The Management of Tics
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Movement Disorders Vol. 24, No. 1, 2009, pp. 15–24 Ó 2008 Movement Disorder Society CME The Management of Tics David Shprecher, DO and Roger Kurlan, MD* Abstract: A tic is a stereotyped repetitive involuntary move- ticularly disabling motor tics. Deep brain stimulation has ment or sound, frequently preceded by premonitory sensa- been reported to be an effective treatment for the most severe tions or urges. Most tic disorders are genetic or idiopathic in cases, but remains unproven. A comprehensive evaluation nature, possibly due to a developmental failure of inhibitory accounting for secondary causes, psychosocial factors, and function within frontal-subcortical circuits modulating voli- comorbid neuropsychiatric conditions is essential to success- tional movements. Currently available oral medications can ful treatment of tic disorders. Ó 2008 Movement Disorder reduce the severity of tics, but rarely eliminate them. Botuli- Society num toxin injections can be effective if there are a few par- Key words: Tourette’s syndrome; tics; ADHD; OCD Tics are stereotyped repetitive involuntary move- resemble purposeful movements. Complex vocal tics ments (motor tics) or sounds (vocal tics). Vocal tics, have linguistic meaning, consisting of partial words also referred to as phonic tics, are produced by the (syllables), words, or phrases. movement of air through the nose, mouth, or throat. Most individuals with tics describe a premonitory urge or sensation (such as a feeling of tension within a mus- DIFFERENTIAL DIAGNOSIS cle) that improves after performing the movement.1 Tics can be voluntarily suppressed, sometimes for Simple motor tics may need to be differentiated extended periods of time, leading to suggestion of the from myoclonic jerks, which are not typically repeti- term ‘‘unvoluntary’’ movement disorder to indicate tive in the same body part like tics. Simple tics are some element of conscious control.2 Motor and vocal commonly accompanied by complex tics and associ- tics are further classified as either simple or complex. ated with premonitory sensations or suppressibility. Simple motor tics are single repetitive jerks. Simple Complex motor tics need to be distinguished from ster- vocal tics are inarticulate noises or sounds. Complex eotypies that are longer lasting, more stereotyped motor tics involve a sequential pattern of individual movements (eg, body rocking, head nodding, and tics or more complex, coordinated actions that can hand/wrist flapping) or sounds (eg, moaning, yelling) that occur over and over again in a more continuous, less paroxysmal fashion. Stereotypies are typically seen in patients with autism, mental retardation, Rett This article is part of the journal’s online CME program. The syndrome, psychosis, or congenital blindness and deaf- CME activity including form, can be found online at http://www. ness. Some tics are slow or twisting in character movementdisorders.org/education/journalcme/ resembling dystonia and are termed ‘‘dystonic tics.’’ *Correspondence to: Dr. Roger Kurlan, Department of Neurology, University of Rochester School of Medicine and Dentistry, 1351 Mt. Contrary to dystonic tics, dystonia per se tends to be Hope Avenue, Suite 100, Rochester, NY 14620. slower and leads to more sustained disturbances in pos- E-mail: [email protected] ture of a limb, the neck, or trunk. Compulsions fre- Potential conflict of interest: Dr. Shprecher has no financial con- flicts of interest. Dr. Kurlan currently receives research support from quently occur in association with tics, can sometimes Boehringer Ingelheim. be difficult to distinguish from complex motor tics, Received 11 August 2008; Revised 9 October 2008; Accepted 13 but typically differ by being done in response to an October 2008 Published online 17 November 2008 in Wiley InterScience (www. obsession, being performed to ward off future problems, 3–5 interscience.wiley.com). DOI: 10.1002/mds.22378 or being done according to certain rules (ritualistic). 15 16 D. SHPRECHER AND R. KURLAN CATEGORIES OF TIC DISORDERS Onset of a primary tic disorder in adulthood is rare, but can occur in those genetically predisposed. In such Primary Tic Disorders cases, a specific trigger (such as a stressful life event, Chronic tics are the characteristic feature of Tour- psychiatric illness, drug exposure, or infection) as well ette’s syndrome (TS). Diagnostic criteria include pres- as a childhood and/or family history of tics, ADHD, or ence of both motor and vocal tics, onset in childhood, obsessive-compulsive symptoms can usually be identi- fluctuations in tic types and severity, and duration of at fied.44–46 least one year.6,7 When chronic tics of both types can- not be identified, the terms chronic motor tic disorder