Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 24-13: Movement Disorders + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Multiple motor and phonic tics Symptoms begin before age 18 years Tics occur frequently for at least 1 year Tics vary in number, frequency, and nature over time +++ General Considerations ++ The diagnosis of the disorder is often delayed for years, the tics being interpreted as psychiatric illness or some other form of abnormal movement Patients are thus often subjected to unnecessary treatment before the disorder is recognized +++ Demographics ++ Tics are noted first in childhood, generally between the ages of 2 and 15 A family history is sometimes obtained + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Motor tics Initial manifestation in 80% of cases Most commonly involve the face, head, shoulders, such as sniffing, blinking, frowning, shoulder shrugging, and head thrusting Phonic tics Initial symptoms in 20% of cases Commonly consist of grunts, barks, hisses, throat clearing, coughs, verbal utterances including coprolalia (obscene speech) A combination of different motor and phonic tics ultimately develop in all patients Echolalia (repetition of the speech of others) Echopraxia (imitation of others' movements) Palilalia (repetition of words or phrases) Some tics may be self-mutilating in nature Nail-biting Hair-pulling Biting of the lips or tongue Obsessive-compulsive disorder and attention deficit hyperactivity disorder are commonly associated and may be more disabling than the tics themselves In addition to obsessive-compulsive behavior disorders, psychiatric disturbances may occur because of the associated cosmetic and social embarrassment +++ Differential Diagnosis ++ Wilson disease + Diagnosis Download Section PDF Listen +++ +++ Diagnostic Procedures ++ Examination usually reveals no abnormalities other than the tics + Treatment Download Section PDF Listen +++ +++ Medications ++ α-Adrenergic agonists are first-line therapies because of favorable side effect profile compared with typical antipsychotics Clonidine (start 0.05 mg orally at bedtime, titrating to 0.3–0.4 mg orally daily, divided three to four times per day) Guanfacine (start 0.5 mg orally at bedtime, titrating to a maximum of 3–4 mg orally daily, divided two times per day) Many specialists favor use of tetrabenazine Atypical antipsychotic risperidone (1–6 mg daily orally) is more effective than placebo in controlling tics and more effective than pimozide in improving symptoms of comorbid OCD, and may be tried before the typical antipsychotic agents Haloperidol Typical antipsychotic of choice Started in a low daily dose (0.25 mg orally) Gradually increase dose by 0.25 mg every 4 or 5 days until there is maximum benefit with a minimum of side effects or until side effects limit further increments A total daily dose of between 2 mg and 8 mg is usually optimal, but higher doses are sometimes necessary Fluphenazine (1–15 mg orally daily) or pimozide (1–10 mg orally daily) are alternatives to haloperidol Typical antipsychotics can cause significant weight gain and carry a risk of tardive dyskinesias and other long-term, potentially irreversible motor side effects Small randomized trials or observational studies have reported benefit from Topiramate Nicotine Tetrahydrocannabinol Baclofen Clonazepam A number of other medications, including deutetrabenazine, valbenazine, and ecopipam, are being studied for the treatment of tics Injection of botulinum toxin type A at the site of the most distressing tics is sometimes worthwhile Bilateral high-frequency deep brain stimulation may help in otherwise intractable cases +++ Therapeutic Procedures ++ Treatment is symptomatic and may need to be continued indefinitely Habit reversal training or other forms of behavioral therapy can be effective alone or in combination with pharmacotherapy + Outcome Download Section PDF Listen +++ +++ Prognosis ++ The disorder is chronic, but the course may be punctuated by relapses and remissions +++ When to Refer ++ All patients +++ When to Admit ++ Patients undergoing surgical (deep brain stimulation) treatment + References Download Section PDF Listen +++ + +Martinez-Ramirez D et al. Efficacy and safety of deep brain stimulation in Tourette syndrome: the International Tourette Syndrome Deep Brain Stimulation Public Database and Registry. JAMA Neurol. 2018 Mar 1;75(3):353–9. [PubMed: 29340590] + +Pringsheim T et al. Practice guideline recommendations summary: treatment of tics in people with Tourette syndrome and chronic tic disorders. Neurology. 2019 May 7;92(19):896–906. [PubMed: 31061208]