Treatment of Tourette syndrome
Encyclopedia
Tourette syndrome
(also Tourette's syndrome or TS) is an inherited
neuropsychiatric
disorder with onset in childhood, characterized by the presence of motor and phonic tic
s. Treatment of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.
Tourette syndrome patients may exhibit symptoms of other comorbid conditions along with their motor and phonic tics. Associated conditions include attention-deficit hyperactivity disorder
(ADD or ADHD), obsessive-compulsive disorder
(OCD), learning disabilities and sleep disorder
s. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function. Co-occurring OCD can also be a source of impairment, necessitating treatment. Not all persons with tics will also have other conditions and not all persons with tics require treatment, but when comorbid disorders are present, they often require treatment.
Stimulants (like Adderall
and Ritalin) are underused in the treatment of ADHD when tics are also present because of unfounded fears that they increase tics. Experimental treatments like deep brain stimulation
, nicotine
, marijuana and complementary and alternative medicine approaches have widespread appeal but unproven safety and efficacy.
conditions, which, when present, are often a larger source of functional impairment than the tics themselves.
There is no cure for Tourette's and no medication which works universally for all individuals without significant adverse effects; knowledge and understanding are the best treatments available for tic
s. Management of the symptoms of Tourette's may include pharmacological
, behavioral and psychological therapies. While pharmacological treatment is reserved for more severe symptoms, other types of treatments may help avoid or improve symptoms of depression
or social isolation
, and improve supportive family functioning. Educating the patient, family, and surrounding community (school, church, friends, etc.) is a key part of treatment.
The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. Because children with tics often present to physicians when their tics are at their highest severity, and because of the waxing and waning nature of tics, medication is not usually started immediately or changed often. Frequently, the tics subside with understanding of the condition and a supportive environment. When medication is necessary, pharmaceutical intervention should be targeted at the most impairing symptoms, taking into account co-occurring conditions such as ADHD
or OCD, which when present, may warrant treatment even when tics are mild.
The classes of medication with the most proven efficacy in treating tics—typical and atypical
neuroleptics
including risperidone
(trade name Risperdal), ziprasidone
(Geodon), haloperidol
(Haldol), pimozide
(Orap) and fluphenazine
(Prolixin)—can have long-term and short-term adverse effects
. The antihypertensive
agents, clonidine
(Catapres) and guanfacine
(Tenex), are also used to treat tics; studies show variable efficacy, but a lower side effect profile than the neuroleptics. Stimulants and other medications may be useful in treating ADHD when it co-occurs with tic disorders. Drugs from several other classes of medications can be used when stimulant trials fail, including guanfacine
(Tenex), atomoxetine (Strattera) and tricyclics
. Clomipramine
(Anafranil), a tricyclic antidepressant
, and SSRIs
—a class of antidepressant
s including fluoxetine
(Prozac), sertraline
(Zoloft), and fluvoxamine
(Luvox)—may be prescribed when a Tourette's patient also has symptoms of obsessive–compulsive disorder.
There are no medications specifically designed to target tics, although some antipsychotic
s (for example, pimozide
) have been FDA
-approved for treating Tourette's. Medications which are used as primary treatment in other conditions are used with some success in treating tics. Neuroleptic medications (antipsychotics), such as haloperidol
(brand name Haldol
) or pimozide
(brand name Orap
), have historically been and continue to be the medications with the most proven efficacy in controlling tics. These medications work by blocking dopamine receptor
s, and are associated with a high side effect profile. The traditional antipsychotic drugs are associated with tardive dyskinesia
when used long-term; and parkinsonism
, dystonia
, dyskinesia
, and akathisia
when used short-term. Additional side effects can be school phobia (a form of separation anxiety
), depression
, weight gain, and cognitive blunting (dulling of cognitive
ability). Another traditional antipsychotic used in treating Tourette's is fluphenazine
(brand name Prolixin), although the evidence supporting its use is less than that of haloperidol
and pimozide
.
Newer neuroleptics, the atypical neuroleptics, are an alternative to the traditional medications used for treating tics. These medications have more selective dopamine blocking effects, or block serotonin
with some blocking of dopamine. The medications in this class used to treat tics include risperidone
(brand name Risperdal), olanzapine
(brand name Zyprexa), ziprasidone
(brand name Geodon), quetiapine
(brand name Seroquel), clozapine
(brand name Clozaril), tiapride, and sulpiride
. They seem to have lower risks of neurological side effects (such as tardive dyskinesia
) when used short-term, but longer trials are needed to confirm this. Some of the side effects associated with these medications are insomnia
, weight gain, and school phobia. Abnormalities in metabolism, cardiac conduction
times, and increased risk of diabetes are concerns with these medications. There is good empirical support for the use of risperidone
, and less support for the others.
