Tic or Treat: Tourette s Disorder

Transcription

1 1 CYC-I Presentation Tourette s Disorder 1 Tic or Treat: Tourette s Disorder Kristin Buroker Marsh, M.D. Child & Youth Psychiatric Consult Project of Iowa February A little history Sometimes called Gilles de la Tourette syndrome for French neurologist Georges Albert Edouard Brutus Gilles de la Tourette In 1885, described 9 patients with involuntary movement Cardinal features: convulsive muscular jerks, inarticulate shouts, corprolalia Noted symptoms wax and wane No general medical deterioration 3 Definitions Tic abrupt, purposeless, recurrent, stereotyped movement or sounds Often experienced as involuntary or as due to an irresistible impulse e.g. tension, tightness Can be suppressed with effort for brief periods of time Waxing and waning over weeks to months is common May mimic voluntary movements or speech or may have an exaggerated or more forceful character

2 2 CYC-I Presentation Tourette s Disorder 4 Definitions Simple tics - sudden, brief, meaningless tics occurring singly Common simple motor tics: blinking, grimaces, rapid head or limb jerks, shrugs, and abdominal tensing Common simple vocal tics: sniffs, barks, coughs, guttural throat clearing, other expiratory vocalizations 5 Definitions Complex tic movements or vocalizations orchestrated into longer or more purposefulappearing constellations Common complex motor tics: biting, throwing, hitting, skipping, touching objects or self, dystonic postures, gestures (obscene = corpropraxia, compulsive imitations = echopraxia) Common complex vocal tics: dysfluencies and aberrations in prosody through formed syllables, words, or phrases phrase types: Stereotyped ejaculations e.g. shut up!, right on! Corprolalia (obscenities) Echolalia Palilalia (echoing self) 6 DSM-IV -TR Transient Tic Disorder Single or multiple motor and/or vocal tics Tics may occur many times a day, nearly every day for at least 4 weeks, but for no longer than 12 consecutive months Onset before age 18 Not due to direct physiological effect of a substance (e.g. stimulants) or a general medical condition (e.g.huntington s) Criteria have never been met for Tourette s disorder or chronic motor or vocal tic disorder

3 3 CYC-I Presentation Tourette s Disorder 7 DSM-IV -TR Chronic Motor or Vocal Tic Disorder Single or multiple motor or vocal tics, but not both, have been present at some time during the illness Tics may occur many times a day, nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months Onset before age 18 Not due to direct physiological effect of a substance (e.g. stimulants) or a general medical condition (e.g.huntington s) Criteria have never been met for Tourette s disorder 8 DSM-IV -TR Tourette s disorder Both multiple motor and one or more vocal tics have been present at some time during the illness, but not necessarily concurrently Tics may occur many times a day (usually in bouts), nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a ticfree period of more than 3 consecutive months Onset before age 18 Not due to direct physiological effect of a substance (e.g. stimulants) or a general medical condition (e.g.huntington s) 9 Tourette s disorder Initial symptoms usually appear between 5-8 years seem like transient motor tics Often mild and involve face, head, or upper extremities Over time, tics become persistent and often have rostral-caudal progression, become complex Onset of motor tics usually precedes vocal tics by a year or two

4 4 CYC-I Presentation Tourette s Disorder 10 Tourette s disorder Course is variable 10 years - early adolescence usually most severe By 18 years, half of patients are tic free (but associated conditions often remain) Variety and temporal patterning of tics is virtually limitless Waxing and waning over weeks to months, may even disappear Markedly decrease or disappear during sleep 11 Tourette s disorder Children may be unaware or may minimize symptoms May try to camouflage the tic-like nature of a gesture Can be suppressed which can mislead others into thinking child can control it with more willpower Tics can have affect on self-esteem and social confidence Teasing Child may feel out of control of their own body 12 Tourette s disorder Associated conditions and symptoms often more debilitating Poor impulse control anger or aggressive outbursts Distractibility Anxiety Mood lability or depression Learning difficulties ADHD (>50%)

5 5 CYC-I Presentation Tourette s Disorder 13 Tourette s disorder OCD Often presents in adolescence, by adulthood 40% of those with TS have OCD Some compulsions impossible to distinguish from complex motor tics Tic-related OCD Greater male preponderance Earlier age of onset Greater predominance of: Obsessions and compulsions concerning religion, sex, and aggression Compulsions involving repeating, counting, ordering, arranging, and symmetry evening up Driven by just right rather than anxiety 14 Differential Diagnosis Presentation and natural history usually sufficient for diagnosis Distribution, timing, ability to suppress: distinguish from ballismus, dystonia, chorea Context: Akathisia and dyskinesia usually associated with stopping or starting medication OCD alone: movements usually deliberate and associated with specific ideation or situations Stereotypies: typically unchanged over months or years, appear to be soothing or pleasurable rather than intrusive or distressing 15 Differential Diagnosis Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS). Controversial not a DSM disorder Described by Swedo et al, 1997 OCD and/or tic disorder Prepubertal onset, ages 3-12 Episodic course (abrupt onset and/or exacerbations) Sx onset/exacerbations associated with two documented GABHS infections Neurologic abnormalities (hyperactivity, tics, chorea)

