Definition

• Motor and phonic ticks with onset in childhood

Pathophysiology

• Disturbance in cortico-striatal-cortical circuit aka mesolimbic

• Disinhibition of the motor and limbic system

• Imaging shows evidence of structural, functional and metabolic changes in the brain

• Reduction in caudate volume

• Changes in glucose usage rates in basal ganglia

• Insula plays a role

• No strong evidence PANDAS plays a role in developing TS

Genetics

• No causative genes identified

Epidemiology

• 1 in 200

• Men more than women

Clinical

• Tics

  • Sudden, brief, intermittent movements (motor)

  • Utterances (phonic)

  • Can be voluntarily suppressed

  • Most patients have comorbid ADHD or OCD

• Motor tics

  • Simple - blink, grimace, shrug, head jerk

  • Complex - Coordinated movements, bizarre gait, kicking, jumping

  • Tonic - Immobility, staring, prolonged contraction

  • Violent neck tics rarely cause injury

• Phonic tics

  • Simple - grunt, bark, moan, sniff, holler

  • Complex - words, comprolalia, repetition, palilalia

• Tic Character

  • Premonitory feelings or sensations

  • Relieved by execution of tic

  • Precipitory factors, temporary suppresssibility, variable seveity, waxing and waning nature, evolution of time

• Precipitating factors

  • Psychosocial stress, anxiety, anger, excitement, fatigue, and illness

• Onset

  • Between 2 and 15

• Natural History

  • Study in Denmark, 300 TS patients, followed for 6 years

  • Older than 16 years

    • 18% of patient tics had resolved

    • 60% had mild or minimal tics

    • 22% had moderate to severe

• Comorbidities in one study of 3500

  • ADHD in 60 %

    • Symptoms emerge 2-3 years before tics

  • OCD in 27%

    • Symptoms emerge 2-3 years after tics

  • Learning Disorder in 23%

  • Conduct disorder / ODD in 15%

  • Anxiety Disorders

    • Lifetime prevalence 30%

  • Mood Disorders

    • Lifetime prevalence of 30%

    • Includes depression

  • Sleep disorders

    • Common

    • Motor tics persist during sleep

  • Other

    • Increased risk of

      • Obesity

      • T2DM

      • CV disorders

History

• Review medical, social and family history for tics

Exam

• Usually normal neuro exam

Imaging

• Usually normal

Diagnosis

• Presence of multiple motor and phonic tics, onset before 18 or 21

Diagnostic criteria

• Both multiple motor tics and one or more phonic tics must be present at some time during the illness, although not necessarily concurrently

• Tics must occur many times a day, nearly every day, or intermittently throughout a period of more than one year

• Anatomic location, number, frequency, type, complexity, or severity of tics must change over time

• Onset of tics before the age of 21 years (the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition [DSM-5] criteria require onset of tics before age 18 years)

• Involuntary movements and noises must not be explained by another medical condition (or by the physiologic effects of substances as per the DSM-5 )

• Motor tics, phonic tics, or both must be witnessed by a reliable examiner at some point during the illness or be recorded by videotape or cinematography

Management

• Education

  • Comorbid conditions

  • Natural history

  • Family, peers, teachers, employers

• Tic management

  • Intervention is indicated when symptoms interfere with social interaction, school, or job performance or cause pain or injury

• Mild Tics

  • Education, counselling and supportive care

• Habit reversal training

  • Habit reversal training with Comprehensive Behavioural Intervention for Tics

  • Two parts

    • Tic awareness training

    • Completing-response training

• Pharmaceutical options

  • Guanfacine

  • Tetranenazine

  • Deuterbenazine

  • Aripiprazole

  • Fluphenazine

• Botox

  • For patients with only focal motor or phonic tics, or patients with violent neck extension tics, trial botox into the affected muscles

Resources:

• Tourette Association of America

• Tourette Canada

• Tourette Syndrome Association of Australia