Clues for differentiating simple from more complex tic disorders
Virgilio Gerald H. Evidente, MD
VOL 108 / NO 5 / OCTOBER 2000 / POSTGRADUATE MEDICINE
CME learning objectives
- To become familiar with the various types of tic disorders
- To learn the common signs of Tourette’s syndrome
- To understand diagnostic approaches helpful in management of tic disorders
The author discloses no financial interests in this article.
This is the fourth of four articles on hyperkinetic movement disorders
Preview: Transient tics are common, particularly among children under the age of 10 years. In fact, about 20% of kids in this age-group have tics that disappear as they get older. Nonetheless, parents often are concerned about what strange movements or sounds mean and what can be done about them. Many have heard horror stories about Tourette’s syndrome and fear a life of social rejection because of uncontrollable “urges.” In this article, the author discusses the various types of tics and the wide range of treatments available for their management.
Evidente VGH. Is it a tic or Tourette’s?: clues for differentiating simple from more complex tic disorders. Postgrad Med 2000;108(5):175-82
Tics are defined as brief, intermittent, repetitive, nonrhythmic, unpredictable, purposeless, stereotyped movements (motor tics) or sounds (phonic or vocal tics). They are associated with an urge, and voluntary suppression results in psychic tension and anxiety. Subsequent “release” of the movements or sounds results in relief.
Although tics appear to be voluntary, the affected person often feels compelled to move to relieve an unexplainable urge. Thus, some authorities consider tics as “semivoluntary” or “unvoluntary” (1). Although tics may resemble other types of hyperkinetic movements (eg, myoclonus, dystonia), the urge is considered the key characteristic that suggests that the movement is a tic rather than another movement disorder.
Classification of tics
Tics are classified as either simple or complex. Simple motor tics are focal movements involving one group of muscles, such as eye blinking, tongue protrusion, facial grimacing, shoulder shrug, or head turning. Complex motor tics are coordinated or sequential patterns of movement that resemble normal motor tasks or gestures. Examples include jumping, throwing, head shaking, making obscene gestures such as “giving the finger” (copropraxia), and imitating gestures of others (echopraxia).
Simple phonic tics are elementary, meaningless noises and sounds, such as grunting, sniffing, clearing the throat, squeaking, coughing, wheezing, belching, hiccuping, whistling, or producing animal sounds. Complex phonic tics include meaningful syllables, words, or phrases (such as saying “okay” or “shut up”); repeating one’s own utterances, especially the last syllables of words (palilalia); repeating someone else’s words or phrases (echolalia); or shouting obscenities or profanities without any reason or provocation (coprolalia).
Motor tics can also be classified according to speed of movement. Those that are brief, sudden, and jerklike are known as clonic tics (eg, blinking, facial twitching). Motor tics that involve brief twisting or posturing are called dystonic tics (eg, torticollis, blepharospasm), whereas those that involve sustained or prolonged movements or contraction of muscles are labeled tonic tics (eg, prolonged bending of the trunk or tensing of the abdomen).
Sensory tics refer to uncomfortable sensations, such as pressure, tickle, cold, warmth, or paresthesias that are localized to certain body parts and that are relieved by the performance of an intentional act in the affected area (2). Rarely, motor tics may be provoked by a mental projection of sensory impressions to other persons or objects and are relieved by touching or scratching that person or object. These are known as phantom tics (3).
Types of tic disorders
The fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) (4) lists three types of tic disorders: Gilles de la Tourette (Tourette’s) syndrome, chronic motor or vocal tic disorder, and transient tic disorder.
