Identification of tics and associated comorbidities: Lessons learned

1. Identification of tics and associated comorbidities: Lessons learned Amy Vierhile, DNP, RN, PPCNP-BC University of Rochester Medical Center Division of Child Neurology NPA Annual Conference

2. Objectives • To differentiate between tics and other common movement disorders • To review the DSM-V criteria for tics and Tourette Syndrome • To recognize the common comorbidities that often occur along with tics and how to diagnose them • To discuss the treatment of tics and comorbidities

3. Case study • Michael, age 9 • Referred for ADHD • Doesn’t do any work in school • Diagnosed with ADHD • Treated with low dose stimulant • No benefit, dose increased • Became tired, lethargic, no improvement in symptoms

4. Clinic presentation • Apathetic, doesn’t like school • Can rally at times, avoids writing • Mom spends hours every night assisting with homework • Parents are certain something is being missed • Bald spot on back of head, engages in hair pulling during down time • Mild sniffing and eye blinking tics for several years

5. Michael’s handwriting

6. Outcome • Treated with fluvoxamine for OCD • Started intensive therapy with Cognitive Behavioral Therapy; therapist worked extensively with school to set up reward plan for work accomplished • Worked with mom to pull back on homework completion • Wears a hat to cover thinning hair in back, not interested in resisting urge to pull hair • Most of work done now in school, earns phone or Ipod for lunch • Works on social skills with school psychologist and a friend once a week • Passed 7th grade, 80 average

7. Take home message • Everything that looks like ADHD isn’t.

8. History • First reported in 1825 by Itard, who identified tics in a French noblewoman • Named for Gilles de la Tourette in 1885 • Now understood to originate in the basal ganglia, which is also responsible for OCD and other movement disorders

9. Statistics • Neurodevelopmental disorder, affects 1 in 100 children ages 5-17 • 3-4:1 males to females • Average age of tic onset: 7 years • Autosomal dominant • Tics generally improve with age

10. What are tics? • Repetitive movements or noises that occur in a stereotyped fashion • Often person is unaware they are doing them • Increase with stress or excitement • Most common motor tics: eye blinking, nose wrinkling • Most common vocal tics: sniffing and throat clearing • Complex tics: involve more than one muscle group • Tic video: https://www.youtube.com/watch?v=aDipNAuZuZI

11. Stereotypies • Repetitive movements, stereotyped • Occur when excited • More common in ASD; can occur in healthy children as well • Often disappear as child becomes more socially aware • Complex stereotypies can be related to ADHD • https://www.youtube.com/watch?v=-ITjGqR119s

12. Seizures • Occasionally mistaken for tics: quick myoclonic jerk, shiver • Very brief alteration in consciousness • Unusual for that to be the only tic • Videos are helpful • https://www.youtube.com/watch?v=0Ufnl3FWz6Q

13. DSM-V criteria for Tourette Syndrome • Both multiple motor and at least one vocal tic (not concurrently) that occur daily or intermittently for over a year • Tics started before age 18 • Not due to another cause (i.e. Sydenham’s chorea, post-encephalitis)

14. Chronic Motor or Vocal Tic Disorder • Either vocal or motor tics, but not both; may also be defined as the same tic off and on for life • Provisional tic disorder is defined as single or multiple motor or vocal tics present less than a year

15. Coprolalia • Utterance of socially unacceptable words • Usually not associated with anger • Present in only 10-15% of patients with Tourette Syndrome • Words are inserted into a sentence inappropriately or shouted out as a single word

16. Treatment of Tics • Most people are unaware they are ticcing • 80% people endorse a premonitory urge • Tics are misdiagnosed: patients go for an eye exam (for blinking) or get treated for allergies (for sniffing) • Unless the tics bother the child (not the parent), we don’t treat them • Tics wax and wane; worsen with stress, excitement, tiredness, illness

17. Treatment, continued… • Worse at the beginning of the school year, better over the summer • May suppress tics during the school day, “let loose” at home; parents see worse tics than teacher • Treatment depends on entire picture, may treat with stimulant or SSRI and not tics directly • May need more than one medicine

18. Comprehensive Behavioral Intervention for Tics (CBIT) • Manualized approach, 8-10 sessions • Taught by trained professional • Teaches person to recognize feeling (urge) right before ticcing and performing a competing response • Very effective • Technique can be applied to other tics

