1 Identification of tics and associated comorbidities: Lessons learnedAmy 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 ADHDDoesn’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 schoolCan 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 OCDStarted 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
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 excitedMore 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, shiverVery 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 SyndromeBoth 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)
16 Treatment of Tics Most people are unaware they are ticcing80% 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 ticsGuanfacine- 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 ticsClonazepam- 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
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 treatmentSignificant 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 worryDon’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 ticsDistracted 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 treatmentDiagnosed 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 OCDSSRIs 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 ( 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 ( 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 OCDParoxetine- 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 12Impairment in 2 or more settings Symptoms have to occur “pretty much” or “very much” 3 presentations: inattentive, hyperactive/impulsive or combined
32 ADHD Inattentive SymptomsInattentive: 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 SymptomsHyperactive/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 ticsTreatment 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 stimulantStimulants 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 preparationsMethylphenidate- 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 preparationsAdderall 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 StimulantsAppetite 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 SyndromeMakes 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 comorbiditiesTreat the most impairing problem first Refer to neurology if things do not improve