Neurologic syndrome characterized by repeated and
involuntary, stereotype motor movement or vocalization (tics).
Tourette Syndrome; Brissaud Syndrome; Maladie des Tics;
Tic Impulsif; Chronic Motor Tics.
Georges Albert Édouard Brutus Gilles de la Tourette
was a French neurologist (1857-1904) who described this rare
psychoneurologic disorder with onset in childhood, usually at 7 to 10
years of age, characterized by echolalia, palilalia and coprolalia, stuttering
and a craving to touch. Gilles de la Tourette became Doctor in
Paris in 1879.
Common; estimates range from 0.05 to 1% in the
general population. Up to 20% of schoolchildren may have a transient tic
disorder at some point.
Controversial; probably autosomal dominant
with incomplete penetrance and variable expression. Environmental factors seem to
influence the risk, severity, and course of the disorder.
Postulated to be a result of dopamine excess or
supersensitivity of the postsynaptic dopamine receptors in the basal ganglia
and frontal cortex. Another hypothesis is a neurophysiologic deficit
secondary to neurotransmitter abnormalities, resulting in failed inhibition
of frontal-subcortical motor circuits.
Diagnostic criteria for Gilles de la Tourette syndrome
recommended by the American Psychiatric Association include both multiple
motor and vocal tics over a period of more than 1 year, voluntary
suppression of symptoms, a waxing and waning course, and onset between
2 and 15 years of age.
Initial symptoms usually are involuntary,
tic-like movements. The disorder progresses with development of echolalia
(repeating another's words or phrases), grunting, and coprolalia (use of
obscene words). Most cases are mild and do not come to medical attention.
Waxing and waning course, with one tic appearing and typically being
replaced by another. Long-term outcome is generally favorable. Commonly
associated with attention deficit hyperactivity disorder,
obsessive-compulsive disorder, behavior problems, and learning disabilities.
Management includes behavior or pharmacologic therapies (neuroleptic
medications and dopamine D2 antagonist agents).
Tics should be differentiated from
other movement disorders, such as chorea, stereotypy, and dystonia. Urine
drug screening for cocaine and stimulants should be considered in a teenager
with sudden onset of tics and inappropriate behavior symptoms. Every patient
with a family history of liver disease associated with hyperkinetic movement
disorder should be investigated to rule out Wilson disease (hepatolenticular
degeneration). Details of current drug therapy are essential to prevent
unwanted drug interactions.
Anxiety, stress, and fatigue often
intensify tics. Behavioral disorders may limit cooperation during induction
Psychoactive drugs, particularly
cocaine and other stimulants, have a tendency to worsen tics. Drug
interactions may arise from existing pharmacologic therapy: neuroleptic
agents (haloperidol), atypical neuroleptic agents (risperidone), α2-adrenergic presynaptic agonists (clonidine), benzodiazepines
(clonazepam), and CNS stimulants (pemoline, used for attention deficit
hyperactivity disorder). Benzodiazepines may result in hypnotic synergism on
induction of anesthesia, and a lower dose of induction agent may be
required. Recovery may be delayed. Pemoline-sevoflurane interaction
reportedly may cause ...