By DSM-IV criteria, Tourette’s syndrome is characterized by the following features:
- Multiple motor and one or more phonic tics (not necessarily concurrently)
- Onset before age 18 years
- Tics that occur many times a day, nearly every day or intermittently for more than a year, with symptom-free intervals not exceeding 3 months
- Variations in anatomic location, number, frequency, complexity, and severity of the tics over time
- Tics that are not related to intoxication with psychoactive substances or central nervous system (CNS) disease (eg, encephalitis)
- Symptoms that cause significant impairment of social, academic, and occupational functioning
If only motor or vocal tics are present (not both), the appropriate diagnosis is chronic tic disorder. If single or multiple motor or vocal tics are present many times a day, nearly every day for at least 4 weeks but no longer than 12 consecutive months, the term “transient tic disorder” applies. Transient tics are seen in 20% of children during their first decade of life.
Several conditions, termed tourettism, may mimic Tourette’s syndrome. Drugs, including stimulants, levodopa (Dopar, Larodopa), and antiepileptic medications (eg, phenytoin [Dilantin], carbamazepine [Atretol, Epitol, Tegretol], lamotrigine [Lamictal]), may cause tourettism. Discontinuation of the offending drug leads to prompt remission of the tics. Neuroleptic medications with potent dopamine2 (D2) antagonist activity (eg, haloperidol [Haldol]) may cause tardive tourettism, which presents with delayed and often permanent tics identical to Tourette’s syndrome.
Other causes of tourettism include infections (eg, Creutzfeld-Jakob disease, encephalitis), toxins (eg, carbon monoxide), Huntington’s disease, head trauma, stroke, neuroacanthocytosis, chromosomal abnormalities, cerebral palsy, neurocutaneous syndromes, and schizophrenia (1).
Clinical features of Tourette’s
Tourette’s syndrome is the most common and severe form of multiple tic disorder, with a prevalence of 10 cases per 10,000 population (5). Its onset is usually between ages 2 and 15 years (mean, 6.5 years) (2). On average, phonic tics begin 1 to 2 years after the onset of motor tics. Symptoms remit by a median age of 18 years in about 75% of cases. In rare cases, Tourette’s syndrome may start during adulthood in the absence of precipitating factors (eg, exposure to neuroleptic drugs, infections, stroke). Focal tics may also appear in adults in relation to peripheral nerve injury in the area of the tic.
Tics increase in frequency and severity with stress, relaxation after physical exertion, excitement, idleness, fatigue, exposure to heat, and use of dopaminergic drugs, steroids, caffeine, and CNS stimulants (1). Rarely, motor tics may be induced by an unexpected startling stimulus (ie, reflex tics or startle-induced tics). Exacerbation or precipitation of tics in children may also occur after infection with group A beta-hemolytic streptococci (6).
Tics usually diminish with performance of engaging mental or physical activities (eg, playing computer games, playing sports) or with consumption of cannabinoid substances (eg, marijuana), alcohol, or nicotine (2,7). Unlike most hyperkinetic movement disorders, tics may persist during light stages of sleep.
Although the disability with Tourette’s syndrome is often confined to social embarrassment, severe motor tics may lead to physical injury, including fractures, cervical radiculomyelopathy, joint dislocation, and falls. Severe phonic tics may also impair respiration, swallowing, and speech.
Tourette’s syndrome is often accompanied by other conditions, particularly attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder. Both of these disorders can contribute substantially to the disability and management problems of the illness.
ADHD afflicts 50% of Tourette’s syndrome patients with or without hyperactivity (2). It manifests as impulsivity, inattention, restlessness, fidgeting, poor concentration, poor school or work performance, and learning impairment. Not uncommonly, ADHD becomes the major problem because severely hyperactive kids, especially those with severe phonic tics or coprolalia, become disruptive to other children in class.
Obsessive-compulsive symptoms are repetitive, stereotyped, involuntary, senseless thoughts or behaviors that intrude into the patient’s consciousness or actions. These symptoms become a disorder (obsessive-compulsive disorder) if they cause significant social impairment and emotional distress. Between 30% and 50% of patients with Tourette’s syndrome also have obsessive-compulsive disorder, which may be more troublesome than the tics. Other behavioral problems that are also seen in Tourette’s syndrome include quick temper, mood swings, overreaction, exhibitionism, negativism, rage attacks, oppositional defiant disorder, bipolar disorder, schizo-affective disorder, and cyclothymic disorder (5).
