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 Dampierres 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 gt3 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
• Huntingtons
• Sydenhams (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, youre 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 peoples
  voices)
• Disinhibition( urge to touch hot or dangerous
  objects)

22
Tic Repertoire

• The range of tics varies enormously
• Each persons 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 31

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)
• Its 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
Basal Ganglia

• 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

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 dont 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
• Its important that the child and parent become
  observers

58
Assessment

• Impact at school
• Cognitive function and achievement
• Attention problems
• Penmanship
• Compulsions (crossing ts 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 _at_ 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 (21)
• (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 CA 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 childs 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 Tourettes but Tourettes doesnt have
  me... HBO