Movement Disorders I: Tics and Stereotypies

Samuel H. Zinner and Jonathan W. Mink

Pediatrics in Review 2010;31;223
DOI: 10.1542/pir.31-6-223

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/31/6/223

Data Supplement (unedited) at:

http://pedsinreview.aappublications.org/content/suppl/2010/07/06/31.6.223.DC2.html
http://pedsinreview.aappublications.org/content/suppl/2010/05/26/31.6.223.DC1.html

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1979. Pediatrics in Review is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2010 by the American Academy of
Pediatrics. All rights reserved. Print ISSN: 0191-9601.

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012

 Article neurology

Movement Disorders I: Tics and Stereotypies

Samuel H. Zinner, MD,*
Objectives After completing this article, readers should be able to:
Jonathan W. Mink, MD,
PhD† 1. Identify the important features of tics and stereotypies.
2. Recognize comorbid conditions and overlapping qualities with tics.
3. Describe treatment approaches for tic disorders.
Author Disclosure
Drs Zinner and Mink
have disclosed no Introduction
financial relationships Movement disorders involve impairment of appropriate targeting and velocity of voluntary
relevant to this movements, dysfunction of posture, the presence of abnormal involuntary movements, or
the performance of normal-appearing movements at inappropriate or unintended times.
article. This
The abnormalities of movement are not due to weakness or abnormal muscle tone but may
commentary does not
be accompanied by weakness or abnormal tone. By convention, movement disorders are
contain a discussion divided into two major categories. The first category is hyperkinetic movement disorders,
of an unapproved/ sometimes referred to as dyskinesias. This term refers to abnormal, repetitive involuntary
investigative use of a movements and includes most of the childhood movement disorders, including tics,
commercial product/ stereotypies, chorea, dystonia, myoclonus, and tremor. The second category is hypokinetic
movement disorders, sometimes referred to as akinetic/rigid disorders. The primary
device.
movement disorder in this category is parkinsonism, manifesting primarily in adulthood as
Parkinson disease or one of many forms of secondary parkinsonism. Hypokinetic disorders
are relatively uncommon in children. Although ataxia, weakness, and spasticity are char-
acterized by motor dysfunction, by common convention these entities are not included
among “movement disorders.”
This review of movement disorders consists of two parts. Part I focuses on the most
common movement disorders of childhood: tics and stereotypies. Part II examines chorea,
dystonia, myoclonus, tremor, and drug-induced movement disorders.
Most movement disorders in childhood arise from dysfunction in basal ganglia (cau-
date, putamen, globus pallidus, subthalamic nucleus, substantia nigra) and frontal cortex.
However, the accomplishment of normal movement requires a multifaceted network of
brain regions, including basal ganglia, frontal cortex, parietal cortex, thalamus, cerebellum,
spinal cord, peripheral nerve, and muscle. Recognition of the
multiple components of the nervous system involved in
motor control is important because the etiologic diagnosis
Abbreviations: often depends on localization.
ADHD: attention-deficit/hyperactivity disorder When faced with a movement disorder, the first step is to
CBIT: comprehensive behavioral intervention characterize the movement. Is the pattern of movements
for tics normal or abnormal? Are there excessive movements or is
CRT: competing response training there a paucity of movement? Is the movement paroxysmal
DSM-IV-TR: Diagnostic and Statistical Manual (sudden onset and “offset”), continual (repeated again and
of Mental Disorders, 4th edition – again), or continuous (without stop)? Has the movement
Text Revision disorder changed over time? Do environmental stimuli or
LD: learning disability emotional states modulate the movement disorder? Can the
OCD: obsessive-compulsive disorder movements be suppressed voluntarily? Is the abnormal
SSRI: selective serotonin reuptake inhibitor movement heralded by a premonitory sensation or urge? Are
TS: Tourette syndrome there findings on the examination suggestive of focal neuro-
logic deficit or systemic disease? Is there a family history of a

*Associate Professor of Pediatrics, University of Washington School of Medicine and Seattle Children’s Hospital, Seattle, Wash.
†
Professor of Neurology, Neurobiology, & Anatomy, Brain & Cognitive Sciences, and Pediatrics, University of Rochester School
of Medicine and Dentistry and Golisano Children’s Hospital at Strong, Rochester, NY.