19. Medications used to treat tics • Guanfacine- start small (0.5 mg/day), can work up to 4 mg as tolerated; side effects: dry mouth, hypotension, sedation, headache, weight gain; also helps with hyperactivity • Clonidine- stronger than guanfacine, more sedation, start with 0.05 mg 3-4 times a day or clonidine ER 0.1 mg twice a day, maximum dosage 0.4 mg/day, side effects same as guanfacine; also helps with hyperactivity • Risperdal- atypical antipsychotic, start with 0.25 mg twice daily, max is 1 .5 mg twice a day; side effects: weight gain, gynecomastia, galactorrhea, sedation; also helps with anxiety and OCD

20. Other medications used to treat tics • Clonazepam- benzodiazepine, start with 0.25 mg twice a day, max dose is 2 mg twice a day; side effects: sedation, tolerance, dependence; may also aid anxiety • Haldol- antipsychotic, start with 0.25 mg twice a day, max 2 mg twice a day; side effects: dyskinesias, sedation, weight gain, dystonic reaction, gynecomastia, school phobia • Topamax- seizure/migraine medication, start with 25 mg a day, max dose is 200 mg/day; side effects: sedation, weight loss, word finding difficulties; may aid mood as well

21. Comorbidities • 50% of people with tics will have ADHD, anxiety/OCD • May be more impairing than the tics • “Back door” for treating tics

22. Emma’s story • 12 year old, 6th grader with loud snorting, obvious facial tics • Labeled as “weird” by classmates, won’t explain tics • Very shy, no close friends, occasionally speaks to classmates • Gets little work done in school, doesn’t participate, won’t raise hand, if called on won’t answer; labeled as oppositional • Reads at 5th grade level, math at 2nd grade level; gets small group math assistance 3 times a week • Temper outbursts at home, having to do something she doesn’t want to, leaving house, unexpected activities • Overly attached to mom, worries about family • No counseling, parents opposed to medications

23. Emma’s diagnoses and treatment • Significant anxiety, Tourette Syndrome • Temper and lack of participation in class related to anxiety • School had never done testing- math disability • Got an IEP • Started counseling • Discussed medication with parents, started on SSRI • Tics have decreased a lot, now has some friends, still shy • Temper outbursts disappeared

24. Anxiety rating scales • Children: SCARED (Screen for Child Anxiety Related Disorders, ages 8 and up), PARS (Pediatric Anxiety Rating Scale, ages 6-17), Spence Children’s Anxiety Scale • Adolescents: HAM-A, PARS, SCARED • Adults: HAM-A

25. Anxiety • Often parents do not know how much their children worry • Don’t just ask if they worry • Common things children worry about: family members, pets, school, friends, bad people, breaking the rules • Need to determine how much of someone’s day is spent worrying • Can be mistaken for ADHD • Ask about family history

26. Anthony’s story • 9 year old with moderate motor and vocal tics • Distracted in class, will perseverate on topics of interest to him • Recites movie lines verbatim • Steals other student’s erasers and pencil nubs, keeps in pockets • Labeled with Conduct Disorder • ASD suggested repeatedly by teachers • Has few friends, some trouble complying with what they want to do, tattles, bossy, rigid

27. Anthony’s diagnoses and treatment • Diagnosed with Tourette Syndrome and Obsessive Compulsive Disorder • Started therapy • Started on an SSRI • Working on becoming more flexible

28. Obsessive Compulsive Disorder • “Just right” phenomena, can lead to rage • Children don’t have “typical” OCD • More sensory component: clothes don’t feel right, loud noises (chewing, humming), picky eaters, rule followers, need to know the plan, very bossy, rigid • Stuck on routines: bedtime, upset if changes • Y-BOCS is validated rating scale • Assesses obsessions and compulsions • Overall time consumed, interference, distress, ability to resist, control

29. Treatment of anxiety and OCD • SSRIs and SNRIs are most helpful in combination with Cognitive Behavioral Therapy; need to wait after dose adjustments for effectiveness; warn about FDA black box warning • Fluoxetine- very effective for OCD, start small (5 mg), max dose 60 mg/day; side effects: weight gain or loss, activation, worsening of mood or behavior, give in am • Fluvoxamine- very effective for OCD, start small (12.5-25 mg), max dose 200 mg/day; side effects: weight gain, sedation, worsening of mood or behavior, give in pm • Sertraline- good for anxiety, start small (12.5-25 mg), max is 200 mg/day; side effects: activation, weight gain, worsening of mood or behavior, give in am