The α2-adrenergic receptor agonists (antihypertensive
agents) show some efficacy in reducing tics, as well as other comorbid features of some people with Tourette's. Originally developed to treat high blood pressure, these medications are a safer alternative to neuroleptic medications for the people with TS that respond to them. This class of medication is often the first tried for tics, as the antihypertensives have a lower side effect profile than some of the medications which more proven efficacy. The evidence for their safety and efficacy is not as strong as the evidence for some of the standard and atypical neuroleptics, but there is fair supportive evidence for their use, nonetheless. This class of medication takes about six weeks to begin to work on tics, so sustained trials are warranted. Because of the blood pressure effects, antihypertensive agents should not be discontinued suddenly. Clonidine
(brand name Catapres) works on tics for about half of people with TS. Maximal benefit may not be achieved for 4–6 months. A small number of patients may worsen on clonidine. Guanfacine
(brand name Tenex) is another antihypertensive that is used in treating TS. Side effects can include sedation, dry mouth, fatigue, headaches and dizziness. Sedation
can be problematic when treatment is first initiated, but may wear off as the patient adjusts to the medication.
Other medications that can be used to treat tics include pergolide
(brand name Permax), botulinum toxin
, and with less empirical support for efficacy, tetrabenazine
and baclofen
.
Caffeine may trigger an exacerbation of tics in some children, so they are advised to limit its consumption.
Patients with Tourette's who are referred to specialty clinics have a high rate of comorbid attention-deficit hyperactivity disorder
(ADHD), so the treatment of ADHD co-occurring with tics is often part of the clinical treatment of Tourette's. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function, accounted for by the comorbid ADHD, highlighting the importance of identifying and treating other conditions when present.
The treatment of ADHD in the presence of tic disorders has long been a controversial topic. Past medical practice held that stimulants (such as Ritalin) could not be used in the presence of tics, due to concern that their use might worsen tics; however, multiple lines of research have shown that stimulants can be cautiously used in the presence of tic disorders. Several studies have shown that stimulants do not exacerbate tics any more than placebo does, and suggest that stimulants may even reduce tic severity. Controversy remains, and the PDR
continues to carry a warning that stimulants should not be used in the presence of tic disorders, so physicians may be reluctant to use them. Others are comfortable using them and even advocate for a stimulant trial when ADHD co-occurs with tics, because the symptoms of ADHD can be more impairing than tics.
The stimulants are the first line of treatment for ADHD, with proven efficacy, but they do fail in up to 20% of cases, even in patients without tic disorders. Current prescribed stimulant medications include: methylphenidate
(brand names Ritalin, Metadate, Concerta), dextroamphetamine
(Dexedrine), and mixed amphetamine salts (Adderall
). Other medications can be used when stimulants are not an option. These include the alpha-2 agonists (clonidine
and guanfacine
), tricyclic antidepressant
s (desipramine
and nortriptyline
), and newer antidepressants (bupropion
, venlafaxine
and atomoxetine). A retrospective
case series
published in 1993 suggested that treatment with bupropion
(trade name Wellbutrin) can worsen tics, but there is no data from placebo-controlled trials to support this. There is good empirical support for the use of desipramine
, bupropion and atomoxetine (brand name Strattera). Atomoxetine is the only non-controlled Food and Drug Administration
(FDA) approved drug for the treatment of ADHD, but is less effective than stimulants for ADHD, is associated with individual cases of liver damage, carries an FDA black box warning
regarding suicidal ideation, and controlled studies show increases in heart rate, decreases of body weight, decreased appetite and treatment-emergent nausea.
(HRT), massed negative practice
, biofeedback
, relaxation, hypnosis
and other behavioral approaches are proposed as alternative treatments of tics, but few have been well assessed. Relaxation technique
s, such as exercise, yoga or meditation, may be useful in relieving stress that may aggravate tics, but the majority of behavioral interventions (such as relaxation training and biofeedback
, with the exception of habit reversal) have not been systematically evaluated and are not proven therapies for Tourette's. Cognitive Behavioral Therapy (CBT) is a useful treatment when OCD is present, and there is increasing evidence supporting the use of habit reversal
in the treatment of tics. Acupuncture
may be beneficial for tic suppression, but studies are inadequate. Small studies have shown some lasting benefit from habit reversal training.
, have popular appeal, but no role has been proven for any of these in the treatment of Tourette syndrome. While a balanced diet may aid in overall health, and avoidance of caffeine may help minimize tics for some children, no particular diet or alternative therapy (vitamin or diet) is supported by scientific evidence. Regular exercise can help reduce stress and improve a child's sense of accomplishment and self-esteem, but the effect of exercise on symptoms remains unstudied.
Nicotine
showed preliminary promise in case studies, but these effects were not reproduced in well-controlled trials several years later. Studies of nicotine derivatives (mecamylamine
, inversine) also showed that they were not effective as a single therapy for the symptoms of Tourette's. Case reports found that marijuana helped reduce tics, but validation of these results requires longer, controlled studies on larger samples. Controlled research on treating Tourette's with a synthetic version of tetrahydrocannabinol
(brand name Marinol), the main psychoactive chemical found in cannabis
, showed the patients taking Marinol had a beneficial response without serious adverse effects.
Deep brain stimulation
has been used in treating a few patients with severe Tourette's, but is regarded as an experimental and dangerous procedure that is unlikely to become widespread. The procedure is well tolerated, but complications include "short battery life, abrupt symptom worsening upon cessation of stimulation, hypomanic or manic conversion, and the significant time and effort involved in optimizing stimulation parameters". There are currently five reports in patients with TS; all experienced reduction in tics and the disappearance of obsessive-compulsive behaviors. "Only patients with severe, debilitating, and treatment-refractory illness should be considered; while those with severe personality disorders and substance abuse problems should be excluded."
