Homework/Chores d. Planning/Organization Create roadmap to reach goal Priorities and timelines Young child needs prompts. Teenagers should not e. Sustained Attention Follow through to complete goal. Avoids distraction Homework/Classroom Inner & Outer distraction f. Flexibility Getting stuck/single-minded Transitions/Demands g. Working Memory Hold and utilize information Applied Learning h. Metacognition Self monitoring How am I doing Young - 1 or 2 pieces Older - many Young child/adult feedback Teenager independent

Treatment Approaches! Education - Individual, person and family. Many people are relieved and can accept the knowledge and can live with symptoms.! Management! Home! School! Peers! Therapies! Supportive - psychotherapy! Behavioral! Groups & Tourette Syndrome Association! Medications

Dr. Oliver Sacks

Practical Management! Sleep! Diet-Nutritional Status - Ferritin - Bowel issues - Food sensitivities allergies! Vitamin D! Exercise

Behavior Approaches

Medication Treatment Balance of Clinical Benefits vs. Harm 1. Alpha 2 Agonists! Clonidine! Guanfacine 2. 2 nd Generation Antipsychotics! Risperidone! Aripiprazole 3. Antipsychotics! Haldol! Fluphenazine! Pimozide

Other Physical Approaches! Botulinum toxin injections! Cannabis (Delta 9-THC)! Transcranial magnetic stimulation! Neurosurgery Deep Brain Stimulation

The Future! Immune system! PANS & PANDAS! Epigenetics! Inflammation

Stress affects:! Structure of the Brain! Circuits! Neurotransmitters and hormones! Long term physical & mental health

Genetics Genes are not our destiny! Epigenetics - gene expression switched on or off by events.

Human Microbiome 100 trillion organisms Out number our cells 10 to 1 Symbiotic the organisms are our friend

What does the Microbiome Do?! Breaks down complex carbohydrates! Produces vitamins and nutrients! Produces short chain fatty acids! Protects against pathogens! Supports detoxification! Modulates the nervous system! Appetite! Mood

Diet & Nutrition! Microbiome! Sensitivities! Leaky Gut

Leaky Gut

Inflammation! Involved in many aspects of mental health and mental diseases.! Markers are increase in ADHD

References! Whittington et al: Treatments for Tourette syndrome in Children and Adolescents A systemic review. Journal of Child Psychology & Psychiatry Vol. 57 Number 9 Sept 2016! Luca Pagharolo et al.: From Genetics to Epigenetics New Perspectives in Tourette Syndrome Research, Frontiers in Neuroscience 2016: 10: 277

References con t! European Guidelines for TS and other tics disorders.! Eur. Child Adolesc. Psychiatry, 2011, 20! Practice Parameters AACAP, Sept 2011! Canadian Guidelines for EB Treatment of TS., ed. Pringsheim, T.! Useful Websites! www.tourette.ca! www.tsa-usa.org

Recommended Books! International Review of Neurobiology: Advances in the Neurochemistry and Neuropharmacology of Tourette Syndrome, Martino, D., Cavanna, A., Vol 112, 2013! Tourette Syndrome, Martino, D., Leckman, J., 2013.

Questions?