Tics and Tourette Syndrome

1. Tics and Tourette Syndrome Tom Moran Consultant Child Psychiatrist

2. Outline of presentation History Definitions Prevalence co-morbidity Aetiological contributions Assessment Intervention but not Behavioural/educational

3. History In 1825 French Physician Jean Itard described the Marquise de Dampierre�s affliction with tics He also described 10 other cases

4. Georges Gilles de la Tourette

5. George Gilles de la Tourette Born near Poitiers Studied medicine at Poitiers Salpetriere, Paris under Charcot Contemporary of Pinel, Babinski, Freud Studied hypnotism with Charcot Studied Maladie de Tic Distinct from hysteria and other choreiform disorders Clinical description of 9 cases of tics in 1885 Named GdlTS by Charcot

6. Tic Tics are sudden repetitive movements or utterances that typically mimic some fragment of normal behaviour involving discrete muscle groups

7. TIC DISORDERS Transient Tic Disorder DSM IV Single or multiple motor+/or vocal tics May come and go Occur often, lasting 4 weeks but not longer than 12 months, and may recur over years Onset before 18 No general medical or substance causation

8. Transient tic Tend to affect the head and neck Mainly motor in nature Have an onset between 3-10 years Boys more than girls May have a number over the years May go unnoticed If no FH Tic or OCD will fade within 1 year (Coffey)

9. TIC DISORDERS Chronic motor or Vocal Tic DSM IV Single or multiple motor or vocal tics but not both Tend not to change in form and persist Occur often and persist beyond one year Onset before 18

10. Chronic tic disorder May take the form of chronic blinking

11. TIC DISORDERS Tourette's Syndrome Multiple motor and one or more vocal tics though not necessarily concurrently Tics occurs often, usually in bouts and change over time Onset before 18 and lasting more than 1 year with no tic-free period >3 months Not due to an other medical condition

12. Range of TS Symptoms MOTOR Simple Tics: fast, darting, meaningless Complex Tics: slower, may be purposeful (includes copro and echopraxia) VOCAL Simple Tics: meaningless noises, sounds, sniffs Complex Tics: words, phrases( includes copro, echo and palilalia) sudden changes in pitch and volume

13. Motor Symptoms Simple Motor Tics Eye blinking, grimacing, lip pouting, shrugs, abdominal tensing, finger movements If no history of simple tics then the diagnosis of TS may be in doubt

14. Motor symptoms Complex Motor Tics Hopping, clapping, touching (self & others) twirling, picking scabs, rolling eyes, kissing, tearing paper They are tics because they appear repeatedly and in bouts Difficult to distinguish from compulsion

15. Motor Symptoms Copropraxia Obscene gestures, grabbing genitals Echopraxia Imitating gestures, movements of others

16. Vocal tics They are rare in other neurological disorders except Huntington�s Sydenham�s (a complication of Strep infections still common is St America)

17. Vocal Symptoms Simple Vocal Cough, spit, grunt, hiss, whistle, �uh�, �eee� Is sniffing a motor or vocal tic? Is sound production the key?

18. Vocal Tics Complex Vocal �Oh boy�, �you know�, �you�re fat�

19. Vocal Tics Rituals Repeating phrases until �Just right� or a fixed number of times Speech Atypicalities Unusual rhythms, tone, pitch, speed

20. Vocal Tics Coprolalia Obscene, aggressive or other socially unacceptable words or phrases Culture specific Palilalia Repeating own words or parts of words Echolalia Repeating others� words, phrases

21. Tic-related mental states An �urge�, often intense, frequently reported after age 10 years It may be akin to what precedes a sneeze Others may have more intense mental images Many people are very sensitive to changes in the sensory world, internal and external: Site sensitivity (clothes tags) Trigger perceptions( noises, certain people�s voices) Disinhibition( urge to touch hot or dangerous objects)

22. Tic Repertoire The range of tics varies enormously Each person�s repertoire is different They are fragments of normal behaviour They occur in bouts with inter-tic interval of 0.5-1.0 seconds Bout of bouts is not unusual Waxing and waning may vary from weeks to months often aggravated by stress Many feel exhausted by the cycle of particular tics

23. Tic Control �bouts of involuntary movements of which the individual is unaware� is an inadequate description Premonitory urges experienced by many Capacity to suppress for periods. �I shake my head� v �my head shakes�

24. Tics and sleep Originally thought not to occurs Now found in all stages of sleep Rothberger: Advances neurology 2001 Sleep problems in 25% clinic pop

25. Factors affecting tics Increase stress Anxiety Excitement Fatigue ?viruses Decrease Activities that require focussed attention and fine motor control ( video games even surgery!)