Tourette syndrome
Tourette syndrome is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical tics and at least one vocal tic; these tics characteristically wax and wane...
(also Tourette's syndrome or TS) is an inherited
Heredity
Heredity is the passing of traits to offspring . This is the process by which an offspring cell or organism acquires or becomes predisposed to the characteristics of its parent cell or organism. Through heredity, variations exhibited by individuals can accumulate and cause some species to evolve...
neuropsychiatric
Neuropsychiatry
Neuropsychiatry is the branch of medicine dealing with mental disorders attributable to diseases of the nervous system. It preceded the current disciplines of psychiatry and neurology, in as much as psychiatrists and neurologists had a common training....
disorder with onset in childhood, characterized by the presence of motor and phonic tic
Tic
A tic is a sudden, repetitive, nonrhythmic, stereotyped motor movement or vocalization involving discrete muscle groups. Tics can be invisible to the observer, such as abdominal tensing or toe crunching. Common motor and phonic tics are, respectively, eye blinking and throat clearing...
s. Treatment of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.
Tourette syndrome patients may exhibit symptoms of other comorbid conditions along with their motor and phonic tics. Associated conditions include attention-deficit hyperactivity disorder
Attention-deficit hyperactivity disorder
Attention deficit hyperactivity disorder is a developmental disorder. It is primarily characterized by "the co-existence of attentional problems and hyperactivity, with each behavior occurring infrequently alone" and symptoms starting before seven years of age.ADHD is the most commonly studied and...
(ADD or ADHD), obsessive-compulsive disorder
Obsessive-compulsive disorder
Obsessive–compulsive disorder is an anxiety disorder characterized by intrusive thoughts that produce uneasiness, apprehension, fear, or worry, by repetitive behaviors aimed at reducing the associated anxiety, or by a combination of such obsessions and compulsions...
(OCD), learning disabilities and sleep disorder
Sleep disorder
A sleep disorder, or somnipathy, is a medical disorder of the sleep patterns of a person or animal. Some sleep disorders are serious enough to interfere with normal physical, mental and emotional functioning...
s. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function. Co-occurring OCD can also be a source of impairment, necessitating treatment. Not all persons with tics will also have other conditions and not all persons with tics require treatment, but when comorbid disorders are present, they often require treatment.
Stimulants (like Adderall
Adderall
Adderall is a brand name of amphetamine salts–based medication used for attention-deficit hyperactivity disorder and narcolepsy. It is a brand-name psychostimulant medication composed of racemic amphetamine aspartate monohydrate, racemic amphetamine sulfate, dextroamphetamine saccharide, and...
and Ritalin) are underused in the treatment of ADHD when tics are also present because of unfounded fears that they increase tics. Experimental treatments like deep brain stimulation
Deep brain stimulation
Deep brain stimulation is a surgical treatment involving the implantation of a medical device called a brain pacemaker, which sends electrical impulses to specific parts of the brain...
, nicotine
Nicotine
Nicotine is an alkaloid found in the nightshade family of plants that constitutes approximately 0.6–3.0% of the dry weight of tobacco, with biosynthesis taking place in the roots and accumulation occurring in the leaves...
, marijuana and complementary and alternative medicine approaches have widespread appeal but unproven safety and efficacy.
Treatment priority
Treatment of Tourette syndrome can be divided into treatment of tics, and treatment of co-occurringComorbidity
In medicine, comorbidity is either the presence of one or more disorders in addition to a primary disease or disorder, or the effect of such additional disorders or diseases.- In medicine :...
conditions, which, when present, are often a larger source of functional impairment than the tics themselves.
There is no cure for Tourette's and no medication which works universally for all individuals without significant adverse effects; knowledge and understanding are the best treatments available for tic
Tic
A tic is a sudden, repetitive, nonrhythmic, stereotyped motor movement or vocalization involving discrete muscle groups. Tics can be invisible to the observer, such as abdominal tensing or toe crunching. Common motor and phonic tics are, respectively, eye blinking and throat clearing...
s. Management of the symptoms of Tourette's may include pharmacological
Pharmacology
Pharmacology is the branch of medicine and biology concerned with the study of drug action. More specifically, it is the study of the interactions that occur between a living organism and chemicals that affect normal or abnormal biochemical function...
, behavioral and psychological therapies. While pharmacological treatment is reserved for more severe symptoms, other types of treatments may help avoid or improve symptoms of depression
Clinical depression
Major depressive disorder is a mental disorder characterized by an all-encompassing low mood accompanied by low self-esteem, and by loss of interest or pleasure in normally enjoyable activities...
or social isolation
Social isolation
Social isolation refers to a lack of contact with society for members of social species. There may be many causes and individuals in numerous generally social species are isolated at times, it need not be a pathological condition. In human society, in those cases where it is viewed as a pathology,...
, and improve supportive family functioning. Educating the patient, family, and surrounding community (school, church, friends, etc.) is a key part of treatment.
