Management of Tourette's Syndrome Reviewed

Laurie Barclay, MD

December 08, 2010

December 8, 2010 — Strategies and guidelines on diagnosing and treating Tourette's syndrome are offered in a clinical practice article reported in the December 9 issue of the New England Journal of Medicine.

"Tourette's syndrome is now viewed as a neuropsychiatric spectrum disorder in which tics are commonly associated with obsessive−compulsive symptoms that do not always meet the full diagnostic criteria for obsessive−compulsive disorder (OCD) and with disturbances of attention that do not always meet the full criteria for attention deficit–hyperactivity disorder (ADHD)," writes Roger Kurlan, MD, from the Movement Disorders Program, Atlantic Neuroscience Institute, Overlook Hospital, Summit, New Jersey. "The combination of tics, OCD, and ADHD is often called 'the Tourette's syndrome triad.' Studies have suggested a familial aggregation and hereditary relationship among these three conditions."

Tourette's syndrome can be diagnosed when motor and vocal tics have been present for at least 1 year. Most children are diagnosed clinically, without the need for neuroimaging or other laboratory testing. Because tic suppression often occurs in physicians' offices, the best time to look for tics is when the patient is entering or leaving the examination room. Coexisting psychiatric conditions can be evaluated with clinical rating scales such as the Yale-Brown Obsessive-Compulsive Scale, the Conners Parent or Teacher ADHD Rating Scales, and the Child Depression Inventory.

When tics are mild and are not disabling, education about the condition and supportive counseling to strengthen self-confidence and self-esteem usually suffice. When tics are disabling because they cause social embarrassment or self-injury, tic-suppressing therapy is indicated. Treatment options include a form of cognitive behavioral therapy (termed habit-reversal treatment) or pharmacotherapy.

The only medications approved by the US Food and Drug Administration for Tourette's syndrome are the classic neuroleptic antipsychotic agents haloperidol and pimozide, which block D2 dopamine receptors. Risperidone, a newer atypical antipsychotic agent, appears to suppress tics with a magnitude of benefit similar to that of the classic neuroleptics, based on randomized controlled trials.

All antipsychotic agents are associated with sedation, depression, increased appetite, and parkinsonism. The atypical antipsychotics have fewer motor complications but often induce significant weight gain and glucose intolerance. Because of these adverse effects, other medications are often used first, such as the α2-adrenergic drugs clonidine and guanfacine, which may also be effective for attention-deficit hyperactivity disorder.

Other possible approaches include combination pharmacotherapy, botulinum toxin, or deep brain stimulation. Appropriate treatment of coexisting conditions is recommended.

"The Practice Parameter Group of the Tourette Syndrome Association has published recommendations for the evaluation, diagnosis, and treatment of Tourette's syndrome and associated psychiatric conditions," Dr. Kurlan writes. "For the treatment of Tourette's syndrome, the recommendations are that guanfacine or clonidine be considered as a first-line medication for moderate or more severe tics, that botulinum toxin may be considered in patients with a single interfering tic, and that more potent medications such as risperidone, pimozide, or fluphenazine may be considered for tics with an inadequate response to the alpha-agonists. A report by the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology, which focused on neurologic uses of botulinum toxin therapy, concluded that it is an appropriate treatment for tics in patients with Tourette's syndrome."

Dr. Kurlan has received consulting fees from Boehringer Ingelheim and Biovail and grant support from the Michael J. Fox Foundation, Kyowa, and Neurologix.

N Engl J Med. 2010;363:2332-2338.

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