TOURETTE SYNDROME Uttom Chowdhury Sept 19 th 2015

TS:What is a tic? A tic is an involuntary, rapid, recurrent, non- rhythmic motor or vocal action A tic is an involuntary, rapid, recurrent, non- rhythmic motor or vocal action It is sudden and purposeless It is sudden and purposeless Simple or complex Simple or complex Motor or vocal Motor or vocal

TS:Motor Symptoms Simple Motor tics: Simple Motor tics: eye blinking, eye rolling, facial grimacing, nose twitching, lip pouting, mouth opening, head nodding, head jerking, shoulder shrugging, arm jerking, abdominal tensing, etc Complex Motor tics: Complex Motor tics: hopping, jumping, touching objects, twirling, etc

TS:Vocal Tics Simple Vocal Tics: Simple Vocal Tics: throat clearing, grunting, barking, whistling, sniffing, etc Complex Vocal Tics: Complex Vocal Tics: phrases- ‘oh boy’, ‘shut up’ coprolalia and echolalia

TS:ICD 10 Transient Tic disorder Transient Tic disorder Chronic Motor or Vocal Tics Chronic Motor or Vocal Tics Combined Multiple Motor and Vocal Tics (Tourette Syndrome) Combined Multiple Motor and Vocal Tics (Tourette Syndrome)

TS:Tourette Syndrome Multiple motor tics and one or more vocal tics- not necessarily concurrently Multiple motor tics and one or more vocal tics- not necessarily concurrently Frequency must be many times a day, nearly every day, for more than one year, with no period of remission lasting longer than 2 months Frequency must be many times a day, nearly every day, for more than one year, with no period of remission lasting longer than 2 months Anatomical location, number, frequency, type, complexity or severity of tics change over time Anatomical location, number, frequency, type, complexity or severity of tics change over time Tourette Syndrome Classification Study Group (1993)

TS:Clinical Onset is mild and infrequent- age 7 Onset is mild and infrequent- age 7 Tics fluctuate in frequency and severity Tics fluctuate in frequency and severity ‘wax and wane’ ‘wax and wane’ Premonitory Sensory Urges-bodily sensations localised to discrete anatomical regions Premonitory Sensory Urges-bodily sensations localised to discrete anatomical regions Control over tics Control over tics

TS:Associated features ADHD (50-70%) ADHD (50-70%) OCD (30-60%) OCD (30-60%) Anxiety disorders Anxiety disorders Depression Depression

Timing of comorbidities

TS:Epidemiology Tics occur in 4-18 % children Tics occur in 4-18 % children Tourette Syndrome % in school based studies Tourette Syndrome % in school based studies Increased in LD population and Autism cohort Increased in LD population and Autism cohort

TS:Aetiology Genetics Genetics Neurochemistry Neurochemistry Neuroimaging Neuroimaging Infection Infection Peri-natal factors Peri-natal factors Learning model Learning model

TS:Assessment History: Tics: onset, nature, course, severity, impairment. OCD, ADHD Developmental Hx Medical Hx Family Hx School Hx Examination: Observed Tics

Differential Diagnosis Neurodevelopmental Disorders: OCD, ADHD, autistic spectrum disorders. OCD, ADHD, autistic spectrum disorders. Tics can occur secondary to a number of factors: Infections: Sydenham’s chorea (streptococcal infection), Creutzfeldt-Jakob disease, neurosyphilis and various forms of encephalitis. Infections: Sydenham’s chorea (streptococcal infection), Creutzfeldt-Jakob disease, neurosyphilis and various forms of encephalitis. Toxins: carbon monoxide poisoning, hypogycaemia. Toxins: carbon monoxide poisoning, hypogycaemia. Drugs: antipsychotics, antidepressants, lithium, stimulants (methylphenidate). Drugs: antipsychotics, antidepressants, lithium, stimulants (methylphenidate). Inherited disorders: Huntington’s disease, tuberose sclerosis, Wilson’s disease and chromosomal disorders such as Down syndrome, Klinefelters syndrome and Fragile X. Inherited disorders: Huntington’s disease, tuberose sclerosis, Wilson’s disease and chromosomal disorders such as Down syndrome, Klinefelters syndrome and Fragile X. Acquired causes: head injury and cerebrovascular accidents. Acquired causes: head injury and cerebrovascular accidents.