6 6 CYC-I Presentation Tourette s Disorder 16 Epidemiology Tics are common Most common between 7-11 years 18% of boys, 11% of girls have tics Tourette syndrome 5-10/10, Etiology Genetics TS: Concordance in monozyotic twins 53%, dizygotic 8% (if count any tic in twin, increases to 77% and 23%) 60% of TS cases are familial Vulnerability may be to TS, tics, OC symptoms Neuroanatomy Basal ganglia and their cortical, thalamic, and midbrain connections implicated Neurochemistry Possibly altered dopaminergic function Possibly noradrenergic function Perinatal factors Stressful life circumstances, severity of first trimester n/v, lower birth weight Autoimmune factors Theoretically aberrant autoimmune mechanisms triggered by group A betahemolytic strep 18 Treatment Education and Support It s a neuropsychiatric illness Not willful, provocative, or crazy Most cases not severe, improve by adulthood Runs in families no blame Work with school Destigmatize symptoms, help with teasing, appropriate services Tourette Syndrome Association Psychotherapy Illness not CAUSED by psychological factors, but exacerbated by stress Tics themselves can be distressing CBT for OCD

7 7 CYC-I Presentation Tourette s Disorder 19 Treatment Medications Balance risk and benefits Reasons to treat tics: physical discomfort, social stigmatization, interference with classroom participation First line for mild cases: clonidine or guanficine Can help tics, ADHD symptoms, and emotional lability More potent agents: neuroleptics Haldol, pimozide, fluphenazine studied the longest 60-90% of pts respond with 65% reduction in symptom severity SEs limit use Atypicals More favorable SE profile Studies support use significant improvement of tics in 12-week, multicenter, double-blind, parallel-group study of risperidone (mean daily dose = 3.8 mg), and pimozide (mean daily dose = 2.9 mg). Symptoms of anxiety and depressive mood improved significantly from baseline in both groups. Obsessive-compulsive behavior improvement reached significance only in the risperidone group 20 Treatment TS and ADHD Treatment still controversial for some reason Since 1983 FDA requires many psychostimulants to list tics as a contraindication to their use Biologically makes some sense (stimulants increase dopamine, tics thought to be due to increased dopamine activity in the basal ganglia, dopamine antagonists (antipsychotics) treat tics) BUT the association between tics and stimulants may be confounding 20% of kids with ADHD develop chronic tic disorder, usually 2-3 years after ADHD symptoms arise Kids might have developed tics regardless of treatment 21 Meta-Analysis: Treatment of ADHD in Children with Comorbid Tic Disorders Bloch, et al. JAACP, 48:9, Sept Methylphenidate, alpha-2 agonists, desipramine, and atomoxetine didn t worsen tic severity Supratherapeutic doses dextroamphetamine (1.28 mg/kg/day) did, but lower doses (0.82 mg/kg/day) did not 2 studies with clonidine, one with guanficine Significantly improved both tic and ADHD severity Hyperactive/impulsive>inattentive symptoms

8 8 CYC-I Presentation Tourette s Disorder 22 Meta-Analysis: Treatment of ADHD in Children with Comorbid Tic Disorders Bloch, et al. JAACP, 48:9, Sept Conclusions: Tx may start with alpha-2 agonist or a stimulant. Decide if targeting tics or not May wish to start with alpha-agonist first if targeting ADHD and tics. Can add stimulant. Combined treatment may produce best outcome. Tourette s Syndrome Study Group, 2002 found combo MPH and clonidine most effective (over placebo, MPH alone, clonidine alone) Did not see increase in tics Concerns about cardiac adverse events and sudden cardiac death with the combination has not been borne out in RCTs Use caution in children with known cardiac defects 23 Treatment TS and OCD SSRI TS/OCD may be less responsive than OCD alone Addition of neuroleptic may improve response Don t forget CBT 24 In case you are just waking up, here are the main things to know about Tics and Tourette s. Tics are common Tics don t necessarily need to be treated Lots of comorbidities with Tourette s disorder look for them and TREAT THEM! Ok to use stimulants in kids with tics

9 9 CYC-I Presentation Tourette s Disorder 25 Resources Tourette s Syndrome Association Link: Iowa TSA Support Group Contact: Lisa Zimmerman lisa@itsasupportgroup.org Upcoming events: March 12, 2012: Tourette s Syndrome Conference in Cedar Rapids Target Audience: Providers, Parents, Grandparents, Teachers, and anyone wanting to learn more about Tourette s Syndrome. Guest Speaker: Susan Conners, a retired school teacher of 30 years from New York who has Tourette s Syndrome herself. She is the founder of the Western New York TSA Chapter and has traveled the US presenting on the disorder. 26 References Textbook of Child and Adolescent Psychiatry, Mina Dulcan, Tourette s Syndrome Study Group: Treatment of ADHD in children with Tourette s syndrome: a randomized, controlled trial. Neurology 58: , Eddy, C. Treatment strategies for tics in Tourette syndrome. Therapeutic Advances in Neurological Disorders. Jan 4(1): Jankovic, J. Tourette syndrome: Evolving concepts. Movement Disorders. Online April Bruggeman R. Risperidone versus pimozide in Tourette's disorder: a comparative doubleblind parallel-group study. J Clin Psychiatry Jan;62(1):50-6. Bloch, et al. Meta-Analysis: Treatment of ADHD in Children with Comorbid Tic Disorders. JAACP, 48:9, Sept Questions?