26. Range of associated TS Conditions Behavioural and Developmental ADHD OCB/D Emotional Lability Meltdowns SIB Social Learning disabilities

27. Non-obscene Complex Socially inappropriate behaviour (NOSI) Roger Kurlan 87 adolescents and adults insulting others 22% socially inappropriate comments 05% socially inappropriate actions 14% Directed at Family 31% Directed at Familiar person 36% Directed at stranger 17% NOSI ? Related to impulse control or obsession

28. Subdivisions of TS Robertson Pure TS motor and vocal tics Full blow TS copro phenomena TS-plus ADHD, OCB, SIB There may be different underlying mechanisms for these.

29. Natural History of TS Usual onset for motor tics 5-6 years Waxing and waning Changing repertoire Coprolalia in 10% general population, 30% clinic population

30. Natural History Most Important Worst period 7 �15 years of age (Leckman) By 18 years 50% virtually tic-free (Robertson)

31. Prevalence of TS Tics 10% before age 10 TS 1% in children 5-8 years old Based on the analysis of others� figures (Robertson)

32. prevalence Increased in Special Ed populations 7% (Eapen, Kurlan) In People with an Autistic Spectrum Disorder 6% (Baron Cohen) 4.6% of 7288 TS cohort had some difficulties on the Autistic Spectrum (Freeman 09)

33. prevalence Much depends on: the age group studied The definition (+/- impairment) The populations studied The study method

34. Prevalence TS Alan Apter (Israel) 28,000 army recruits aged 16-18 screened 4.3/100,000

35. Sex Distribution Boy: Girl 3:1

36. Cultural Distribution It occurs in all cultures Clinical characteristics are similar It is very rare in sub Saharan Africa and fewer reports in African Americans ? reason

37. Associated Problems with TS ADHD It occurs in 40-90% of the child population with TS Usually precedes the onset of tics May cause greater impairment than tics Severity of ADHD may be more predictive of social impairment

38. Associated problems with TS OCB/D Occurs in 11-80% Emerges after the onset of tics Compulsions come first May be difficult to differentiate between a complex tic and a compulsion �A tic is more of an itch, a compulsion more of a want. A tic is physical, a compulsion a mental feeling.�

39. Associated Problems with TS OCB/D

40. Associated problems with TS Depression No evidence for genetic link (Robertson) It�s multfactorial Secondary disability Stigma

41. Aetiology Psychological theory dismissed Large families with affected members suggested it was familial Genetic underpinnings are not understood Regions on many chromosomes are suggested to be implicated

42. Aetiology Leckman Group Link with: Severe Nausea in first trimester Psychological stress on the mother in pregnancy Increased use of coffee, cigs and alcohol ID twin with lower weight Hypoxia and interventions in labour Low apgar

43. genetics Chronic Tic or TS MZ Twins 77% concordance TS/Ch Tic DZ Twins 23%

44. TS Risk Mother/Father has TS from family with TS

45. TS Risk If neither parent has TS in TS spectrum family 2nd degree relative (grand-child, niece/nephew) Half the risk if a parent had TS

46. neuroanatomy Basal ganglia A group of structures linked to frontal lobes and cerebral cortex Responsible for Implementing movement Procedural memory (bike, car) Regulating muscle tone

47. Basal ganglia Caudate nuclei most implicated part of BG Smaller in TS children and adults

48. Motor movement involves: selection of desired movement blocking of opposing movements and of similar movement in adjoining body parts. Facilitated by Basal Ganglia BG apply the brake Tics linked to a defect in the braking system Basal Ganglia

49. aetiology PANDAS Model Paediatric Autoimune Neurophsychiatric Disorders Assoc with Strep infection OCD and/or tic disorder Onset 3-12 years Choreiform movements Acute abrupt onset Time link with Group A b-haemolytic strep Episodic course v chronic in TS Postulated post infectious auto-immune mechanism Swedo 1998 Am J Psych

50. aetiology . Strep not causative but individuals may inherit a susceptibility both to TS and the way they react to some infections (Robertson)

51. Current view Treat proven strep throats with PCN If Tics or OCD are impairing use conventional treatments Antibiotic prophylaxis not recommended

52. aetiology Neurochemistry Several systems implicated by Neurochemical measurement Neuroimaging studies Response to certain medications

53. neurochemistry Dopamine Response to early medications haloperidol and some post mortem findings Noradrenaline Response to clonidine and gauafacine Noradrenaline release by stress, excitement

54. neurochemistry Serotonin Response of OCD to SRIs GABA (the braking chemical) GABA and DA system linked Tics respond to Clonazepam (GABAergic)