Etiology and pathogenesis
Idiopathic tic disorders and Tourette’s syndrome are multifactorial in etiology. Although genetic factors play a major role in causing Tourette’s syndrome, the environment seems to influence the risk, severity, and course of the disorder. Genetic factors are present in about 75% of cases, with 25% presenting with bilineal transmission (both parents affected) (1,2,8). However, the exact genetic basis for Tourette’s syndrome has been elusive. Autosomal dominant, autosomal recessive, and sex-linked inheritance have been considered but have been rejected (9). Numerous candidate genes have been tested and excluded, and complete genome screens have failed to show significant results (10).
Neuropsychiatric disorders, such as tic disorders and obsessive-compulsive disorder, may develop after streptococcal infection (termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection [PANDAS]). The mechanism is most likely autoimmune-related, whereby antibodies against bacterial antigens cross-react with proteins in the brain (6). The manner in which genetic factors and environmental factors interrelate in Tourette’s syndrome is unknown.
Tic disorders involve abnormalities in the CNS levels of neurotransmitters or neuropeptides, particularly dopamine, norepinephrine, opioid peptides, and serotonin (11). This involvement is suggested by the effectiveness of drugs that modulate these substances (eg, dopamine antagonists, alpha-adrenergic agonists, opiate antagonists, serotonin-active antidepressants, nicotine patches) (11,12). Nigral and pallidal levels of glutamate are reduced in Tourette’s syndrome (11), and some investigators suggest that endogenous cannabinoid transmission may also be involved (7).
In anatomic agreement with these biochemical characteristics, functional magnetic resonance imaging, positive emission tomography, and single-proton emission computed tomography studies suggest that ADHD, obsessive-compulsive disorder, and Tourette’s involve abnormal neurophysiology of the basal ganglia-thalamocortical pathways (13).
Tics and the conditions with which they are associated can be difficult to treat. Education of the patient and family about tics and Tourette’s syndrome is essential. The promotion of support and understanding should form the basis of both pharmacologic and nonpharmacologic treatment strategies. Pharmacologic treatment, because of potential side effects, should start with agents that can do the least harm.
Clonidine hydrochloride (Catapres), an alpha2-adrenergic agonist, is the drug of choice for most clinicians because of its efficacy in controlling tics and its safety in children, even with long-term use. Initial dosage is 0.05 to 0.1 mg per day, which can be increased by 0.05 to 0.1 mg weekly (average dose, 0.3 mg daily). Guanfacine hydrochloride (Tenex) in a dosage of 0.5 to 1.5 mg twice daily is another alpha2-adrenergic agonist that may reduce tics. However, it is not labeled for use in children less than 12 years old (5,11). Benzodiazepines, particularly clonazepam (Klonopin) and diazepam (Valium), may also diminish tics but often cause significant sleepiness, lethargy, and mental clouding (2). The calcium channel blockers nifedipine (Adalat, Procardia) and verapamil may also suppress tics in some cases.
The potent D2 antagonists haloperidol (Haldol) in a dose of 0.5 to 4 mg at bedtime and pimozide (Orap) in a dose of 1 to 8 mg at bedtime are two of the most effective neuroleptics in suppressing tics (2,5,11). However, they are associated with significant side effects, including mental clouding, lethargy, and extrapyramidal side effects, such as tardive dyskinesia (which may be permanent), parkinsonism, and akathisia.
Risperidone (Risperdal) is an atypical neuroleptic with strong 5-hydroxytryptamine2 receptor antagonist action. In dosages of 1 to 2 mg twice or three times daily, it is better tolerated than haloperidol. Being a potent D2 receptor antagonist, risperidone may also cause tardive dyskinesia and parkinsonism but usually only at doses exceeding 6 mg per day.
