David Baxter PhD
Late Founder
The Tics of Tourette's Often Go Undiagnosed
January 18, 2005
By JANE E. BRODY, New York Times
A woman who lived for years in my neighborhood periodically appeared at a window and shouted obscenities into the street. Passers-by were appalled, but I felt what had to be the painful humiliation of someone who had no ability to control this seemingly antisocial behavior.
I realized that the woman was afflicted with Tourette's syndrome, a lifelong neurological disorder with symptoms that contrary to popular belief, only rarely include the involuntary shouting of obscenities.
I now know that the disorder is associated with a wide range of confusing symptoms that often result in delays in diagnosis and treatment that can last years. The problem was eloquently described in a two-part article last August in Contemporary Pediatrics.
In his report, Dr. Samuel H. Zinner, a pediatrician at the University of Washington specializing in developmental and behavioral problems, points out that the syndrome "often goes undiagnosed or misdiagnosed."
"Misconceptions about this tic disorder are customary," he adds, "with the syndrome often perceived as characterized by bizarre, fitful behaviors or comical outbursts of uncontrollable profanity."
The fact is that "relatively few patients yell out obscenities," Dr. Zinner said, adding that "most patients are affected only mildly and usually escape notice," even by their doctors. Complicating the diagnostic puzzle is the ability of patients, consciously or otherwise, to suppress their tics when expressing them could be embarrassing, as would occur in visits to the doctor.
Dr. Zinner adds that the tics of Tourette's "range widely in their severity, form, frequency and intensity" and are associated with other disorders that "are usually more serious or disabling than the tics themselves." These may include attention deficit hyperactivity disorder, obsessive-compulsive disorder, learning disabilities, anxiety or mood disorders and difficulty sleeping.
These associated problems are often what first brings the problem to medical attention and may result in doctors' overlooking the underlying tic disorder.
The disorder was first medically described in 1885 by the French neurologist Georges Gilles de la Tourette. But until the 1960's it, like other conditions later found to have neurological bases, was incorrectly viewed as a psychological problem. Psychotherapy cannot cure it and, despite what doctors and therapists sometimes tell parents of patients, people do not outgrow it.
Dr. Zinner says the disorder is far more common than is generally recognized, even by the Tourette Syndrome Association, which estimates that 1 person in 2,000 is affected. Rather, recent studies suggest that the real number of those with chronic tics is more like 1 in 100, suggesting that 750,000 children in this country have Tourette's. The disorder affects four times as many boys as girls and often runs in families.
Despite having chronic brain disorders that often interfere with learning, children with Tourette's do not quality for coverage in most states under the Individuals With Disabilities Education Act, which helps finance special education.
A Wide Variety of Tics
People with Tourette's are often ridiculed and stigmatized as weird, scary or even crazy, depending on the nature of their tics and how obvious, complex and uncontrollable they may be.
Tics come in many forms, both "phonic" (sound-producing) and "motor" (abnormal movements), and each can be either simple or complex. Simple phonic tics include chronic sniffing, grunting, throat clearing, clicking and screaming. Complex ones include speech interruptions like stuttering and repetition of single words or phrases and coprolalia, the expression of socially unacceptable words or phrases.
Simple motor tics may manifest as eye blinking, nose wrinkling, jaw thrusting, shoulder shrugging or neck jerking, or the tics may involve more complex movements like jumping, touching, twirling when walking, retracing steps, imitating someone else's movements or making sudden obscene gestures.
Symptoms typically become more complex with time. They usually first appear in early childhood, by age 6 or 7, as simple motor tics, with phonic tics and more complex motor tics developing in the next few years or perhaps not until adolescence.
Expression of tics typically occurs in bouts that may be separated by seconds or minutes, weeks or even months. Their expression is often preceded by a premonitory urge, not unlike a worsening itch that finally demands to be scratched. Performing the tic brings temporary relief.
Consciously or otherwise, people with Tourette's often learn to suppress their tics, but there is a cost. The discomfort and distraction caused by the unfulfilled urge can be more disruptive than the tic itself. When suppressed tics are finally released, they are often more intense and frequent.