The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. Because children with tics often present to physicians when their tics are at their highest severity, and because of the waxing and waning nature of tics, medication is not usually started immediately or changed often. Frequently, the tics subside with understanding of the condition and a supportive environment. When medication is necessary, pharmaceutical intervention should be targeted at the most impairing symptoms, taking into account co-occurring conditions such as ADHD
Attention-deficit hyperactivity disorder
Attention deficit hyperactivity disorder is a developmental disorder. It is primarily characterized by "the co-existence of attentional problems and hyperactivity, with each behavior occurring infrequently alone" and symptoms starting before seven years of age.ADHD is the most commonly studied and...
or OCD, which when present, may warrant treatment even when tics are mild.
The classes of medication with the most proven efficacy in treating tics—typical and atypical
Atypical antipsychotic
The atypical antipsychotics are a group of antipsychotic tranquilizing drugs used to treat psychiatric conditions. Some atypical antipsychotics are FDA approved for use in the treatment of schizophrenia...
neuroleptics
Antipsychotic
An antipsychotic is a tranquilizing psychiatric medication primarily used to manage psychosis , particularly in schizophrenia and bipolar disorder. A first generation of antipsychotics, known as typical antipsychotics, was discovered in the 1950s...
including risperidone
Risperidone
Risperidone is a second generation or atypical antipsychotic, sold under the trade name . It is used to treat schizophrenia , schizoaffective disorder, the mixed and manic states associated with bipolar disorder, and irritability in people with autism...
(trade name Risperdal), ziprasidone
Ziprasidone
Ziprasidone was the fifth atypical antipsychotic to gain FDA approval . In the United States, Ziprasidone is Food and Drug Administration approved for the treatment of schizophrenia, and the intramuscular injection form of ziprasidone is approved for acute agitation in schizophrenic patients...
(Geodon), haloperidol
Haloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
(Haldol), pimozide
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
(Orap) and fluphenazine
Fluphenazine
Fluphenazine is a typical antipsychotic drug used for the treatment of psychoses such as schizophrenia and acute manic phases of bipolar disorder. It belongs to the piperazine class of phenothiazines....
(Prolixin)—can have long-term and short-term adverse effects
Adverse effect (medicine)
In medicine, an adverse effect is a harmful and undesired effect resulting from a medication or other intervention such as surgery.An adverse effect may be termed a "side effect", when judged to be secondary to a main or therapeutic effect. If it results from an unsuitable or incorrect dosage or...
. The antihypertensive
Antihypertensive
The antihypertensives are a class of drugs that are used to treat hypertension . Evidence suggests that reduction of the blood pressure by 5 mmHg can decrease the risk of stroke by 34%, of ischaemic heart disease by 21%, and reduce the likelihood of dementia, heart failure, and mortality from...
agents, clonidine
Clonidine
Clonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
(Catapres) and guanfacine
Guanfacine
Guanfacine is a sympatholytic. It is an agonist of the α2A subtype of norepinephrine receptors. These receptors are concentrated heavily in the prefrontal cortex and the locus coeruleus, with the potential to improve attention abilities via modulating post-synaptic α2A receptors in the prefrontal...
(Tenex), are also used to treat tics; studies show variable efficacy, but a lower side effect profile than the neuroleptics. Stimulants and other medications may be useful in treating ADHD when it co-occurs with tic disorders. Drugs from several other classes of medications can be used when stimulant trials fail, including guanfacine
Guanfacine
Guanfacine is a sympatholytic. It is an agonist of the α2A subtype of norepinephrine receptors. These receptors are concentrated heavily in the prefrontal cortex and the locus coeruleus, with the potential to improve attention abilities via modulating post-synaptic α2A receptors in the prefrontal...
(Tenex), atomoxetine (Strattera) and tricyclics
Tricyclic antidepressant
Tricyclic antidepressants are heterocyclic chemical compounds used primarily as antidepressants. The TCAs were first discovered in the early 1950s and were subsequently introduced later in the decade; they are named after their chemical structure, which contains three rings of atoms...
. Clomipramine
Clomipramine
Clomipramine is a tricyclic antidepressant . It was developed in the 1960s by the Swiss drug manufacturer Geigy and has been in clinical use worldwide ever since.- Indications :...
(Anafranil), a tricyclic antidepressant
Tricyclic antidepressant
Tricyclic antidepressants are heterocyclic chemical compounds used primarily as antidepressants. The TCAs were first discovered in the early 1950s and were subsequently introduced later in the decade; they are named after their chemical structure, which contains three rings of atoms...
, and SSRIs
Selective serotonin reuptake inhibitor
Selective serotonin re-uptake inhibitors or serotonin-specific reuptake inhibitor are a class of compounds typically used as antidepressants in the treatment of depression, anxiety disorders, and some personality disorders. The efficacy of SSRIs is disputed...
—a class of antidepressant
Antidepressant
An antidepressant is a psychiatric medication used to alleviate mood disorders, such as major depression and dysthymia and anxiety disorders such as social anxiety disorder. According to Gelder, Mayou &*Geddes people with a depressive illness will experience a therapeutic effect to their mood;...
s including fluoxetine
Fluoxetine
Fluoxetine is an antidepressant of the selective serotonin reuptake inhibitor class. It is manufactured and marketed by Eli Lilly and Company...
(Prozac), sertraline
Sertraline
Sertraline hydrochloride is an antidepressant of the selective serotonin reuptake inhibitor class. It was introduced to the market by Pfizer in 1991. Sertraline is primarily used to treat major depression in adult outpatients as well as obsessive–compulsive, panic, and social anxiety disorders in...