Pediatrics in Review Vol.31 No.6 June 2010 223

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
neurology tics & stereotypies

Table 1. Phenomenologic Classification of Movement Disorders

Movement Disorder Brief Description
Tics Stereotyped, intermittent, sudden, discrete, repetitive, nonrhythmic movements, most
frequently involving head and upper body.
Stereotypy Patterned, episodic, repetitive, purposeless, rhythmic movements.
Chorea Chaotic, random, repetitive, brief, purposeless movements that are rapid but not as
rapid as myoclonus.
Dystonia Repetitive, sustained, abnormal postures and movements; abnormal postures typically
have a twisting quality.
Myoclonus Sudden, brief, shocklike movements that may be repetitive or rhythmic.
Tremor Rhythmic oscillation about a central point or position involving any one or more
body parts.
Parkinsonism Hypokinetic syndrome characterized by rest tremor, slow movement (bradykinesia),
rigidity, and postural instability.

similar or related condition? Does the movement disor-
der abate with sleep?
In clinical practice, the diagnosis of a movement dis-
order requires a qualitative appreciation of the move-
ment type and context. Classification of the disorder
phenomenologically requires a description of the charac-
teristics of the movements (Table 1). Even under the best
circumstances, abnormal movements can be difficult to
define, and movement disorders may be difficult to char-
acterize. Chorea can resemble myoclonus. Dystonia can
resemble spasticity. Paroxysmal movement disorders
such as dystonia and tics may resemble other paroxysmal
neurologic problems, namely seizures. Movements in
some contexts may be normal and in others may indicate
underlying disease. Movements that suggest a degener-
ative disorder in adolescents (myoclonus) may be com-
pletely normal in an infant (benign neonatal myoclonus).
It can be difficult to diagnose a specific movement disor-
der without seeing the abnormal movements. Thus, ob-
taining video examples of the child’s movement may be
essential to making a correct diagnosis.
Tics
Background and Historical Overview
Tic disorders, including Tourette syndrome (TS), are
among the most common of neurologic conditions. Sur-
prisingly, however, they escaped significant scientific at-
tention until recent decades, in part due to their protean
and evolving qualities. Although tic disorders, including
TS, no longer are considered rare, misconceptions per-
sist, rooted in obsolete theories. For example, during the
era of the late 1800s, when Georges Gilles de la Tourette
first described the condition that later would bear his
name, “hysteria” and “neurosis” were the prevailing
interpretations regarding cause; later interpretations sug-
gested tics to be representations of narcissism.
The etiologic model shifted suddenly toward a neu-
rologic explanation in the early 1960s after neuroleptic
medication was discovered to diminish tic production in
patients who had TS. At that time, although tics them-
selves were viewed as common among school-age chil-
dren, TS was viewed distinctively and grimly. Medical
journals of the era described TS in such terms as “mon-
strous affliction,” offering prognostic statements that
described a uniformly poor outcome, often culminating
in personality deterioration or foreshadowing insanity,
schizophrenic psychosis, and certain commitment to
mental hospitals. So stigmatizing was the disorder that
one author recommended avoiding use of the Tourette
eponym because its pleasant sound was considered too
incongruous with the presumed grave outcome. Experts
of the era considered TS to be extremely rare, suggesting
that many physicians would never see a case. In a 1966
report, the Mayo Clinic had made the diagnosis in just 7
of 1.5 million newly admitted patients over the preceding
3 decades; another review reported only 4 cases among
59,000 admissions to a psychiatric clinic.
Today, TS and related disorders are viewed as com-
mon, although the neurobiologic underpinnings remain
poorly understood. It generally is agreed that TS has a
genetic basis and that tics arise from basal ganglia-
thalamocortical circuits. However, the specific causative
mechanisms are not known.
Definitions
Tics are nonrhythmic, repetitive, and intermittent mus-
cle contractions resulting in “stereotyped” (ie, per-
formed identically each time) movements. When these
movements involve laryngeal-pharyngeal muscles and
produce noise, tics are termed “phonic” or “vocal.” All
other tics are termed “motor,” although functionally,
phonic tics also are motoric. (For a video of a patient who