Certain stimuli - like noise, a word or an image - may provoke the urge for tics, and conditions like stress, anxiety, fatigue and intense emotional excitement can prompt their expression.
More than half of Tourette's patients have attention deficit hyperactivity disorder, and signs of it may appear even before the tics. Other neurological problems associated with the disorder include loss of impulse control, obsessive thoughts and compulsive behaviors, learning disabilities and difficulty organizing, planning, making decisions and following rules.
People with Tourette's may also suffer from generalized anxiety, phobias, panic attacks, depression and sleep disorders. A few experience sudden explosive attacks of rage.
Suppressing Symptoms
Some people with Tourette's saw a glimmer of hope in a recent report of one man with debilitating symptoms of Tourette's that were immediately relieved by deep brain stimulation. This treatment involves the implantation of electrodes into the part of the brain that controls movements.
The electrodes are attached by wires to a pacemaker implanted in the chest, a technique used with some success to curb the abnormal movements of Parkinson's disease.
The technique has been tried on several patients with Tourette's, but it is still highly experimental, with no data yet on its overall effectiveness, possible complications, side effects or duration of benefit.
Another relatively new approach for severe, very specific tics involves temporarily paralyzing the affected muscle group by injecting it with Botox (botulinum toxin), which can suppress the tic for several months.
Meanwhile, depending on the nature and severity of a patient's symptoms, medications are available to treat them. They include alpha-adrenergic agonists like guanfacine (Tenex), neuroleptics like haloperidol (Haldol) and benzodiazepine clonazepam (Klonopin).
As with all drugs, there are side effects, and Dr. Zinner urges the use of the lowest dosages and fewest drugs needed to help a person function effectively. Because tics may wax and wane, months of therapy may be needed when trying new medications to accurately determine their effectiveness.
Dr. Zinner, however, cautioned against a common tendency for families to use a variety of alternative remedies and dietary restrictions, since none have proved useful.
Information from the Tourette Syndrome Association is online at Home or at Home - Tourette Canada.
January 18, 2005
By JANE E. BRODY, New York Times
A woman who lived for years in my neighborhood periodically appeared at a window and shouted obscenities into the street. Passers-by were appalled, but I felt what had to be the painful humiliation of someone who had no ability to control this seemingly antisocial behavior.
I realized that the woman was afflicted with Tourette's syndrome, a lifelong neurological disorder with symptoms that contrary to popular belief, only rarely include the involuntary shouting of obscenities.
I now know that the disorder is associated with a wide range of confusing symptoms that often result in delays in diagnosis and treatment that can last years. The problem was eloquently described in a two-part article last August in Contemporary Pediatrics.
In his report, Dr. Samuel H. Zinner, a pediatrician at the University of Washington specializing in developmental and behavioral problems, points out that the syndrome "often goes undiagnosed or misdiagnosed."
"Misconceptions about this tic disorder are customary," he adds, "with the syndrome often perceived as characterized by bizarre, fitful behaviors or comical outbursts of uncontrollable profanity."
The fact is that "relatively few patients yell out obscenities," Dr. Zinner said, adding that "most patients are affected only mildly and usually escape notice," even by their doctors. Complicating the diagnostic puzzle is the ability of patients, consciously or otherwise, to suppress their tics when expressing them could be embarrassing, as would occur in visits to the doctor.
Dr. Zinner adds that the tics of Tourette's "range widely in their severity, form, frequency and intensity" and are associated with other disorders that "are usually more serious or disabling than the tics themselves." These may include attention deficit hyperactivity disorder, obsessive-compulsive disorder, learning disabilities, anxiety or mood disorders and difficulty sleeping.
These associated problems are often what first brings the problem to medical attention and may result in doctors' overlooking the underlying tic disorder.
The disorder was first medically described in 1885 by the French neurologist Georges Gilles de la Tourette. But until the 1960's it, like other conditions later found to have neurological bases, was incorrectly viewed as a psychological problem. Psychotherapy cannot cure it and, despite what doctors and therapists sometimes tell parents of patients, people do not outgrow it.
