Hen’s teeth .... Or not??

1. Hen’s teeth .... Or not?? Neuropsychiatric conditions in childhood Dr Kirsty Yates Community Paediatrics, GNCH

2. The problem: 5 year old boy “His behaviour is terrible. He makes these weird movements all the time . He doesn’t seem to be learning at school and they’re also complaining about his behaviour!”

3. What else do you want to know??

5. What are your initial thoughts?? • I’m not worried – reassure mum • I would like some more information

6. Family History Past Medical History Examination Social History

7. Background • Ex prem: Twin II 34+4 wk C/S • Maternal methadone and diazepam • SCBU – vomiting –ºNAS • Physically healthy • Seen for child protection medical 3y 1m. GDD – follow up

8. Development Poor handwriting Help dressing Concerns Delayed speech Persisting echolalia Needed SALT 1 yr Delayed learning History of soiling Sleep difficulties Play with others Activity and inattention

9. Family history • Both parents drug users • Hep B and C positive • Dad Plummer court • Chronic hepatitis and ?trophoblastic disease • Maternal hx depression – inpatient. • No history of movement disorder in family

10. Social History • Limited support – mum previously a LAC • Dad recently detained HMP • CSC involved • Financial difficulties 5 5 13 25 23 20

11. Examination • Normal • Observation: • Active, poor concentration, alert to noises in surroundings • Tics: Vocal and motor Screeching, grunting, blinking, grimacing, posturing • Echolalia • Pretend play - bus driver, plastic food • Poor eye contact

12. Summary of Main symptoms • Tics, restless, inattention, aggression, repetitive • behaviours, learning, speech, peer relationships • Significant psychosocial difficulties What is the differential diagnosis?

13. Differential at this point?? • TS • ASD • ADHD • LD • Attachment disorder • Environmental

15. Causes of wiggles and squiggles Personality disorder Bipolar disorder Depression Disruptive Beh. Anxiety Age of child LD Tics/TS ADHD Age(4-7 years) at assessment PDD Abuse/neglect

16. Tics • Sudden, rapid, repetitive, involuntary, stereotyped purposeless movements • Vocal or motor • Simple or complex • Common • 10% <10yrs age 25% all childhood • All races and cultural groups • 4x more common boys • Higher in special schools

17. Causes of TICS • ASD/Aspergers • Huntingtons disease • Wilsons disease • Fragile X • Hallervorden-Spatz • Idiopathic • Familial • TS • Acquired • Carbon monoxide poisoning • Drugs • Trauma/Tumour

18. Differential diagnosis of Repetitive behaviours • Stereotypies • Compulsions • Perserveration • SIB • Chorea • Choreoathetosis • Dystonia • Tremor • Myoclonus

19. Categories of Tic disorders • DSM IV • Transient tic disorder • Chronic motor or vocal tic disorder • Combined motor and vocal tic disorder (Tourette)

20. What is Tourette Syndrome? • Neuropsychiatric condition • Gille de la Tourette - 1885 • Spectrum of severity • 1 in 100 childhood population • Childhood onset

21. Diagnosis • Multiple motor tics + one or more vocal tics at some point • >1 year duration • Periods of remission <2 months • Tics change over time in location, frequency, type, complexity & severity. • <18yrs onset • Not explainable by other medical conditions

22. Clinical Characteristics • Mean age onset 7 yrs (2-18y) • Tics • Echophenomenon • Coprolalia/ Copropraxia • Paliphenomena • Other stuff....

23. Tic Progression

24. Aetiology • Precise location in brain unknown ?basal ganglia/frontal cortex – dopamine transport, release & uptake • Biological , genetic (concordance in twins) • PANDAS • Exacerbations by environmental factors

25. What does it feel like?

26. Difficulties and Misconceptions • Coprolalia – RARE! 1-3/10 adults • Suppressing tics/Hiding Tics • Often improve when absorbed in a task • Co-morbidities may be the presentation

27. What should you say? • It’s not their fault, • Acceptance and understanding essential • Tics can change; Course can wax and wane • Tics be suppressed, but often payback • Exacerbations at times of stress, boredom, excitement and illness

28. Tics and the “other stuff” • Physical, educational, economical and social consequences • 12% have tics only • Often Tics not the main problems. Tics as a marker

29. Common Co-morbidites Sleep LD

30. Famous people with Tourette Syndrome

31. Treatment • Drug treatment available for Tics but often side effects with sedation and weight gain, extra-pyramidal side effects • Should be started & monitored by specialist. • Strategies: • Ignoring the tics • CBT – OCD element • Behavioural analysis • Competing response, relaxation, massed negative • Future: ?DBS, ?Immunological therapies

32. Further Information • Tourette syndrome association uk. • www.tourettes-action.org.uk • www.tsa.org • Books • “Why do you do that? A Book about Tourette Syndrome for Children and Young People” UttomChowdhury and Mary Robertson. • “Hi, I’m Adam: A Child’s Book about Tourette Syndrome” Adam Buehrens • Tics and Tourette syndrome. A Handbook for Parents and Professionals. UttomChowdhury

33. Take home messages • Tics are common • Tourettes has a spectrum of severity and is more common than we think • Tics as a symptom on their own do not necessarily require treatment but parental education and understanding paramount. • Tics/TS can be a marker for other neurobiological conditions that have worse consequences

34. Questions?