Malady Wise

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Tourette Syndrome

Maladywise

Motor and phonic tics with childhood onset

Cause

  • Likely meso-limbic circuit issue - leading to disinhibition

  • Structural, functional, metabolic changes

    • Structure = Reduced caudate volume

    • PET shows basal ganglia glucose uptake changes

    • Insula plays role in premonitory urges

    • ? GAS contrbuting but no evidence PANDAS contributes

Genetics

  • No gene identified

Epidemiology

  • 1 in 200

  • M>F = 4:1

  • Prenatal smoking = more likely

Features

  • Motor tics

    • Simple = Blink, grimace, shrug, head jerk

    • Complex = Coord moves, gait, kick, jump, scratch

    • Dystonic = Oculogyric, sustain mouth opening, torticollis, body posture

    • Force neck tics rarely cause injury

  • Phonic tics

    • Simple = Grunt, bark, moaning, throat clearing, sniff, holler

    • Complex = Coprolalia (obscene words), echolalia (repetition of words), and palilalia (repetition of a phrase or word with increasing rapidity)

Character

  • Premonitory sensation, relived by execution of the tic

  • Precipitation factors

  • Temporary suppressibility

  • Variable severity

  • Waxing and waning

  • Evolution over time

Onset

  • Typical onset age 2 to 15 - average 6 years

  • 96% manifest by age 11

  • Tic severity peaks age 10 to 12

  • Improvement in adolescence and adulthood

  • 1/3 resolve completely, 1/3 improve, 1/3 continue

  • Risk of moderate to severe tics = 20 to 25%

  • Risk factor for moderate to severe = OCD, ADHD, family history of neuropsychiatric disorders

Comorbidities

  • ADHD in 30 to 60%

    • Symptoms emerge 2 to 3 years after onset of tics

  • OCD in 10 to 50%

    • Symptoms emerge a few years after onset of tics

    • More severe over time

    • Repetitive counting, arranging, hoarding, touching, tapping, rubbing, and error checking

  • Learning disorder in 23%

  • ODD in 15%

  • Self injury behaviour in 17%

  • No comorbidities in 12%

Evaluation

  • Neuro exam usually normal

  • Imaging usually normal

Differential

  • Transient tics of childhood in 25% of kids

Education

Algorithm


Management

  • Mild

    • Education, Supportive care, Watch

  • Debilitating

    • Habit reversal training

      • Moderate effects

    • Cognitive Behavioural Intervention for Tics

    • Medication

Medication

Alpha agonists

  • Guanfacine

  • Clonidine

    • 50mcg oral daily

    • Can titrate up to 300mcg in up to 4 divided doses (3 to 5mcg/kg total daily)

Antidopaminergic drugs

  • Haloperidol

  • Pimozide

  • Aripiprazole

VMAT2

  • Tetrabenazine

Topirimate

Botox