Dr. Zinner says the disorder is far more common than is generally recognized, even by the Tourette Syndrome Association, which estimates that 1 person in 2,000 is affected. Rather, recent studies suggest that the real number of those with chronic tics is more like 1 in 100, suggesting that 750,000 children in this country have Tourette's. The disorder affects four times as many boys as girls and often runs in families.
Despite having chronic brain disorders that often interfere with learning, children with Tourette's do not quality for coverage in most states under the Individuals With Disabilities Education Act, which helps finance special education.
A Wide Variety of Tics
People with Tourette's are often ridiculed and stigmatized as weird, scary or even crazy, depending on the nature of their tics and how obvious, complex and uncontrollable they may be.
Tics come in many forms, both "phonic" (sound-producing) and "motor" (abnormal movements), and each can be either simple or complex. Simple phonic tics include chronic sniffing, grunting, throat clearing, clicking and screaming. Complex ones include speech interruptions like stuttering and repetition of single words or phrases and coprolalia, the expression of socially unacceptable words or phrases.
Simple motor tics may manifest as eye blinking, nose wrinkling, jaw thrusting, shoulder shrugging or neck jerking, or the tics may involve more complex movements like jumping, touching, twirling when walking, retracing steps, imitating someone else's movements or making sudden obscene gestures.
Symptoms typically become more complex with time. They usually first appear in early childhood, by age 6 or 7, as simple motor tics, with phonic tics and more complex motor tics developing in the next few years or perhaps not until adolescence.
Expression of tics typically occurs in bouts that may be separated by seconds or minutes, weeks or even months. Their expression is often preceded by a premonitory urge, not unlike a worsening itch that finally demands to be scratched. Performing the tic brings temporary relief.
Consciously or otherwise, people with Tourette's often learn to suppress their tics, but there is a cost. The discomfort and distraction caused by the unfulfilled urge can be more disruptive than the tic itself. When suppressed tics are finally released, they are often more intense and frequent.
Certain stimuli - like noise, a word or an image - may provoke the urge for tics, and conditions like stress, anxiety, fatigue and intense emotional excitement can prompt their expression.
More than half of Tourette's patients have attention deficit hyperactivity disorder, and signs of it may appear even before the tics. Other neurological problems associated with the disorder include loss of impulse control, obsessive thoughts and compulsive behaviors, learning disabilities and difficulty organizing, planning, making decisions and following rules.
People with Tourette's may also suffer from generalized anxiety, phobias, panic attacks, depression and sleep disorders. A few experience sudden explosive attacks of rage.
Suppressing Symptoms
Some people with Tourette's saw a glimmer of hope in a recent report of one man with debilitating symptoms of Tourette's that were immediately relieved by deep brain stimulation. This treatment involves the implantation of electrodes into the part of the brain that controls movements.
The electrodes are attached by wires to a pacemaker implanted in the chest, a technique used with some success to curb the abnormal movements of Parkinson's disease.
The technique has been tried on several patients with Tourette's, but it is still highly experimental, with no data yet on its overall effectiveness, possible complications, side effects or duration of benefit.
Another relatively new approach for severe, very specific tics involves temporarily paralyzing the affected muscle group by injecting it with Botox (botulinum toxin), which can suppress the tic for several months.
Meanwhile, depending on the nature and severity of a patient's symptoms, medications are available to treat them. They include alpha-adrenergic agonists like guanfacine (Tenex), neuroleptics like haloperidol (Haldol) and benzodiazepine clonazepam (Klonopin).
As with all drugs, there are side effects, and Dr. Zinner urges the use of the lowest dosages and fewest drugs needed to help a person function effectively. Because tics may wax and wane, months of therapy may be needed when trying new medications to accurately determine their effectiveness.
Dr. Zinner, however, cautioned against a common tendency for families to use a variety of alternative remedies and dietary restrictions, since none have proved useful.
Information from the Tourette Syndrome Association is online at Home or at Home - Tourette Canada.
