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Tourette’s Syndrome and Tics

The word "tic" has been used for at least four centuries to refer to a variety of sensory and motor conditions. For example, "tic douloureux" refers to a pain syndrome. Today, the term tic is best applied to a subgroup of movement disorders characterized by rapid and irregularly repetitive movements that usually involve the head and neck muscles, including the voice apparatus Tourette's syndrome, tics, torette's syndrome, torette syndrome, tourettes syndrome, tic torettes syndrome. Tics may manifest as a symptom of various different conditions and, thus, have been broadly classified as

Transient idiopathic tics are extremely common in children, with up to 13 percent of boys and 11 percent of girls manifesting tics of sufficient frequency or severity as to require medical attention. Typical age at onset is seven years. For most children, tics spontaneously resolve within one year after onset. Transient tics rarely begin after age 17, so new-onset tics in adults, especially those over age 50, should alert the physician to search for an

Chronic idiopathic tics, which comprise the true "tic disorders," also emerge during childhood but are less prevalent than transient tics. Tourette's syndrome

Classification of Tic Disorders


Transient tic disorder

Chronic tic disorder (motor or vocal tics)

Tourette's syndrome (motor and vocal tics)

Adult-onset tic disorder (rare)


Basal ganglia disease (e.g., Sydenham's chorea) Metabolic insults (e.g., carbon monoxide poisoning, hypoglycemia)

Drug-induced tics (e.g., amphetamines, cocaine, neuroleptics)

Congenital tics (e.g., rubella, birth anoxia, Asperger's syndrome, Rett syndrome)

Genetic tics (e.g., fragile X syndrome, Down syndrome, XYY syndrome, XXX+9p syndrome)

Psychiatric tics (e.g., schizophrenia, obsessive-compulsive disorder)

has an incidence of 59 per 10,000 persons.29 With chronic tic disorders, tics may improve during adolescence and reappear in later adulthood. In some patients, tics persist and can result in a lifetime of disability. Chronic tic disorder includes a genetic component; male first-degree relatives have up to a 30 percent higher risk of 


Younger children are frequently unaware of tics. When brought to medical attention by a teacher or parents, the chief complaint is often termed "hyperactivity.'' Periods of inattention in these patients may be indicative of epilepsy, especially if tics occur during 

Tics manifest as habitual behaviors or mannerisms, such as winking, facial grimacing, head-jerking or shoulder-shrugging, or, less commonly, as directed movements, such as the hand wiping the nose. Complex tics consist of a series of simple tics in succession or complicated behaviors performed repetitively. Occasionally, tics may cause self-injury, such as slapping one's own face or biting one's own hand. Vocal tics range from brief sounds, such as that made when clearing the throat, to the shouting of

Adults and children over age 10 may complain of a sensation, such as an itch, that creates an urge to perform the tic movement. These patients may report that although they can voluntarily suppress the tics, the effort involved in doing so creates a build-up of tension that becomes unbearable. Eventually, the tension must be "released" by performing the tic. However, the relief thus produced is only temporary, and the patient soon experiences the sensation and urge again; this process may be repeated up to several hundred times a day. Tics often occur in bouts throughout the day, with exacerbations developing during periods of anxietyor public exposure and momentary relief during relaxed or pleasurable activities. Tics occur less frequently during a febrile illness or with alcohol intake and

Patients with tics may have associated behavioral disorders that compound the level of disability caused by the tics. Attention-deficit/hyperactivity disorder, obsessive-compulsive disorder and affective disorders are common comorbid conditions. Thus, obtaining a comprehensive medication history is particularly important for both children and adults who present with tics. Methylphenidate (Ritalin) may have been prescribed for a child with a tic disorder in an attempt to control the hyperactivity and inattention that often accompany tics. However, use of this stimulant may worsen tics. Cocaine and anabolic steroids have also

Differential Diagnosis of Tic Disorders in Adults


Body part affected

Clues to diagnosis


Bilateral eyelids

Bilateral blinking or closing of eyes

Hemifacial spasm

Unilateral facial muscles

Limited to one side of the face

Focal epilepsy

Any body part

Rhythmic pattern, presence of an aura, characteristic EEG findings


Arms, trunk

Jerks occur singly


Head, limbs (upper more than lower)

Tremors are continuous

Huntington's chorea

Trunk, limbs

Movements are not suppressible, positive family history

Sydenham's chorea

Trunk, limbs

Movements are not suppressible, antecedent streptococcal infection, positive ASO titers


Trunk, proximal limbs

Slow writhing movements


Limbs more than trunk

Pacing, getting in and out of a chair, etc.


