Tuesday, August 23, 2011

Managing Tics and Tourette's

Tic disorders are characterized by sudden, repetitive, involuntary movements or vocalizations which appear out of context and usually last a brief second. These disorders must appear before the age of 18, they are most common in Caucasian males and cannot result from other causes such as the ingestion of stimulants or other medical conditions. Tics are either transient with a duration of less than 12 consecutive months or chronic lasting more than a year. They are fairly common in childhood and the majority of cases resolve on their own. However, in some children the tics may persist and become more complex and severe. They can be either primary (unknown cause) or secondary to things such as anxiety, stress or fatigue. Common motor tics include blinking, jerking, picking, grunting, sniffing and shoulder shrugs. Vocal tics are less common and include sounds such as coughing, grunting, belching and throat clearing. Complex tics are slower, longer, and more purposeful; they include facial grimacing, biting, banging, arm or hand flapping, coprolalia (use of obscene words) or copropraxia (obscene gestures). The best known of these disorders is Tourette's syndrome. 

Types of tic disorders

Transient (simple) tic disorder: most commonly appears at a young age and affects between 10% and 20% of school-age children. Transient tics are characterized by the presence of one or more tics, usually motor, for at least one month but less than one year. Children may experience multiple episodes of these transient tics which will usually vary in intensity over time.
Chronic tics: will last for a year or more. During that time, the patient is never without symptoms for more than three consecutive months. They may be either motor or vocal, but not both, and the symptoms must begin before age 18. The severity of the symptoms and impairment is usually much less than for patients with Tourette's disorder. Chronic tics are much less common and occur in less than one in 100 children (1%).
Tourette's syndrome: occurs less common, is more severe and is characterized by the presence of both motor and vocal tics. The estimate is that around 250,000 (one in a thousand or less) people in the U.S. have this condition. Tourette's is three or four times more common in males than females and symptoms typically begin between ages 7 and 17. In 75% of Tourette's patients, the symptoms appear by age 10 or so. The severity of Tourette's syndrome often changes over time, waxing and waining. The tics occur many times a day, usually in bouts, most days or intermittently for more than one year. Fortunately, the symptoms often improve as Tourette's patients get older. Tourette's syndrome may also be more likely to occur in children whose mothers smoked or drank alcohol in pregnancy or were low birth weight infants. Adolescents with Tourette's frequently experience a number of additional problems including: aggressiveness, behavioral disorders, self-harming behaviors,  immaturity, social withdrawal, physical complaints, psychological disorders including anxiety or panic attacks, stuttering, sleep disorders, and inappropriate sexual behaviors.

Types of phonic tics: 1) simple phonic tics which are meaningless sounds or noises like throat clearing, coughing or sniffling and 2) complex phonic tics which include syllables, words or phrases. The "echo phenomenon" is the immediate repetition of one's or another individual's words. Coprolalia (rare, 10% or less of tic disorders) is made up of inappropriate, obscene or aggressive words. It often appears first in adolescence and causes considerable distress for individuals, teachers and their families.

Younger children (under the age of 10 or so) with simple tics will generally find them difficult to control, whereas, most older people can suppress them for varying lengths of time. Often, tics are more obvious in a relaxed situation, such as watching television. Tics may be worsened by things such as stress, anxiety, illness, fatigue or excitement. Additives in food (ie, red dye 40), certain medications or stimulants may worsen tics. The symptoms of tic disorders are usually absent while sleeping. Playing a sport or concentrating on an enjoyable task (ie, books, puzzles...) are known to reduce the severity and frequency of tics.

Tics seem to worsen during the adolescent years as the symptoms become more unpredictable from day to day. Teenagers may often refuse to go to school when their tics are severe. Older children with more complex tics frequently describe feelings of strong urges relieved by the performance of a motor tic in that particular area.  A sensation of relief and reduction in anxiety levels generally follows the tic. Many sufferers also describe an inner conflict over whether or not to give in to these urges. The energy required to suppress the tics may also contribute to anxiety, preoccupation, fatigue and social withdrawal. Low self-esteem and feelings of hopelessness are also common with tic disorders.

Causes
No definitive cause of tics has been discovered; but it is believed that abnormal activity of the neurotransmitters dopamine and serotonin (chemical messengers in the brain ) may be the cause. Multi factorial genetic factors are present in about 75% of cases. Researchers also believe there is an link between genetic and environmental factors. Tic disorders may be worsened by recreational drugs such as amphetamines or cocaine or certain prescription stimulants such as Ritalin or Focalin. Occasionally, medications such as antihistamines, antidepressants, antiseizure medications, and opiates have been shown to worsen tics. Some tics may be triggered by ones environment; a barking dog may initiate a barking tic or cough may continue as vocal tic.

PANDAS: stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus. In some cases tic disorders and obsessive-compulsive disorders have been shown to develop after streptococcal infections between the ages of two and eight. No precise cause for this connection has been found, although it appears to be related to the immune system and basal ganglia of the brain. Tics will often worsen with subsequent strep infections. The treatment is different and somewhat controversial if PANDAS is suspected in that intravenous immunoglobulin (IVIG), 750 mg/kg/day for two consecutive days, may be administered and the patient may then be placed on supressive antibiotics.
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Diagnosis

There are no diagnostic laboratory tests to screen for tic disorders. Physical and neurological examinations are generally normal, except for the tics. Surprisingly, it is also common for the patient to have fewer tics in a doctor's office than at home. The diagnosis can be made by directly observing or videotaping the patient in a more natural setting. Children quickly learn to mask their symptoms and convert them to more socially acceptable movements and sounds. 

