Malady Wise

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Tourette Syndrome

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

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