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& Tourette’s Infomed

Giles Elrington [email protected] ’s & Tourettes Infomed

Giles Elrington [email protected] Definitions Cavanna AE, Sero S BMJ 2013;347:f4964 • Tics: involuntary, sudden, rapid, recurrent, non-rhythmic movements (motor tics) and vocalisations (vocal or phonic tics).

• Tourette’s syndrome: the chronic presence of at least two motor tics and one vocal tic since childhood.

Tourette’s prevalence

• 0.3-1% of schoolchildren affected • 200 000-330 000 people affected in UK • Peak severity in early teens • 2/3 improve by adulthood • Not associated with • 10% no associated psychiatric comorbidity • 60% have OCD or ADHD • Personality disorder over-represented OCD symptoms

GTS Primary OCD • Symmetry concerns • Cleaning rituals • “Evening –up” behaviours • Compulsive washing • Obsessional counting • Fears of contamination • “Just right” perceptions &

• 10% of GTS patients • 20-30% in specialist clinics • Not required for diagnosis • Cultural specificity Famous OCD, tic or TS sufferers

• Dr • Howard Hughes • Dan Ackroyd • David Beckham • W A Mozart What was Gilles de la Tourette’s given name?

• Gilles • Georges • Albert • Edouard • Brutus • All of the above

Classification of tics 1

Type of tic Examples

Simple motor tics Blinking; grimacing; shrugging; tongue protruding; contracting platysma, rectus abdominis or glutei Complex motor tics Head shaking; shoulder rolling; touching; jumping; squatting; spitting; ; copropraxia Simple vocal tics Sniffing; grunting; throat clearing; coughing; humming; yelping; barking

Complex vocal tics Coprolalia; ; Classification of tics 2

Type of tic Examples

Sensory Sensation usually within body part, can be perceived as external, may be relieved by movement

Mental Repetitive thoughts resembling echolalia, palilalia & coprolalia but not verbalised; may be described as mental images of the word or phrase Causes of tics 1: idiopathic

• Transient – Motor or vocal (not both) – 1-12 month duration – Onset <21 years of age – No secondary cause • Chronic motor or vocal – As above (transient) but >12 months – Adult variants can occur • Gilles de la Tourette’s syndrome – Multiple motor plus 1 or more vocal tic – Onset normally <21 years of age Causes of tics 2: secondary

• Specific neurodegenerative – Huntington’s – • Associated with neurodevelopmental disorders – Learning disability (including chromosomal), , • Following acute brain injury – (lethargica) – Sydenhams , TBI, CO poisoning, hypoglycaemia • Related to drug exposure – Stimulants (including & ) – Levodopa – Neuroleptics (“tardive ”) – , , barbiturates – Quiniolone antibiotics Diagnostic approach to a child with a single tic • Masterly inactivity – Common esp in boys – most resolve naturally • Chronic tic:- – Low threshold for Wilson’s disease screen – Motor & vocal: consider GDT syndrome Diagnostic approach motor & vocal tics • Differentiate tic from chorea or • Consider – neuroleptic exposure – Brain injury – CO poisoning • Look for FH other than GDT or OCD • Exclude – Huntington’s & neuroacanthocytosis – Encephalitis Brain imaging in Tourette’s

• No clear consensus on abnormalities • Wide range incidental findings • Possible loss normal slight asymmetry caudate • PET & fMRI research tools only Lab studies in Tourette’s

• Genetics – Twin & family studies suggest major genetic component – Genetic studies limited by phenotype definition • Immunology – Overlap with PANDAS & Sydenham’s chorea – antibodies low specificity Management of GTS

• Specialist referral for all patients • Behavioural • Medication – pimozide – aripiprazole risperidone sulpiride tetrabenazine clonidine topiramate – SSRI for OCD – botulinum toxin for tic – not benzos • Surgery – deep brain stimulation