Tourette Syndrome

What is Tourette Syndrome?

Tourette syndrome is a common neuropsychiatric condition characterized by multiple motor and vocal tics.  Tics are involuntary, brief, intermittent movements such as eye blinking and facial grimacing.  They also include involuntary vocalizations such as simple noises and repetition of words.  Tourette syndrome is typically diagnosed in children but may be delayed until adolescence as well.  Most patients have improvement in their symptoms as they approach adulthood.

Individuals with Tourette syndrome often have coexisting psychiatric disorders such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD).  Due to the importance of this condition, it is critical that patients understand its causes, symptoms, and treatment.

What Causes Tourette Syndrome?

Tourette syndrome is believed to be caused by an interplay between social, environmental, and genetic abnormalities.  Patients often have a family history of both parents being affected by the condition.  Multiple gene mutations are associated with the disease, although there is no single gene mutation that results in the syndrome.

Studies show that disturbance the striatal-thalamic-cortical (mesolimbic) spinal system may be responsible for features of Tourette syndrome.  For unclear reasons, prenatal maternal smoking is associated with an increased risk for the disease.

How Common is Tourette Syndrome?

Tourette syndrome is a common neuropsychiatric condition that usually affects children.  The condition often requires referral to a child neurologist or psychiatry specialist, especially if first-line and conservative measures are unsuccessful.

The prevalence of Tourette syndrome in children is approximately 0.52%. Prenatal maternal smoking is a risk factor for the illness.

Signs and Symptoms

Symptoms of Tourette syndrome often include multiple motor and vocal tics.  Tics are involuntary, brief, intermittent movements such as eye blinking and facial grimacing.  They also include involuntary vocalizations such as simple noises and repetition of words.

Patients typically have an irresistible urge or sensation before the tic, which is relieved after the tic is completed.  Individuals can also temporarily suppress tics voluntarily.

Simple tics may include:

  • Eye blinking
  • Facial grimacing
  • Shoulder shrugging
  • Head jerking

Complex tics may include sequences movements such as body gyrations, kicking, or jumping.

Involuntary vocalizations may include

  • Simple noises
  • Coprolalia (obscene words)
  • Echolalia (repetition of words)
  • Palilalia (rapidly repeating a word faster)

Besides tics, the neurological examination in patients with Tourette disorder is typically normal.  Some patients have an increased rate of normal blinking.

Sleep-related symptoms of Tourette syndrome frequently include insomnia, restlessness, and nightmares.  Studies also show in increased prevalence of migraine and tension-type headache.  Coexisting psychiatric disorders such as attention deficit hyperactivity disorder and obsessive compulsive disorder are also common.

Diagnosis

The diagnosis of Tourette syndrome is based on the presence of motor and vocal tics in children or adolescents.  Tics must occur several times throughout the day, almost every day, or intermittently over more than 1 year.

Involuntary motor tics and vocalizations should not be explained by another medical or psychiatric condition, or occur as a result of medication or substance use.  Coexisting attention deficit hyperactivity disorder and obsessive compulsive disorder are highly supportive of the diagnosis.

Your doctor may order laboratory and imaging tests to rule out other neurologic or metabolic diseases.  Commonly ordered blood tests include a CMP (comprehensive metabolic panel), CBC (complete blood cell count), and thyroid function tests (TSH, free T4).  Your doctor may obtain a urine drug screen for stimulants such as cocaine or amphetamines.  If they are concerned for structural brain disease such a tumor or stroke, they will often request a brain MRI.  They may also obtain an electroencephalogram (EEG) if they are concerned for seizure disorder.

Tourette Syndrome Medication

Tourette syndrome is treated with education, counseling, and medical therapy.  Mild symptoms often respond to conservative measures such as counselling and education alone.  Patients with bothersome symptoms are often started on Xenazine (Tetrabenazine).

Some patients can benefit from Botox (botulinum toxin) injections into the muscles that produce the tic.  Psychotherapy with habit reversal training may help improve symptoms.

For those who have coexisting attention deficit hyperactivity disorder (ADHD), alpha-adrenergic agonists such as Intuniv (Guanfacine) or Catapres (Clonidine) may be effective.  Patients with Tourette syndrome and obsessive-compulsive disorder (OCD) can benefit from treatment with selective serotonin reuptake inhibitors (SSRIs):

References:

  1. Kurlan R. Clinical practice. Tourette's Syndrome. N Engl J Med 2010; 363:2332. - https://www.nejm.org/doi/full/10.1056/NEJMx120010
  2. Jankovic J, Kurlan R. Tourette syndrome: evolving concepts. Mov Disord 2011; 26:1149. - https://www.ncbi.nlm.nih.gov/pubmed/21484868

The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.