224 Pediatrics in Review Vol.31 No.6 June 2010

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012

Table 2. Tic Classifications With Examples
General Type Simple
Motor ● Sudden
● Brief
● “Meaningless”
● Isolated to muscle group: Facial and neck
(blinking, eye movements, nose twitching,
lip movements, grimacing, opening eyes
widely, neck jerks, jaw snaps, gaze shifts),
abdomen (tensing), extremities
(clenching, jerking), or other body part
Phonic ● Sudden
● “Meaningless”
● Often “allergy”-like (grunting, sniffing,
throat clearing, coughing)
● Sometimes nonvocal (voiceless expulsions
of air from mouth or nostrils, sucking,
tongue-clicking, hissing)
● Animal noises (barking, chirping,
whistling)
has Tourette syndrome demonstrating facial and vocal
tics, see the Data Supplement.)
Both motor and phonic tics can be characterized
further as “simple” or “complex” (Table 2). Simple tics
tend to appear meaningless, be anatomically isolated, or
be of brief duration but are excessive in frequency or
intensity. Complex tics may mimic a gestural or linguistic
purpose, involve several muscle groups, or be more sus-
tained, such as the holding of a posture. Other features of
complex tics can include socially unacceptable expres-
sions, such as coprolalia (utterance of foul or other
inappropriate language) and copropraxia (making offen-
sive gestures), or a self-injuring action. The range of
possible tic manifestations is virtually infinite, however,
obscuring clear boundaries between simple and complex
classifications.
The Diagnostic and Statistical Manual of Mental Dis-
orders, 4th edition-Text Revision (DSM-IV-TR), pub-
lished in 2000, classifies tic disorders as “transient” (last-
ing between 1 and 12 months) or chronic (lasting more
than 12 months). Chronic tic disorders are classified
further either as “chronic motor or vocal tic disorder” or
as “Tourette’s disorder,” the latter requiring the pres-
ence of both motor and phonic tics. Note that the term
“vocal” implies a laryngeal contribution, although some
noise-producing tics involve only naso- or oropharyngeal
muscles, such as sniffing or tongue clicking, so the term
“phonic” may be more precise. Additional criteria for all
tic disorders include tic onset prior to 18 years of age and
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
neurology tics & stereotypies
Complex
● Slower and longer
● “Purposeful” (head shaking, trunk flexion, scratching,
touching, gesturing such as waving or reaching, finger
tapping, jumping, kicking, stomping, or vulgar gesturing
[“copropraxia”] such as giving the finger, touching
forbidden body parts)
● Other possible features: Dystonic (sustained,
exaggerated, or distorted facial expressions or body
postures), imitative (“echopraxic”), self-abusive (sudden
snapping back of neck, pulling of fingers, lip-licking
causing chapping)
● Often sudden
● “Meaningful” linguistic elements (syllables, words,
phrases, or obscenities [“coprolalia”] such as “shut up,”
profanities, or uncouth social observations)
● May be imitative (“echoic”)
● Speech atypicalities (changes in speech meter, pitch,
blocking one’s speech, or repeating one’s own speech
[palilalia])
the presence of tics nearly daily or intermittently during
the qualifying period of time.
A departure from the original DSM-IV, published in
1994, is the removal of the criterion that the disturbance
must cause marked distress or significant impairment in
social, occupational, or other important areas of func-
tioning. This distinction is noteworthy because the ab-
sence of this criterion further supports the neurologic
basis of tic disorders. When the presentation does not
completely fulfill the criteria in any of the three classifi-
cations, a fourth classification, “tic disorder – not other-
wise specified,” can be chosen.
Epidemiology
Tic disorders begin in childhood, usually emerging dur-
ing the first decade. Transient motor tics occur in as
many as one in four children and may represent a normal
variant of childhood neurodevelopment. Because tics
ordinarily are mild in severity and because of their com-
monness, they often are overlooked entirely. The preva-
lence of TS today is recognized to be about 1%, a
dramatic increase from even very recent estimates. Boys
are four times as likely as girls to have TS.
Most individuals who have chronic tic disorders expe-
rience only mild and unambiguous tics, with simple
motor tics, such as eye blinking, by far the most common
and earliest manifestation. Complex tics are less common
and are very unlikely to occur as the first tic, if at all.
However, among people who have chronic tic disorders,
Pediatrics in Review Vol.31 No.6 June 2010 225
neurology tics & stereotypies
tics often become more complex over time. Among
those who have non-TS chronic tic disorders, phonic tics
are much less common than motor tics. When present,
phonic tics are much more likely to be of the simple,
rather than complex, variety. Very few people who have
TS ever develop coprolalia, despite the disproportionate
media portrayals of this often startling feature and earlier
investigators’ expectations of a dismal unfolding of
symptoms. When chronic, tic severity usually peaks in the
preadolescent years, and most affected adolescents expe-
rience significant improvement or complete resolution
into adulthood.
Pathogenesis
The pathogenesis of tic disorders is complicated and not
well understood, although a wealth of biomedical re-
search strongly supports a defective filtering, or “sensor-
imotor gating,” mechanism, resulting in urges to per-
form elements of otherwise purposeful activity at
inappropriate times, intensities, or frequencies. The cen-
tral concept focuses on the basal ganglia and their role
within brain-based circuits that also include the neocor-
tex and thalamus. Other key brain regions, such as the
midbrain, may be involved as well. When functioning
properly, the basal ganglia assist in the execution of
desired behaviors expressed by these circuits, but the
basal ganglia also function to prevent the completion of
undesired behaviors.
Many desired behaviors, when learned through repe-
tition, may become automatic or sequenced as “prepack-
aged and ready-to-use,” perhaps stored and reinforced in
these circuits. Imprecise regulation results in the expres-
sion of bits of these behaviors, such as tics. This model
also helps to explain related or comorbid conditions that,
like tics, share features of loss of behavioral inhibition,
such as compulsions or impulsive behaviors, and to ex-
plain why these behaviors often mimic purposeful activ-
ities.
Chronic primary tic disorders and many of their asso-
ciated conditions are strongly influenced genetically, al-
though the inheritance pattern is unclear. Environmental
factors, such as sleep insufficiency, stress, and possibly in
utero exposure to maternal smoking, also are important
as mediators of symptom severity. In 2005, the first
reported gene associated with TS was identified on chro-
mosome 13 in 3 of 174 patients, although subsequent
studies of this gene’s association with TS have yielded
mixed findings. In 2007, strong linkage to chromosome
2p was reported in a large genome scan. It is likely that
the genetics of TS are more complex than previously
believed. An autosomal dominant mode of transmission
226 Pediatrics in Review Vol.31 No.6 June 2010
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
with incomplete penetrance and possible polygenic and
additive factors and environmental influences seems
likely.
Clinical Aspects
Transient tic disorders may be milder than chronic con-
ditions and are less likely to be associated with other
developmental and behavioral conditions. However, it is
important to note that diagnostic criteria make no men-
tion of tic severity. Tics in TS may be mild and unrecog-
nized by casual observers. When tics are chronic, they
typically change in anatomic location, frequency, type,
complexity, and severity or interference. New tics may
replace older ones or add to a growing repertoire. Other
important features of tic disorders include a waxing and
waning course, often observed as bouts of tics over
periods of seconds, minutes, hours, days, weeks, months,
or longer.
Chronic tic disorders frequently are associated with
one or more comorbid conditions (Table 3), and it is
these conditions, rather than the tics themselves, that
most often are the source of the greatest psychosocial and
functional challenges. Children who have TS but no
comorbid conditions probably have a similar quality of
life and function as children who have no TS. As de-
scribed earlier, the feature of behavioral disinhibition in
tics is shared with some aspects of attention-deficit/
hyperactivity disorder (ADHD) as well as obsessive-
compulsive disorder (OCD) and obsessive-compulsive
behaviors and may suggest common neurologic under-
pinnings. In fact, ADHD occurs in at least 50% of clini-
cally referred children who have TS, and OCD occurs in
about 33%. Symptoms of ADHD usually precede the
onset of tics in most individuals, often presenting during
the toddler and preschool years.
Comorbid Conditions of
Table 3.
Tourette Syndrome
● Attention-deficit/hyperactivity disorder
● Obsessive-compulsive disorder and obsessive-
compulsive behaviors
● Learning disabilities/learning difficulties
● Anxiety disorders
● Mood disorders
● Oppositional defiant disorder
● Self-injurious behaviors
● Speech and language disorders (eg, hesitations,
disfluency)
● Intermittent explosive disorder/anger dysregulation