(Zoloft), and fluvoxamine
Fluvoxamine
Fluvoxamine is an antidepressant which functions as a selective serotonin reuptake inhibitor . Fluvoxamine was first approved by the U.S. Food and Drug Administration in 1993 for the treatment of obsessive compulsive disorder . Fluvoxamine CR is approved to treat social anxiety disorder...
(Luvox)—may be prescribed when a Tourette's patient also has symptoms of obsessive–compulsive disorder.
Treatment of tics
Antipsychotic
An antipsychotic is a tranquilizing psychiatric medication primarily used to manage psychosis , particularly in schizophrenia and bipolar disorder. A first generation of antipsychotics, known as typical antipsychotics, was discovered in the 1950s...
s (for example, pimozide
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
) have been FDA
Food and Drug Administration
The Food and Drug Administration is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments...
-approved for treating Tourette's. Medications which are used as primary treatment in other conditions are used with some success in treating tics. Neuroleptic medications (antipsychotics), such as haloperidol
Haloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
(brand name Haldol
Haloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
) or pimozide
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
(brand name Orap
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
), have historically been and continue to be the medications with the most proven efficacy in controlling tics. These medications work by blocking dopamine receptor
Dopamine receptor
Dopamine receptors are a class of metabotropic G protein-coupled receptors that are prominent in the vertebrate central nervous system . The neurotransmitter dopamine is the primary endogenous ligand for dopamine receptors....
s, and are associated with a high side effect profile. The traditional antipsychotic drugs are associated with tardive dyskinesia
Tardive dyskinesia
Tardive dyskinesia is a difficult-to-treat form of dyskinesia that can be tardive...
when used long-term; and parkinsonism
Parkinsonism
Parkinsonism is a neurological syndrome characterized by tremor, hypokinesia, rigidity, and postural instability. The underlying causes of parkinsonism are numerous, and diagnosis can be complex...
, dystonia
Dystonia
Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning or reaction to...
, dyskinesia
Dyskinesia
Dyskinesia is a movement disorder which consists of effects including diminished voluntary movements and the presence of involuntary movements, similar to tics or choreia. Dyskinesia can be anything from a slight tremor of the hands to uncontrollable movement of, most commonly, the upper body but...
, and akathisia
Akathisia
Akathisia, or acathisia, is a syndrome characterized by unpleasant sensations of inner restlessness that manifests itself with an inability to sit still or remain motionless...
when used short-term. Additional side effects can be school phobia (a form of separation anxiety
Separation anxiety
Separation anxiety may refer to:*Separation anxiety disorder or Separation anxiety test*Spider-Man & Venom: Separation Anxiety, a 1995 SNES video game*Separation Anxieties, a 2000 album by 12 Rods...
), depression
Clinical depression
Major depressive disorder is a mental disorder characterized by an all-encompassing low mood accompanied by low self-esteem, and by loss of interest or pleasure in normally enjoyable activities...
, weight gain, and cognitive blunting (dulling of cognitive
Cognition
In science, cognition refers to mental processes. These processes include attention, remembering, producing and understanding language, solving problems, and making decisions. Cognition is studied in various disciplines such as psychology, philosophy, linguistics, and computer science...
ability). Another traditional antipsychotic used in treating Tourette's is fluphenazine
Fluphenazine
Fluphenazine is a typical antipsychotic drug used for the treatment of psychoses such as schizophrenia and acute manic phases of bipolar disorder. It belongs to the piperazine class of phenothiazines....
(brand name Prolixin), although the evidence supporting its use is less than that of haloperidol
Haloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
and pimozide
Pimozide
Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine . On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome...
.
Newer neuroleptics, the atypical neuroleptics, are an alternative to the traditional medications used for treating tics. These medications have more selective dopamine blocking effects, or block serotonin
Serotonin
Serotonin or 5-hydroxytryptamine is a monoamine neurotransmitter. Biochemically derived from tryptophan, serotonin is primarily found in the gastrointestinal tract, platelets, and in the central nervous system of animals including humans...
with some blocking of dopamine. The medications in this class used to treat tics include risperidone
Risperidone
Risperidone is a second generation or atypical antipsychotic, sold under the trade name . It is used to treat schizophrenia , schizoaffective disorder, the mixed and manic states associated with bipolar disorder, and irritability in people with autism...
(brand name Risperdal), olanzapine
Olanzapine
Olanzapine is an atypical antipsychotic, approved by the FDA for the treatment of schizophrenia and bipolar disorder...
(brand name Zyprexa), ziprasidone
Ziprasidone
Ziprasidone was the fifth atypical antipsychotic to gain FDA approval . In the United States, Ziprasidone is Food and Drug Administration approved for the treatment of schizophrenia, and the intramuscular injection form of ziprasidone is approved for acute agitation in schizophrenic patients...
(brand name Geodon), quetiapine
Quetiapine
Quetiapine , is an atypical antipsychotic approved for the treatment of schizophrenia, and bipolar disorder....
(brand name Seroquel), clozapine
Clozapine
Clozapine is an antipsychotic medication used in the treatment of schizophrenia, and is also used off-label in the treatment of bipolar disorder. Wyatt. R and Chew...
