TIC DISORDERS

History In 1885, Georges Gilles de la Tourette, a French neurologist, described patients with childhoodonset tics and coexisting emotional symptoms and behavioral problems, such as inattention, hyperactivity, impulsivity, obsessions, and compulsions. Gilles de la Tourette coined the term coprolalia.

Phenomenology
Tics are defined as sudden, repetitive movements, gestures, sounds, or utterances that typically mimic some fragment of normal behavior. non rhythmic stereotyped muscle contraction resulting in movements or vocalizations that are experienced as involuntary. Tics change in frequency, anatomical location, number, complexity, and severity over minutes, hours, and days, as well as over longer periods of time. Tics may persist during sleep.

Phenomenology
Tics are experienced as irresistible but can be suppressed for varying periods of time. There is usually a reduction in the frequency and severity of tics during concentration on mental or physical tasks such as reading. Stress, excitement, boredom, fatigue, and exposure to heat can exacerbate tics. Some children, and occasionally adults, are unaware of their tics.

Phenomenology
Tics as a voluntary response to an uncomfortable feeling that precedes them. These feelings, or premonitory sensations, and they can be localized or general, and physical or mental, in nature. These premonitory sensations distinguish tic disorders from other movement disorders. However, there are often non-localizable and more generalized premonitory phenomena, such as urges, sensations, or anxiety. Many individuals report a just right phenomenon that to relieve the uncomfortable urge they must repeat a particular movement until it feels good or it feels just right.

Types of Tics
Tics typically involve one muscle or a group of muscles and may be characterized by their anatomical location, number, frequency, duration, and complexity. SIMPLE TICS MOTOR VOCAL COMPLEX TICS MOTOR VOCAL

Simple motor tics

Involves single muscle or group of muscles Clonic tic (< 100 msec): head jerks, eye blinking, nose twitching Dystonic (> 300 msec): ocular deviation, blepharospasm, bruxism, torticollis, mouth opening, shoulder rotation Tonic (> 500 msec): isometric contractionabdominal, limb tensing

Complex motor tics

Typically slower, rhythmic, and involve multiple muscle groups (e.g., simultaneous eye deviation, dystonic postures, even sequences of simple motor tics). Some complex motor tics appear more purposeful, such as stereotyped crouching and hopping, repetitive touching or rubbing, and, less commonly, obscene gestures (copropraxia). Coordinated sequential movements. Non purposeful: head shaking, trunk bending

 It is important to determine whether the behavior is being pursued as a strategy to relieve anxiety or as a preventive measure, rather than simply to satisfy an urge. If anxiety relief or prevention is the case, a compulsion rather than a tic is likely.

Simple vocal (phonic) tics

Simple vocal tics are usually brief staccato-like sounds caused by quick diaphragmatic contractions and resulting forceful movement of air through the nose and mouth. Single, meaningless sounds and noises Not all sounds arise from the vocal cords Throat clearing, grunting, sniffing, hissing, spitting, screaming, coughing, whistling

Complex vocal tics

Include utterances such as words, phrases, and repeating ones own words (palilalia) or the words of others (echolalia). Linguistically meaningful utterances and verbalizations. Uttering obscene words/phrases (coprolalia) or other socially inappropriate word/phrases is not essential for diagnosis and is uncommon with approximately 10% of TD cases so affected.

NOSOLOGY
symptom-based taxonomy. Onset before 18 yr. DSM-IV-TR and ICD-10, tic disorders include transient tic disorder, chronic motor tic disorder, chronic vocal tic disorder, Tourette's disorder [de la Tourette] and tic disorder not otherwise specified.

NOSOLOGY
By DSM-IV-TR criteria, a transient tic disorder is characterized by the presence of motor and/or vocal tics for greater than 4 weeks but less than 1 year. A chronic motor or vocal tic disorder is diagnosed when either motor or vocal tics, but not both, are present for more than 1 year. Tourette's disorder (combined vocal and multiple motor tic disorder [de la Tourette]

EPIDEMIOLOGY
Tourettes syndromePrevalence- 4 to 5 per 10,000 M>F

Chronic motor or vocal tic disorderPrevalence- 1% to 2% Transient tic disorderPrevalence-5% to 24%

Clinical presentation & Course

Tic symptoms usually begin in childhood with an average age of diagnosis of 7 years. Prodromal behavioral symptoms- irritability, attention deficit, poor frustration tolerance. Facial and ocular tics (i.e., blinking, squinting, eye darting, or eye rolling) typically appear first. In many patients, motor tics remain isolated to the face. Motor tics appearing in other anatomical regions often present sequentially from the head and face to the neck, shoulders, trunk, and extremities.