30. Other medications for anxiety and OCD • Paroxetine- excellent for anxiety/panic; start small (5 mg), max dose 40 mg/day; side effects: sedation, weight gain, worsening mood or behavior, give in pm • Duloxetine- good for anxiety; start small (12.5 mg twice a day), work up to 120 mg/day; side effects: headache, weight loss, dry mouth, nausea • Venlafaxine- good for anxiety; start small (12.5 mg twice day), work up to 75 mg/day; side effects: nausea, dizziness, drowsiness, dry mouth

31. ADHD • Onset of at least some symptoms before age 12 • Impairment in 2 or more settings • Symptoms have to occur “pretty much” or “very much” • 3 presentations: inattentive, hyperactive/impulsive or combined

32. ADHD Inattentive Symptoms • Inattentive: more common in girls and anxious people • Not following through on tasks • Forgetful in daily activities • Easily distracted • Difficulty sustaining attention in tasks or play • Loses things • Needs to have their name called several times to get attention • Little attention to detail • Disorganized

33. ADHD Hyperactive/Impulsive Symptoms • Hyperactive/Impulsive: more common in preschoolers • More fidgety and squirmy than peers • Out of seat when he/she shouldn’t be • Runs or climbs excessively in inappropriate situations • Makes a lot of noise • Acts as if he is “on the go” or “driven by a motor” • Talks too much • Blurts out answers before a question is completed • Has trouble waiting his turn • Interrupts more than expected

34. ADHD Treatment • Do not avoid stimulants due to tics • Treatment of ADHD may relieve tics • Medication is an aid and not intended to “fix” all symptoms • School modifications • Preferential seating • Wiggle seat, theraband, Velcro, fidget toys, gum • Cues that something important is being said • “Chunking” work • Deliver daily attendance, stand to complete work • IEP vs 504 plan

35. How ADHD Medication Works

36. Medications • 80% of people with ADHD respond favorably to stimulant • Stimulants target dopamine and norepinephrine • Methylphenidate and amphetamine products • Amphetamines are more than twice as powerful • Nonstimulants target norepinephrine only, about 60% effective • Alpha agonists, such as guanfacine (Intuniv) and clonidine (Kapvay); can aid with tics as well • Atomoxetine (Strattera)

37. Medication Rules • Dose according to response: push the dose up until you see optimal response with minimal side effects • People often don’t dose high enough • Wait out side effects if possible for 2 weeks • Have parents give it at home so they can see the difference • Have teacher rate the child on each dose

38. Methylphenidate preparations • Methylphenidate- short acting, lasts 3-4 hours, rebound • Concerta-lasts 12 hours, must be swallowed, osmotic pump system • Metadate CD- lasts 8 hours, can be sprinkled or swallowed, 30% beads release immediately, 70% over the 8 hours • Ritalin LA- lasts 8 hours, can be sprinkled or swallowed, 50% beads release immediately, 50% 4 hours later • Focalin XR- lasts 10 hours, can be sprinkled or swallowed • Dexmethylphenidate tablets- last 5 hours, can be crushed • Quillivant XR- lasts 12 hours, liquid • Quillichew- lasts 12 hours, chewable tablet • Methylphenidate ER- lasts 7-8 hours, must be swallowed

39. Amphetamine preparations • Adderall XR- lasts 8-10 hours, can be sprinkled or swallowed • Mixed amphetamine salts- lasts 6 hours, can be crushed • Dextrostat- lasts 6 hours, can be crushed • Vyvanse- lasts 13 hours, can be sprinkled, swallowed or dissolved in water • Dyanavel XR- lasts 13 hours, liquid • Evekeo- lasts 4-6 hours, can be crushed

40. Side Effects of Stimulants • Appetite suppression • time dosing so it wears off before meals • supplement with Pediasure, Carnation Instant Breakfast • balance stimulants and non-stimulants • consider cyproheptadine • Insomnia • use shorter acting stimulants • wake child early to give medication • Stomach ache- give with food • Emotional lability- may improve over time; if not, what are you missing?

41. Multiple medications for Tourette Syndrome • Makes it difficult to sort out side effects; i.e. activation from SSRI can be mistaken for hyperactivity of ADHD; need to keep good notes • Do not treat side effects with another medication if possible • Always chasing something

42. Conclusions • Tics may be just the beginning • Screen for comorbidities • Treat the most impairing problem first • Refer to neurology if things do not improve