Tics and Tourettes
1) TICS
-Simple or complex movements or sounds/words/phrases that abruptly and
briefly interrupt normal activity, with return to normal in between.
-Stereotyped, associated with a premonitory urge/odd sensation/discomfort
that is relieved by performing the tic, suppressible (though with worsening of
urge while suppressing), decreases with distraction, waxes and wanes and can
remit, can change semiology or location with time, worse with stress.
-Of note, patient may not become aware of urge until years after tics begin.
-CAN occur in sleep.
-Often associated with OCD, ADHD, behavior/psych/impulse control/self-injurious
behavior.
-Prevalence ranges 0.5-24%.
Secondary etiologies:
-Hereditary (Tourette’s, HD, dystonic syndromes, neuroacanthocytosis, Rett’s)
-Drugs (dopamine blockers, cocaine, levodopa, anticonvulsants, amphetamines,
Ritalin, antipsychotics)
-Infectious/immune (encephalitis, CJD, syndenham chorea, antiphospholipid)
-Toxins (CO)
-Other (static encephalopathy, mental retardation, autism spectrum, stroke,
brain tumor, trauma)
-psychogenic
-NO link between strep and tics (ie, PANDAS is not a cause)
2) Tourette’s Syndrome
-Cause unknown, but is known as a genetic disease.
-95% start between age 4 and 13, typically between age 5-8.
-Prevalence 0.5-1% in kids, 0.05% in adults (adult forms are usually reemergence
of childhood tics)
-Male: female is 4:1; mean onset 6 years old.
-50% have family history, often with both parents affected with TS, OCD, or
ADHD. Specific genes not known. If family member has tics, risk of person having
tics is 10-17%.
-Copralalia is present in a minority of cases.
-Natural history: Classic order of events is ADHD age 4, simple motor tics age 7,
then phonic tics, then OCD and more complex tics.
-90% have some other concomitant psychiatric disorder.
TS diagnostic criteria (DSM V):
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have two or more motor tics (for example, blinking or shrugging the shoulders) and at
least one vocal tic (for example, humming, clearing the throat, or yelling out a word or
phrase), although they might not always happen at the same time.
have had tics for at least a year. The tics can occur many times a day (usually in bouts)
nearly every day, or off and on.
have tics that begin before he or she is 18 years of age.
have symptoms that are not due to taking medicine or other drugs or due to having
another medical condition (for example, seizures, Huntington disease, or postviral
encephalitis).
Tx of tics:
-All treatment is symptomatic; natural history is that most remit spontaneously by
20’s.
-First question: Do they NEED treatment? Is it disruptive/annoying/painful? If so,
treat.
-Behavior therapy/treatment of underlying psych issues is a good place to start,
then consider pharmacotherapy. Goal is to make tics manageable, not necessarily
to make patient tic-free.
First-line pharmacology:
a) Alpha-2 agonists: Guanefacine (Tenex) 0.5-4mg/day, or clonidine 0.050.3mg/day; particularly good if ADHD also present (watch for sedation,
hypotension)
b) Dopamine blockers: Pimozide (Orap) 0.5-8mg/day or Haldol 0.5-10mg/day
(watch for prolonged QTc, parkinsonism, tardive dyskinesia, depression/anxiety,
and weight gain)
c) Dopamine depleter: Tetrabenazine 25-150mg/day (watch for sedation,
parkinsonism, depression; DON’T stop abruptly).
Second-line pharmacology:
-Topamax, Risperdol (0.25-3mg/day), Klonopin, Botox (for focal tics like
blephrospasm), SSRI (if concomitant psych issues)
-???? Bilateral GPi DBS.