(brand name Clozaril), tiapride, and sulpiride
Sulpiride
Sulpiride is an...
. They seem to have lower risks of neurological side effects (such as tardive dyskinesia
Tardive dyskinesia
Tardive dyskinesia is a difficult-to-treat form of dyskinesia that can be tardive...
) when used short-term, but longer trials are needed to confirm this. Some of the side effects associated with these medications are insomnia
Insomnia
Insomnia is most often defined by an individual's report of sleeping difficulties. While the term is sometimes used in sleep literature to describe a disorder demonstrated by polysomnographic evidence of disturbed sleep, insomnia is often defined as a positive response to either of two questions:...
, weight gain, and school phobia. Abnormalities in metabolism, cardiac conduction
Electrical conduction system of the heart
The normal intrinsic electrical conduction of the heart allows electrical propagation to be transmitted from the Sinoatrial Node through both atria and forward to the Atrioventricular Node. Normal/baseline physiology allows further propagation from the AV node to the ventricle or Purkinje Fibers...
times, and increased risk of diabetes are concerns with these medications. There is good empirical support for the use of risperidone
Risperidone
Risperidone is a second generation or atypical antipsychotic, sold under the trade name . It is used to treat schizophrenia , schizoaffective disorder, the mixed and manic states associated with bipolar disorder, and irritability in people with autism...
, and less support for the others.
Antihypertensive
The antihypertensives are a class of drugs that are used to treat hypertension . Evidence suggests that reduction of the blood pressure by 5 mmHg can decrease the risk of stroke by 34%, of ischaemic heart disease by 21%, and reduce the likelihood of dementia, heart failure, and mortality from...
agents) show some efficacy in reducing tics, as well as other comorbid features of some people with Tourette's. Originally developed to treat high blood pressure, these medications are a safer alternative to neuroleptic medications for the people with TS that respond to them. This class of medication is often the first tried for tics, as the antihypertensives have a lower side effect profile than some of the medications which more proven efficacy. The evidence for their safety and efficacy is not as strong as the evidence for some of the standard and atypical neuroleptics, but there is fair supportive evidence for their use, nonetheless. This class of medication takes about six weeks to begin to work on tics, so sustained trials are warranted. Because of the blood pressure effects, antihypertensive agents should not be discontinued suddenly. Clonidine
Clonidine
Clonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
(brand name Catapres) works on tics for about half of people with TS. Maximal benefit may not be achieved for 4–6 months. A small number of patients may worsen on clonidine. Guanfacine
Guanfacine
Guanfacine is a sympatholytic. It is an agonist of the α2A subtype of norepinephrine receptors. These receptors are concentrated heavily in the prefrontal cortex and the locus coeruleus, with the potential to improve attention abilities via modulating post-synaptic α2A receptors in the prefrontal...
(brand name Tenex) is another antihypertensive that is used in treating TS. Side effects can include sedation, dry mouth, fatigue, headaches and dizziness. Sedation
Sedation
Sedation is the reduction of irritability or agitation by administration of sedative drugs, generally to facilitate a medical procedure or diagnostic procedure...
can be problematic when treatment is first initiated, but may wear off as the patient adjusts to the medication.
Other medications that can be used to treat tics include pergolide
Pergolide
Pergolide is an ergoline-based dopamine receptor agonist used in some countries for the treatment of Parkinson's disease....
(brand name Permax), botulinum toxin
Botulinum toxin
Botulinum toxin is a protein produced by the bacterium Clostridium botulinum, and is considered the most powerful neurotoxin ever discovered. Botulinum toxin causes Botulism poisoning, a serious and life-threatening illness in humans and animals...
, and with less empirical support for efficacy, tetrabenazine
Tetrabenazine
Tetrabenazine is a drug for the symptomatic treatment of hyperkinetic movement disorder and is marketed under the trade names Nitoman in Canada and Xenazine in New Zealand and some parts of Europe, and is also available in the USA as an orphan drug. On August 15, 2008 the U.S...
and baclofen
Baclofen
Baclofen is a derivative of gamma-aminobutyric acid . It is primarily used to treat spasticity and is under investigation for the treatment of alcoholism....
.
Caffeine may trigger an exacerbation of tics in some children, so they are advised to limit its consumption.
Treatment of ADHD in the presence of tic disorders
Attention-deficit hyperactivity disorder
Attention deficit hyperactivity disorder is a developmental disorder. It is primarily characterized by "the co-existence of attentional problems and hyperactivity, with each behavior occurring infrequently alone" and symptoms starting before seven years of age.ADHD is the most commonly studied and...
(ADHD), so the treatment of ADHD co-occurring with tics is often part of the clinical treatment of Tourette's. Patients who have ADHD along with Tourette's may also have problems with disruptive behaviors, overall functioning, and cognitive function, accounted for by the comorbid ADHD, highlighting the importance of identifying and treating other conditions when present.