and chronic vocal tic disorder are used. Tics lasting INITIAL EVALUATION less than 1 year are termed transient tic disorder. With The management of tics is made challenging by a very high comorbidity rates of both attention deficit general lack of definitive evidence to support or refute hyperactivity disorder (ADHD) and obsessive-compul- commonly used treatments. This review is drawn from sive disorder (OCD), TS may represent a multifaceted a synthesis of existing research data and the authors’ developmental neuropsychiatric brain disorder.8 personal experience in this area. A thorough clinical TS is thought to result from a developmental failure history and neurologic examination are generally suffi- of inhibitory function within the frontal-subcortical cir- cient to screen for evidence of a secondary tic disorder, cuits governing voluntary movements,9 possibly lead- and neuroimaging or electroencephalography are usu- ing to impaired surround-off inhibition (the ability to ally not needed unless there are unexpected findings. A suppress unwanted movements).10 Dysfunction of do- more global developmental process may be suggested pamine neurotransmission is strongly implicated in this by history of early neurologic insults, a delay in devel- process, with current evidence pointing to low tonic opmental milestones, or the occurrence of seizures. A synaptic dopamine levels and excessive phasic release critical aspect of the initial evaluation is to check for of dopamine in the basal ganglia.11 Some neuroimag- the presence and severity of commonly associated neu- ing evidence suggests an increase in presynaptic dopa- ropsychiatric disorders, ADHD, and OCD. We also ask mine transporters12–15 or excessive dopaminergic inner- about a family history of tics, ADHD, and OCD. vation.16 Mood disorders, other anxiety disorders, impulse con- trol problems, and rage attacks should also be assessed. It is also important to know about psychosocial stres- Secondary Tic Disorders sors. The important goal is to accurately define the Particularly if accompanied by other movement dis- contributors to existing problems. For example, prob- orders or by other neurologic abnormalities, a second- lems with attention in school could be related to ary cause for tics should be considered. Tics often ADHD, medication side effects, mental fatigue caused indicate the presence of a global brain developmental by efforts to suppress tics, obsessive thinking, anxiety, disorder in conditions like mental retardation, autism, depression, or a learning disability. An important com- and pervasive developmental disorder. Similarly, a va- ponent of the history is to determine which symptoms riety of genetic and neurodegenerative conditions can are disabling (ie, causing problems in daily function- cause tics, including Wilson disease, neuroacanthocyto- ing) in order to select those target symptoms appropri- sis,17,18 neurodegeneration with brain iron accumula- ate for therapy. tion,19–21 and Huntington disease.22 Other potential causes of tics include lesions involving frontal-subcort- ical circuits (trauma,23 carbon monoxide poisoning,24 EDUCATING THE PATIENT, FAMILY, hypoxic-ischemic encephalopathy, and stroke25,26); AND SCHOOL central nervous system (CNS) infections (neuroborre- Education of parents, teachers, and peers is a critical liosis,27 viral encephalitis28,29) and CNS immune disor- initial intervention. We inform patients and their ders (antiphospholipid antibody syndrome,30 Syden- parents that it is appropriate to tell others that they ham’s chorea31). Tics can be a manifestation of neuro- have tics, meaning that they cannot help making cer- leptic drug-related tardive dyskinesia32 or withdrawal tain movements or sounds. We provide patients and emergent syndrome.33 Induction or exacerbation of tics parents with current information about the causes of has been reported with lamotrigine,34,35 carbamaze- tics (genetic factors, brain neurochemical imbalances) pine,36,37 cocaine,38–40 caffeine,41 and other stimu- and emphasize that they are not signs of psychological lants.42,43 or emotional illness, a common misperception. Learn- Movement Disorders, Vol. 24, No. 1, 2009 MANAGEMENT OF TICS 17 ing about the importance of genetic factors often sen symptoms, it is important to probe for these and relieves a sense of guilt in the patient and parent. consider interventions such as individual or family Although serious psychosocial factors can exacerbate counseling. For patients with mild symptoms, educa- tics, we explain how tics change in type over time and tional and psychological interventions may be suffi- that they naturally fluctuate in severity, so it is not nec- cient to bring symptoms to a tolerable level of severity. essary to search for psychological problems every time Symptoms