Tourette’s syndrome

Most common and severe multiple tic disorder.

Tics are known to ebb in severity and are not problematic by the adult years.

An inherited neurological disorder with onset in childhood, characterized by the presence of multiple motor tics and at least one vocal tic.

Most commonly onset occurs during childhood, increases in severity and frequency during adolescence.

Reaches peak severity in second decade of life , after which most patients experience improvement.

Tics characteristically wax and wane.

In a subset of individuals symptoms continue across their lifespan in a waxing and waning pattern.

Often associated with comorbid conditions such as obsessive-compulsive and attention deficit/hyperactivity disorder.

Genetic and environmental factors are involved.

Overwhelming majority of cases are inherited and some cases are sporadic.

Exact mode of inheritance is not known, and no gene has been identified.

Patients have about a 50% chance of passing the genetic process to their children, but the process is of variable expression and incomplete penetrance.

Not everyone who inherits the genetic vulnerability will show symptoms.

Family members may show different severities of symptoms, or no symptoms at all, mild tic disorders or obsessive compulsive symptoms without tics.

Males more likely to express tics.

Environmental, infectious, or psychosocial factors can influence the severity of the process.

Tics are probably derived from dysfunction in cortical and subcortical regions, the thalamus, basal ganglia and frontal cortex.

Tics constantly change in number, frequency, severity and anatomical location.

The tics of this disease are temporarily suppressible and preceded by a premonitory urge.

A disorder with childhood onset, although symptoms can begin during infancy or in late adolescence.

The usual age of onset of tics occurs in patients aged 2-15 years, with a mean age range of 6-7 years.

Approximately 75% of patients have symptoms by age 11.

While tics may appear suddenly, they usually are of gradual onset and have intervening spontaneous remissions.

50-70% of patients have facial tics as their initial symptom, while tics involving neck or shoulder movements, are the next most common presention, followed by involuntary movements of the upper extremities.

Vocal tics occur as initial symptoms in only 12-37% of patients.

Tics of the lower extremities and trunk are the least common manifestations.

Coprolalia is a complex vocal tic that appears 4-7 years after onset of the process, and occurs in fewer than one third of the patients.


Coprolalia it is not required for a diagnosis of Tourette’s and only about 10% of Tourette’s patients exhibit coprolalia.

In severe disease may see self injury tics, copropraxia, echolalia, and talking with different accenta.

Tics may exacerbate by physical or emotional distress, and during periods of relaxation following stress.

Motor and phonic tics may persist during all stages of sleep.

Reduction or increased intensity in tics may occur when patients are distracted by concentrating on mental or physical tasks.

Tics also typically are exacerbated by dopaminergic drugs and CNS stimulants, including methylphenidate.

Transient tic disorder of childhood is similar to TS, but it lasts for less than a year.

Chronic multiple tic disorder has a similarity to TS but remains present in adulthood.

Chronic single tic disorder is a motor or vocal tic in adulthood.

It is a clinical diagnosis, therefore, no specific laboratory or genetic tests help establish the diagnosis.

Diagnostic Criteria include: multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily at the same time, tics occur many times a day, nearly every day or intermittently, throughout a period of more than 1 year, there must not be a tic-free period of more than 3 consecutive months, onset is before 18 years, causes significant impairment in social, occupational, or other important areas of functioning, and is not due to the direct physiologic effects of a substance or a general medical condition.

A neuropsychiatric illness that begins in childhood.

Onset usually between ages 2 and 15 years.

Prevalence ranges from 0.1%-3.8%.

Simple motor tics are sudden, brief, patterned movements such as eye blinking, facial grimace, head jerks, or shoulder shrugs.

Complex motor tics can involve a series of simple tics, or a purposeful activity, such as jumping, touching, or performing obscene gestures.

Vocal tics consisting of sounds such as throat clearing, coughing, sneezing, may occur.

Complex vocal tics include echolalia, speaking of foul language, on repeating one’s own words.

Patients may have a preceding urge before their tics.

Tics may be suppressed transiently by patients and 10 B. diminished by focused mental or physical activities.

Tics usually do not affect activities of daily living, occupation or recreational activities.

Following the expression of a tic leads to a feeling of relief.

Tics may be worse with emotional stress.

Fatigue and illness increase the frequency of tics.

Tics may occur with boredom and can occur with sleep and rapid eye movements.

Symptoms wax and wane and typically improve in adulthood.

Symptoms remit in 75% of patients by median age of 18 years.

Characterized by waxing and waning motor and vocal tics lasting over one year.

Involuntary emission of unacceptable word or phrases seen in 5-15% of individuals.

Highly familial.

Variants of neuronal transmembrane molecule (SLITRK1), rarely associated with Tourette’s syndrome.

Evidence suggests disorder associated with involvement of dopaminergic neurotransmission and abnormalities involving cortical striatal thalamic cortical circuit (Jeffries KJ).

Rarely associated with a functional mutation in the HDC gene encoding L-histidine decarboxylase, the rate limiting enzyme in histamine biosynthesis.

First-line treatments include medications such as alpha-adrenergic blockers, typical and atypical antipsychotics, and benzodiazepines.

Drug of choice is clonidine.

Medication therapy considered if tics are functionally disabling and/or are not remediable to nonpharmacologic interventions.

Classes of medication used in the treatment include neuroleptics, nonneuroleptic dopamine antagonists, and immune modulators.

Antihypertensive agents clonidine and guanfacine are also used to treat tics, but studies show variable efficacy.

Attention deficit hyperactivity disorder occurs in up to 90% of patients.

Treatment aimed at tic reduction, and management of coexisting obsessive-compulsive disorder and attention deficit hyperactivity disorder.

When first-line treatment fails, botulinum toxin injections and behavioral therapy may be instituted.

Centromedian-parafascicular complex deep brain stimulation for medically

refractory patients is associated with good overall tic control and improvement in general function.

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