TS:Management Psycho-education Psycho-education Self esteem/anger/bullying/support Self esteem/anger/bullying/support School based School based Medical Medical Psychological Psychological OCD OCD ADHD ADHD

Medication When to medicate? Painful Tics Self Harm Tics Social Difficulties Functional Impairment

Medication Antipsychotics Antipsychotics Typical and Atypical Non-Antipsychotics Non-Antipsychotics

Antipsychotics in Treatment of Tics Medication Starting Dose (mg) Usual Dose (mg/day) Haloperidol* Pimozide* Risperidone* Fluphenazine Sulpiride Olanzapine Aripiprazole

Side-effects with Antipsychotics Neurological-parkinsonism, dystonia, akathisia, tardive dyskinesia Neurological-parkinsonism, dystonia, akathisia, tardive dyskinesia Prolonged QT interval Prolonged QT interval Depression Depression Weight gain Weight gain School phobia School phobia

Non-Antipsychotics Alpha-adrenergic agonist Clonidine Clonidine Guanfacine Guanfacine Side effects: sedation, dry mouth, headache, irritability, rebound hypertension on stopping abruptly

Other medication Pergolide-mixed dopamine agonist Pergolide-mixed dopamine agonist Botulinum toxin Botulinum toxin Tetrabenazine Tetrabenazine Baclofen-GABA B receptor agonist Baclofen-GABA B receptor agonist Nicotine Patch Nicotine Patch

Non-Antipsychotics in Treatment of Tics Medication Starting Dose (mg) Usual Dose (mg/day) Clonidine* Guanfacine* Pergolide* Botulinum Toxin units Tetrabenazine Baclofen Nicotine Patch 77-21

Other drugs aripiprazole aripiprazole Nicotine; mecamylamine Nicotine; mecamylamine Benzodiazepines, baclofen Benzodiazepines, baclofen Tetrabenazine Tetrabenazine Topiramate, Topiramate, Cannabinoids Cannabinoids Botulinum toxin Botulinum toxin Immunomodulation; penicillin prophylaxis Immunomodulation; penicillin prophylaxis Transcrainial magnetic stimulation Transcrainial magnetic stimulation Neurosurgery; deep brain stimulation Neurosurgery; deep brain stimulation Naloxone + other opioids Olanzapine Antiandrogen SSRIs Na Valproate levetiracetam Nifedipine

Psychological Treatments Massed Negative Practice Massed Negative Practice Operant Conditioning Operant Conditioning Relaxation Training Relaxation Training Exposure Response Prevention Exposure Response Prevention Habit Reversal Habit Reversal

Prognosis Retrospective data: Retrospective data: Peak tic severity age 10 Peak tic severity age 10 Generally repertoire of tics decreases during adulthood Generally repertoire of tics decreases during adulthood Small minority have severe, persistent tics Small minority have severe, persistent tics Rates of remission vary from 0-50% depending on study Rates of remission vary from 0-50% depending on study

OCD: What is OCD? Obsessions are thoughts, ideas, images or impulses that enter the persons mind repeatedly Obsessions are thoughts, ideas, images or impulses that enter the persons mind repeatedly Compulsions are stereotyped behaviour that is repeated again and again as a result of the obsessions Compulsions are stereotyped behaviour that is repeated again and again as a result of the obsessions

OCD:Obsessions CONTAMINATION CONTAMINATION AGGRESSIVE AGGRESSIVE SEXUAL SEXUAL HOARDING HOARDING MAGICAL THOUGHTS MAGICAL THOUGHTS SOMATIC SOMATIC RELIGIOUS RELIGIOUS

OCD:Compulsions WASHING/CLEANING WASHING/CLEANING CHECKING CHECKING REPEATING REPEATING COUNTING COUNTING ORDERING ORDERING ARRANGING ARRANGING HOARDING HOARDING

Symptom Dimensions 1234 AggressiveSymmetryContaminationHoarding SexualRepeatingCleaning ReligiousCounting SomaticSorting Checking

OCD:OCD AND TOURETTE SYNDROME Male Male Earlier age of onset of OCD symptoms Earlier age of onset of OCD symptoms Obsessions related to aggressive and sexual thoughts, compulsions related to touching, hoarding and counting rituals Obsessions related to aggressive and sexual thoughts, compulsions related to touching, hoarding and counting rituals OCD without tics tend to have more obsessions around contamination and cleaning OCD without tics tend to have more obsessions around contamination and cleaning Treatment-SSRI’s and neuroleptics Treatment-SSRI’s and neuroleptics

Do some patients with OCD and/or Tourette syndrome have PANDAS? In OCD and TS cohorts In OCD and TS cohorts Evidence of recent streptococcal infection Evidence of recent streptococcal infection Anti-basal ganglia antibodies Anti-basal ganglia antibodies

Treatment of post-streptococcal OCD/tics Now several population studies supporting the association Now several population studies supporting the association Children with abrupt onset OCD/tics should have a throat culture Children with abrupt onset OCD/tics should have a throat culture If positive they should have antibiotic treatment If positive they should have antibiotic treatment Clinical trials of antibiotic prophylaxis +ve and -ve Clinical trials of antibiotic prophylaxis +ve and -ve OCD and tics should be treated in the usual way OCD and tics should be treated in the usual way