 Motor tics typically precede vocal tics. Initial tic symptoms (motor or vocal) are often simple in character, with complex symptoms developing later. Vocal tics typically start at age 8 to 9 years; morecomplex tics often begin later, at about age 11 to 12 years. Obsessions, compulsions, impulsivity, personality problems in 50% with TS.

COURSE & OUTCOME

DIFFERENTIAL DIAGNOSIS
MOVEMENT DISORDERS chorea, athetosis, hemiballismus, dystonia, myoclonus, tremor NEUROLOGICAL DISEASES Wilsons dis, PD, Sydenham`s chorea, HD, Neuroacanthocytosis etc. In psychiatric settings- neuroleptic related dyskinesia, Pervasive developmental disorder Tremors, Mannerisms & Stereotypic movements.

Differential Diagnosis of Abnormal Movements

Akathisia Athetosis Ballismus Chorea Compulsion Myoclonus Periodic sleep movements Restless legs Spasm Tremor Dyskinesia-Choreiform or dystonic movement that is involuntary, stereotyped, and not suppressible Dystonia- Sustained, tonic contraction that progresses to abnormal postures Stereotypy- Repetitive, usually meaningless, gestures, habits, or automatisms. Rhythmic, self-stimulating/soothing, intentional

 Mannerisms or gestures typically lack the involuntary, repetitive, and stereotyped nature of tics. Stereotypies tend to occur more commonly (but not exclusively) in children and adults with developmental disabilities and mental retardation. It is also possible to have a tic disorder and another movement disorder.

Clonidine- an 2-adrenergic presynaptic antagonist recommended as first-line treatment for most patients with mild to moderate Tourette's disorder. Treatment is initiated at 0.0250.05 mg/day and increased in increments of 0.0250.05 mg/day every 35 days or as side effects (sedation) allow effective treatment doses are in the range of 0.10.3 mg/day (8microg/kg) and are given in divided doses (46 hours apart). The onset of action is slower for tic suppression (36 weeks) than for ADHD symptoms

Guanfacine an 2-adrenergic postsynaptic agonist. Starting doses are 0.5 mg/day with increases of 0.5 mg every 57 days up to 34 mg/day. Side effects, generally less significant and more tolerable than with clonidine.

Neuroleptic Agents Haloperidol and pimozide have been the only FDA approved agents for the treatment of Tourette's disorder. In general, neuroleptic agents are not recommended for mild tics. Of the typical neuroleptics, haloperidol and pimozide have been the most investigated, with double-blind, placebo-controlled studies supporting their use.

 Haloperidol- 14 mg/day are usually adequate for tic suppression. Fluphenazine (1.510 mg/day) Pimozide (26 mg/day) Tiapride(150500 mg/day) Sulpiride (2001000 mg/day) Risperidone- equally effective as pimozide and clonidine for tic reduction. 1-3mg/day. the added benefit of augmenting SSRIs in treating tic-related OCD. Ziprasidone Olanzepine Quetiapine Aripiprazole

Benzodiazepines can be useful in decreasing cooccurring anxiety in patients with TD and as an adjunct to SSRIs for OCD in TD. Clonazepam (0.54 mg/day). Tricyclic Antidepressants- no dose ranges established. SSRIs- no evidence to support the use. Stimulants- ADHD symptoms with comorbid tic disorder, monitoring for the exacerbation of tics. Atomoxetine- SNRI, doses 0.5-1mg/kg

Other alternatives: IVIG, plasmapheresis, and corticosteroids Naltrexone (25 to 75 mg/day) for patients with self-injurious behaviors Baclofen Nicotine Pramipexole Botulinum toxin Surgery- Bilateral capsulotomy, DBS, Transcranial magnetic stimulation

Non-pharmacological interventions
HRT was initially described by Azrin and Nunn in 1973. The purpose of HRT is to disrupt reinforcement cycles by performing an alterative and less conspicuous behavior (i.e., competing response) when facing triggering stimuli The two principle components of HRT are awareness training and competing response training. Awareness training involves learning to become aware of and eventually monitor premonitory urges (e.g, building tension, tickle, itch, etc.) and subsequent tic behavior (e.g., head shaking).

 Competing response training involves learning to perform an alternative behavior utilizing the same muscle groups used to carry out the tic. Behavioral training in the form of neurofeedback is an emerging treatment that has yet to establish clinical utility.

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