The treatment of ADHD in the presence of tic disorders has long been a controversial topic. Past medical practice held that stimulants (such as Ritalin) could not be used in the presence of tics, due to concern that their use might worsen tics; however, multiple lines of research have shown that stimulants can be cautiously used in the presence of tic disorders. Several studies have shown that stimulants do not exacerbate tics any more than placebo does, and suggest that stimulants may even reduce tic severity. Controversy remains, and the PDR
Physicians' Desk Reference
The Physicians' Desk Reference is a commercially published compilation of manufacturers' prescribing information on prescription drugs, updated annually...
continues to carry a warning that stimulants should not be used in the presence of tic disorders, so physicians may be reluctant to use them. Others are comfortable using them and even advocate for a stimulant trial when ADHD co-occurs with tics, because the symptoms of ADHD can be more impairing than tics.
The stimulants are the first line of treatment for ADHD, with proven efficacy, but they do fail in up to 20% of cases, even in patients without tic disorders. Current prescribed stimulant medications include: methylphenidate
Methylphenidate
Methylphenidate is a psychostimulant drug approved for treatment of attention-deficit hyperactivity disorder, postural orthostatic tachycardia syndrome and narcolepsy. It may also be prescribed for off-label use in treatment-resistant cases of lethargy, depression, neural insult and obesity...
(brand names Ritalin, Metadate, Concerta), dextroamphetamine
Dextroamphetamine
Dextroamphetamine is a psychostimulant drug which is known to produce increased wakefulness and focus as well as decreased fatigue and decreased appetite....
(Dexedrine), and mixed amphetamine salts (Adderall
Adderall
Adderall is a brand name of amphetamine salts–based medication used for attention-deficit hyperactivity disorder and narcolepsy. It is a brand-name psychostimulant medication composed of racemic amphetamine aspartate monohydrate, racemic amphetamine sulfate, dextroamphetamine saccharide, and...
). Other medications can be used when stimulants are not an option. These include the alpha-2 agonists (clonidine
Clonidine
Clonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
and guanfacine
Guanfacine
Guanfacine is a sympatholytic. It is an agonist of the α2A subtype of norepinephrine receptors. These receptors are concentrated heavily in the prefrontal cortex and the locus coeruleus, with the potential to improve attention abilities via modulating post-synaptic α2A receptors in the prefrontal...
), tricyclic antidepressant
Tricyclic antidepressant
Tricyclic antidepressants are heterocyclic chemical compounds used primarily as antidepressants. The TCAs were first discovered in the early 1950s and were subsequently introduced later in the decade; they are named after their chemical structure, which contains three rings of atoms...
s (desipramine
Desipramine
Desipramine is a tricyclic antidepressant . It inhibits the reuptake of norepinephrine and to a lesser extent serotonin. It is used to treat depression, but not considered a first line treatment since the introduction of SSRI antidepressants...
and nortriptyline
Nortriptyline
Nortriptyline is a second-generation tricyclic antidepressant marketed as the hydrochloride salt under the trade names Sensoval, Aventyl, Pamelor, Norpress, Allegron, Noritren and Nortrilen. It is used in the treatment of major depression and childhood nocturnal enuresis...
), and newer antidepressants (bupropion
Bupropion
Bupropion is an atypical antidepressant and smoking cessation aid. The drug is a non-tricyclic antidepressant and differs from most commonly prescribed antidepressants such as SSRIs, as its primary pharmacological action is thought to be norepinephrine-dopamine reuptake inhibition...
, venlafaxine
Venlafaxine
Venlafaxine is an antidepressant of the serotonin-norepinephrine reuptake inhibitor class. First introduced by Wyeth in 1993, now marketed by Pfizer, it is licensed for the treatment of major depressive disorder , as a treatment for generalized anxiety disorder, and comorbid indications in...
and atomoxetine). A retrospective
Retrospective
Retrospective generally means to take a look back at events that already have taken place. For example, the term is used in medicine, describing a look back at a patient's medical history or lifestyle.-Music:...
case series
Case series
A case series is a medical research descriptive study that tracks patients with a known exposure given similar treatment or examines their medical records for exposure and outcome. It can be retrospective or prospective and usually involves a smaller number of patients than more powerful...
published in 1993 suggested that treatment with bupropion
Bupropion
Bupropion is an atypical antidepressant and smoking cessation aid. The drug is a non-tricyclic antidepressant and differs from most commonly prescribed antidepressants such as SSRIs, as its primary pharmacological action is thought to be norepinephrine-dopamine reuptake inhibition...
(trade name Wellbutrin) can worsen tics, but there is no data from placebo-controlled trials to support this. There is good empirical support for the use of desipramine
Desipramine
Desipramine is a tricyclic antidepressant . It inhibits the reuptake of norepinephrine and to a lesser extent serotonin. It is used to treat depression, but not considered a first line treatment since the introduction of SSRI antidepressants...
, bupropion and atomoxetine (brand name Strattera). Atomoxetine is the only non-controlled Food and Drug Administration
Food and Drug Administration
The Food and Drug Administration is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments...
(FDA) approved drug for the treatment of ADHD, but is less effective than stimulants for ADHD, is associated with individual cases of liver damage, carries an FDA black box warning
Black box warning
In the United States, a black box warning is a type of warning that appears on the package insert for prescription drugs that may cause serious adverse effects...
regarding suicidal ideation, and controlled studies show increases in heart rate, decreases of body weight, decreased appetite and treatment-emergent nausea.
Behavioral treatments
Habit reversal trainingHabit reversal training
Habit reversal training is a "multicomponent behavioral treatment package originally developed to address a wide variety of repetitive behavior disorders"....