Physicians often disagree as to whether tic symptoms should be classified as tics or compulsions; as there is a significant overlap between tic disorders and obsessive-compulsive disorders (OCD). More than half of patients with tic disorders or Tourette's have been diagnosed with OCD; however, only a third of those meet the full criteria for OCD. Distinguishing tics from compulsions and true OCD can be very difficult. Compared to only obsessive-compulsive disorder in individuals, there will likely be an earlier age of onset and a greater percentage of males. These patients will see less response to medications such as selective serotonin reuptake inhibitors (SSRIs) and may require a neuroleptic (considered a stronger medication) to bring about improvement.

As many as 50% of children with Tourette's disorder have symptoms of attention-deficit hyperactivity disorder (ADHD). Symptoms include poor concentration, a short attention span and restlessness. On average, ADHD symptoms will manifest about two years before the tics begin. ADHD combined with a tic disorder is associated with more severe tics and greater social withdrawal. These impairments lead to higher underachievement in school and more social disabilities than one disorder alone. Although children with tics or ADHD do not  appear to have a significant impairment in intellectual functioning, they do have abnormal visual-perceptual performance and reduced visual-motor skills. Children with tic disorders are much more likely to require special education programs as these children often have underlying learning disabilities.


A team approach is best with the affected child's parents, health care providers and teachers. A comprehensive treatment plan should include the following:

 Direct observation.
 Self-reports of tic activity by the patient and parents.
 Educating the patient and family about the course of the disorder.
 Involvement with school personnel to create a positive learning environment.  
 A comprehensive assessment including cognitive ability, motor skills, behavior and social
 functioning.


Treatment
 Includes behavioral or cognitive/behavioral therapy in combination with medical treatment as
 needed.  

 

Behavioral and cognitive/behavioral therapy:


Habit reversal (is the most commonly used technique): combines relaxation exercises, awareness, and contingency management for positive reinforcement. This method shows a high success rate, 70+% . Adding the cognitive technique of distraction has been shown to help patients resist urges and to restore the patients sense of control over the tic.
Massed negative practice (MNP): is another frequently used behavioral therapy technique. The patient is asked to deliberately perform the tic movement for specified periods of time with brief periods of rest  until a conditioned level of fatigue is reached. Patients have shown some decrease in tic frequency, but the long-term benefits of massed negative practice are unclear.
Contingency management: is based on positive reinforcement, usually the by parents. Children are praised and rewarded for not performing tics and for replacing them with alternative behaviors. Contingency management appears to be of limited use outside of controlled enviroments
Self-monitoring: consists of having the patient record tics and is fairly effective in reducing some tics by increasing awareness.

 

Medications

Medication is the main treatment for refractory motor and vocal tics and should be used in conjunction with behavioral or cognitive/behavioral therapy. Because the symptoms of tic disorders overlap those of OCD and ADHD, it is essential to determine which symptoms are causing the greatest concern and impairment. 

Alpha-adrenergic receptor agonists: including clonidine (Catapres) and guanfacine (Tenex) are often first line therapy. Sedation occurs in 20% or more of cases and can often be controlled through adjusting the dosage.
Neuroleptics (antipsychotic medications): including haloperidol (Haldol) and pimozide (Orap) have significant side effects including decreased concentration, cognitive impairment, tremor, sedation, depression and rarely, tardive dyskinesia (a movement disorder that consists of lip, mouth, and tongue movements). 
Phenothiazines:  such as chlorpromazine (Thorazine) or triflupromazine (Stelazine)  may be used when haloperidol or pimozide has proven ineffective.
Atypical antipsychotics: and other agents that block dopamine receptors include risperidone (Risperdal) and clozapine (Clozaril).
Tetrabenazine: is a promising new medication and is marketed under the trade names Nitoman in Canada and Xenazine and has fewer side effects than other typical neuroleptics. It can also be used in combination with other antipsychotic medications, allowing for lower doses of both medications with substantial relief.
Selective serotonin reuptake inhibitors (SSRIs): which include such medications as fluoxetine (Prozac) and sertraline (Zoloft), can be used to treat depression and the obsessive-compulsive behaviors associated with tic disorders.
Benzodiazepines: including lorazepam (Ativan) or clonazepam (Klonipin) are used in some cases to lower patients anxiety levels.

Nutrition

Dietary changes and nutritional supplements may help treat the symptoms of tic disorders. Unknown  food or chemical allergies may worsen tic disorders. Nutritional deficiencies may also influence the severity of tic disorders. Recommendations include organic foods, avoiding pesticides, antioxidants, supplements including folic acid, magnesium, zinc and B vitamins; eliminating caffeine, and avoiding artificial sweeteners, colors and dyes (especially red dye 40).

 

Prognosis

There is presently no absolute cure for tic disorders and there is no method to determine whether the tics will be mild, severe, chronic or transient. The general consensus is that if a tic disorder is the only diagnosis, the prognosis is favorable and most patients report that their tics decrease markedly or disappeared as they enter the teenage years.  A number of studies suggest complete remission rates to be around 50% and appear to be related to early treatment when he or she was a child. While the tics themselves may decline, however, other associated problems such as obsessive-compulsive disorders and behavioral problems become more pronounced. Learning disabilities may also worsen in early adolescence. Panic attacks, anxiety, depression and alcoholism are most significant in the early adult years. Persons who were misunderstood, abused and stigmatized experience greater functional impairment as adults than those who were supported, understood and treated as children.

 

Prevention

There is some evidence that severe maternal emotional stress during pregnancy as well as severe nausea and vomiting during the first trimester may affect tic development. People with tic disorders are sensitive to stress and attempting to maintain a low-stress environment will help minimize severity of tics. Dietary changes and nutritional supplements as outlined above may help in the severity of the tics.