Neck, limbs

Fixed abnormal postures

Tardive dyskinesia

Mouth, tongue

History of drug exposure


Proximal limbs (upper more than lower)

Abnormal MRI results in subthalamic region

Wilson's disease

Proximal limbs (upper more than lower)

Positive family history, characteristic blood and urine copper study findings, Kayser-Fleischer rings


In 1885, Gilles de la Tourette published the first reports of patients exhibiting "... motor incoordinations and ... inarticulate shouts accompanied by articulated words with echolalia and coprolalia .... ' Tourette's syndrome begins in early childhood with simple motor tics that are limited to a single muscle group, such as eye-blinking or head-jerking. Within one year after onset, complex motor tics appear, consisting of more elaborate facial grimacing and shoulder and hand movements. Repetition of one's own behaviors (palimimia) and imitation of others' behaviors or movements (echomimia) are

Tourette's syndrome has been described as following a "rostrocaudal' progression despite the fact that the lower body is rarely involved. Vocal tics become apparent in early adolescence and must be present to definitively establish the diagnosis. These

Table 11

Differential Diagnosis of Tic Disorders in Children


Body part affected

Usual age at onset

Transient tic disorder

Eyes, face, shoulders

Five to 11 years

Paroxysmal torticollis

Abnormal neck postures

Usually less than three months

Transient dystonia

Abnormal arm postures

Less than five months

Spasmus mutans

Head tremor, eyes (nystagmus)

Four to 12 months; rarely seen in children over age three

Palatal tremor (essential type)

Soft palate, eyes, shoulders

Two years and over

Paroxysmal tonic upgaze

Eyes (ataxia may occur during episodes)

One to eight months

Benign myoclonus

Entire body

Newborns, infants


Chin, extremities



Head, arms


Sydenham's chorea

Trunk, extremities

Seven to 15 years

corprolalia. Obsessive-compulsive behaviors may manifest several years later in up to 60 percent of patients but are not required for diagnosis, The generally accepted diagnostic criteria for Tourette's syndrome are listed in Table 12.


The diagnostic work-up usually focuses on disease entities that are suspected based on the history and physical examination. An EEG may distinguish true tics from focal or myoclonic epilepsy. In children and young adults, special blood studies may include antistreptolysin O (ASO) titers (Sydenham's choreaJ or assessment of urinary copper and serum ceruloplasmin (Wilson's disease). In general, imaging studies are rarely useful in the absence of 


Pharrnacotherapy. Transient tics usually do not require treatment unless they interfere with overall function. In such cases, low-dose clonazepam administered once or twice per day is usually sufficient to control tics. Baclofen (Lioresal) and anticonvulsant agents, such as carbamazepine, have also been used with

Chronic tic disorders often require directed treatment. Most studies evaluating drug efficacy in Tourette's syndrome have shown that dopamine type D2-receptor blockade reduces motor and vocal tics. Pimozide (Orap), 1.0 mg each evening, is considered first-line therapy for this

Haloperidol (HaldolJ, 0.25 mg each evening, is a good second choice. Risperidone (Risperdal), a less potent D2-receptor blocker, has also shown efficacy in recent open-label studies when administered at daily doses of 1.0 to 4.0 rag.33 Dosages of

Adverse effects associated with D2-receptor blockers used in patients with tic disorders are similar to those seen in patients who take these agents for psychiatric illness and include sedation, akathisia, extrapyramidal symptoms, parkinsonism, cognitive impairment, weight gain and phobias. Weight gain is more commonly seen with risperidone. In addition, as with other

Clonidine (Catapres), which acts at central alpha-2 adrenergic receptors, has shown some effectiveness in suppressing tics associated with Tourette's syndrome but is not FDA-approved for this indication. The usual starting dose is 0.05 mg each


Associated developmental and behavioral conditions produce significant disability and may require additional directed treatment. Obsessive-compulsive disorder is perhaps the most often recognized comorbidity and is classified as "tic-related" or "nontic-related" based on its pharmacology. Tic-related obsessive-compulsive disorder has an earlier onset and is