Clozapine, an atypical neuroleptic agent with D4 receptor antagonist activity, does not cause tardive dyskinesia but may cause agranulocytosis. Olanzapine (Zyprexa), another atypical neuroleptic agent that has very few extrapyramidal side effects, does not cause agranulocytosis but is often poorly tolerated by children.
The dopamine-depleting agents reserpine and tetrabenazine may be useful second-line or adjunctive drugs for tic control but may cause depression and parkinsonism. However, tetrabenazine has not been approved by the US Food and Drug Administration, and reserpine is becoming difficult to obtain.
Intramuscular or subcutaneous injections of botulinum toxin (Botox) may control motor and vocal tics that are unresponsive to oral medications (14). Opiate antagonists (eg, naloxone hydrochloride [Narcan], naltrexone hydrochloride [Depade, ReVia]) may temporarily reduce tics. The dopamine agonist pergolide mesylate (Permax), in a mean dose of 177 micrograms per day, may benefit children with Tourette’s syndrome and comorbid restless legs syndrome (15).
For the treatment of obsessive-compulsive disorder, the selective serotonin reuptake inhibitor (SSRI) fluoxetine (Prozac) and the tricyclic antidepressant clomipramine (Anafranil) are most effective (2). Other SSRIs and tricyclic antidepressants may also benefit patients who have obsessive-compulsive disorder. The combination of either a serotonin-active drug (eg, fenfluramine hydrochloride, lithium, buspirone hydrochloride [BuSpar]) or a dopamine antagonist (eg, risperidone) with an antidepressant may also prove beneficial. Clonidine and opiate antagonists may also offer some relief of obsessive-compulsive disorder.
For ADHD, the psychostimulants are most effective, particularly methylphenidate hydrochloride (Ritalin), dextroamphetamine sulfate (Dexedrin, Dextrostat), and pemoline (Cylert) (2). Their long-term use in children seems safe, with no significant effects on the cardiovascular system or on growth. Rarely, psychostimulants at usual doses may exacerbate tics. In such cases, antidepressants, clonidine, and the monoamine oxidase inhibitor selegiline hydrochloride (Carbex, Eldepryl) may be used as alternatives (16,17).
Behavioral and cognitive treatment
A variety of behavioral and cognitive treatment approaches have been used in Tourette’s syndrome, including habit reversal training, awareness training, competing response training (where the opposite movement to the motor tic is performed), and self-monitoring (11). These techniques have mixed results and have not been validated by controlled studies.
Various neurosurgical procedures have been performed for debilitating, pharmacologic-resistant tics. Recently, stereotactic surgery with high-frequency stimulation of the thalamus was reported to be effective (18). Data concerning the risks and benefits of these procedures for the treatment of Tourette’s syndrome are limited; thus, stereotactic procedures remain experimental at this time.
About 85% of children with Tourette’s syndrome experience diminution or remission of their symptoms during or after adolescence (5). Tics persisting beyond teenage years usually become permanent, and idiopathic adult-onset tics usually persist. A poorer prognosis in adulthood is associated with perinatal complications, chronic physical illness, unsupportive and unstable family milieu, comorbid mental and developmental disorders, and exposure to cocaine and anabolic steroids (11).
Tics are characterized by sterotyped, purposeless, and irregularly repetitive movements and usually can be classified as chronic motor or vocal tic disorders, transient tic disorders, or Tourette’s syndrome. The latter is a complex disorder associated with multiple tics and often accompanied by other conditions, such as ADHD and obsessive-compulsive disorder. Treatment can be difficult, and drug therapy should begin with agents least likely to cause problems for the patient. Education of the patient and family and support from the physician and other care providers are essential elements of effective management.
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For a helpful guide to electronic and print resources on hyperkinetic movement disorders for physicians and patients, see the Resource Guide in this issue.
Dr Evidente is senior associate consultant in neurology, Parkinson’s Disease and Movement Disorders Center, Mayo Clinic Scottsdale, Scottsdale, Arizona. Correspondence: Virgilio Gerald H. Evidente, MD, Department of Neurology, Mayo Clinic Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259.