55. Referral for Treatment to CAMHS Predictors of referral Activity level Angry outbursts Behaviour management Socialising difficulty A minority need treatment for TICs

56. Assessment The key is a detailed history from multiple sources Clarify co-morbidity Mental State Neurological exam National Hospital Interview Schedule The Yale Global Tic Severity Scale We don�t rely on what you see in clinic: observe in the waiting area

57. Assessment The child as an individual Clarify strengths Begin to explore the waxing/waning Clarify the degree of interference in various domains What makes tics worse including �excitement�, fatigue Do this over time It�s important that the child and parent become observers

58. Assessment Impact at school Cognitive function and achievement Attention problems Penmanship Compulsions (crossing t�s etc)

59. Investigation Nil from laboratory Nil from MRI

60. Treatment of TS Does a diagnosis help? Meaning of diagnosis Monitoring Education about the condition Recognise waxing/waning/aggravating factors Do no harm �Hand holding� through crises No parent wants to see a child distressed

61. treatment Intervention at school Information School meeting CD Rom Educating the class Special needs assistant / resource hours Special arrangement for exam

62. Treatment of TS Qs we think about with families Do I treat? What symptoms do I treat? Do they bother you? Do they bother others? Do they prevent you from doing things? Low dose to gain �control� rather than cure What worked for others in the family?

63. Pharmacotherapy Target symptoms Tics ADHD O-C Symptoms

64. tics % improvement for medication showing superiority to placebo in treatment of tics

65. CLONIDINE DOSE Coffey Start at 0.025mg daily and increase by 0.025mg every 1-2 weeks Pre-pubertal children need TID/QID dosing Start 0.05mg and increase by 0.05mg to BD dosing Max 8.0 microgms/kg/day

66. Clonidine S.E Sedation @ 60 minutes. Will ease Headache, stomach aches Dizziness secondary to lwr BP Monitor BP and ECG Beware abrupt withdrawal

67. Antipsychotics used in treatment of tics

68. Severe Tics Atypical Neuroleptics Starting dose of Risperidone for pre-pubertal children: 0.125-0.25mg nocte increasing weekly to 0.5-1.5mg BD 1-3mg for adolescents

69. Atypical Side Effects Monitor Weight, BMI, blood sugar, prolactin, liver function and CVS

70. Treatment Habit Reversal Awareness Training Describe tics Practice early self detection Identify situations

71. TS + ADHD Mild moderate TS when ADHD is most significant Stimulant with caution Low dose 2.5mg MPH Atomoxetine

72. Omega 3 fatty Acids Pro-serotonergic and anti-inflammatory 20 week double-blind placebo controlled Omega-3 EPA/DHA (2:1) (vanilla) 500-6000mg Placebo Olive Oil (vanilla) Looking for improvement above usual meds

73. Omega -3 34 children and adolescents enrolled eventually Improvement in YGTSS and CY-BOCS in both placebo and treatment groups No significant difference Problems ? Impact of current treatment ?natural history ?impact of the olive oil Needs replication in treatment naive groups No contra indications

74. Pharmacotherapy for TS Explosive Vocal Tics Botox injections of the vocal cords every 3 months Botox also used for other muscle groups with good effect

75. treatment Botulinum Injected into discrete muscle groups Blocks Acetylcholine release at neuromuscular junction Vocal tics: eye blinking: some dystonic tics Marras. Neurology 2001

76. Drug treatment for Tics Duration of treatment The long term value is not clear Most trials are 6-12 weeks Should treatment be short-term and aggressive? In view of lack of evidence maintenance is the rule Coffey supports summer withdrawal

77. OCD Treatment C&A Psychopharmacology News Feb 2002 Drug Dose Benefit Clomipramine* max 5mg/kg/d 37% Fluvoxamine* 50-200mg/d 42% Sertraline* 25-200mg/d 42% Paroxetine* 10-60mg/d 51% Fluoxetine 20mg/d 45% Citalopram 10-40mg 75% *FDA approved

78. Treatment of OCD in TS Potential side effects of SSRIs Activation Hypomania Sleep disturbance Anorexia/nausea esp. Fluvoxamine Diarrhoea

79. Treatment of TS Conclusion: Treat the person not just the symptoms Focus on the child�s strengths Educate about the natural history Be positive and hopeful Educate those around the child Wait and see with regard to drugs Target disabling symptoms

80. Pete Bennett

81. Tim Howard

82. Web sites www.tsai www.tsa-usa.org www.tourettes-action.org.uk

83. Books /video Nix your tics Duncan McKinlay Breaking Free from OCD Jo Derisley, Isobel Heyman et al I have Tourette�s but Tourette�s doesn�t have me... HBO