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