(HRT), massed negative practice
Massed negative practice
Massed negative practice is a proposed treatment for the tics of Tourette syndrome in which the individual with Tourette's "practices" tics continuously until a conditioned level of fatigue is reached...
, biofeedback
Biofeedback
Biofeedback is the process of becoming aware of various physiological functions using instruments that provide information on the activity of those same systems, with a goal of being able to manipulate them at will...
, relaxation, hypnosis
Hypnosis
Hypnosis is "a trance state characterized by extreme suggestibility, relaxation and heightened imagination."It is a mental state or imaginative role-enactment . It is usually induced by a procedure known as a hypnotic induction, which is commonly composed of a long series of preliminary...
and other behavioral approaches are proposed as alternative treatments of tics, but few have been well assessed. Relaxation technique
Relaxation technique
A relaxation technique is any method, process, procedure, or activity that helps a person to relax; to attain a state of increased calmness; or otherwise reduce levels of anxiety, stress or anger...
s, such as exercise, yoga or meditation, may be useful in relieving stress that may aggravate tics, but the majority of behavioral interventions (such as relaxation training and biofeedback
Biofeedback
Biofeedback is the process of becoming aware of various physiological functions using instruments that provide information on the activity of those same systems, with a goal of being able to manipulate them at will...
, with the exception of habit reversal) have not been systematically evaluated and are not proven therapies for Tourette's. Cognitive Behavioral Therapy (CBT) is a useful treatment when OCD is present, and there is increasing evidence supporting the use of habit reversal
Habit reversal training
Habit reversal training is a "multicomponent behavioral treatment package originally developed to address a wide variety of repetitive behavior disorders"....
in the treatment of tics. Acupuncture
Acupuncture
Acupuncture is a type of alternative medicine that treats patients by insertion and manipulation of solid, generally thin needles in the body....
may be beneficial for tic suppression, but studies are inadequate. Small studies have shown some lasting benefit from habit reversal training.
Experimental treatments
Complementary and alternative medicine approaches, such as dietary modification, allergy testing and allergen control, and neurofeedbackNeurofeedback
Neurofeedback , also called neurotherapy, neurobiofeedback or EEG biofeedback is a type of biofeedback that uses realtime displays of electroencephalography or functional magnetic resonance imaging to illustrate brain activity, often with a goal of controlling central nervous system activity...
, have popular appeal, but no role has been proven for any of these in the treatment of Tourette syndrome. While a balanced diet may aid in overall health, and avoidance of caffeine may help minimize tics for some children, no particular diet or alternative therapy (vitamin or diet) is supported by scientific evidence. Regular exercise can help reduce stress and improve a child's sense of accomplishment and self-esteem, but the effect of exercise on symptoms remains unstudied.
Nicotine
Nicotine
Nicotine is an alkaloid found in the nightshade family of plants that constitutes approximately 0.6–3.0% of the dry weight of tobacco, with biosynthesis taking place in the roots and accumulation occurring in the leaves...
showed preliminary promise in case studies, but these effects were not reproduced in well-controlled trials several years later. Studies of nicotine derivatives (mecamylamine
Mecamylamine
Mecamylamine is a nonselective and noncompetitive antagonist of the nicotinic acetylcholine receptors that was introduced in the 1950s as an antihypertensive agent.- Uses :...
, inversine) also showed that they were not effective as a single therapy for the symptoms of Tourette's. Case reports found that marijuana helped reduce tics, but validation of these results requires longer, controlled studies on larger samples. Controlled research on treating Tourette's with a synthetic version of tetrahydrocannabinol
Tetrahydrocannabinol
Tetrahydrocannabinol , also known as delta-9-tetrahydrocannabinol , Δ1-THC , or dronabinol, is the main chemical psychoactive substance found in the cannabis plant. It was first isolated in 1964. In pure form, it is a glassy solid when cold, and becomes viscous and sticky if warmed...
(brand name Marinol), the main psychoactive chemical found in cannabis
Cannabis
Cannabis is a genus of flowering plants that includes three putative species, Cannabis sativa, Cannabis indica, and Cannabis ruderalis. These three taxa are indigenous to Central Asia, and South Asia. Cannabis has long been used for fibre , for seed and seed oils, for medicinal purposes, and as a...
, showed the patients taking Marinol had a beneficial response without serious adverse effects.
Deep brain stimulation
Deep brain stimulation
Deep brain stimulation is a surgical treatment involving the implantation of a medical device called a brain pacemaker, which sends electrical impulses to specific parts of the brain...
has been used in treating a few patients with severe Tourette's, but is regarded as an experimental and dangerous procedure that is unlikely to become widespread. The procedure is well tolerated, but complications include "short battery life, abrupt symptom worsening upon cessation of stimulation, hypomanic or manic conversion, and the significant time and effort involved in optimizing stimulation parameters". There are currently five reports in patients with TS; all experienced reduction in tics and the disappearance of obsessive-compulsive behaviors. "Only patients with severe, debilitating, and treatment-refractory illness should be considered; while those with severe personality disorders and substance abuse problems should be excluded."
External links
- NINDS. Methylphenidate and Clonidine Help Children With ADHD and Tics. (April 2, 2002)
- Packer, Leslie. Treatment of Tics and Tourette's syndrome.