Learning disabilities (LDs) are another common co-
occurring problem, although children who have TS may
face obstacles to effective learning due to reasons other
than LD, including ADHD, obsessive thoughts or com-
pulsions, and complications due to other comorbid con-
ditions, such as anxiety, poor frustration tolerance, insuf-
ficient sleep, and executive function difficulties such as
poor organizational skills. Tics themselves can interfere
with operations such as reading, writing, listening, and
speaking. In addition, tics can be distracting and some-
times physically challenging, further jeopardizing the
child’s ability to attend.
Explosive anger and aggression, or episodic “rage
attacks,” are seen in 25% or more of referred patients who
have TS, and parents overwhelmingly regard these events
as the most problematic feature. Their origin likely is
multifactorial, related both to primary disorders and
secondary consequences of having a chronic disorder.
These attacks may be related to comorbid conditions,
including aggressive obsessions or other anxieties, loss of
impulse control or other disinhibited urges, and hyper-
arousal.
Although tics often improve or resolve in adolescence
and young adulthood, it is not yet possible to predict a tic
course for individual patients. A growing identification of
endophenotypes, such as fine motor skills deficits, is
emerging that may suggest degrees of basal ganglia dys-
function that could help serve as clues to predict greater
tic severity in adulthood.
Diagnosis
Diagnosing tic disorders is clinical and usually straight-
forward, not requiring any laboratory or imaging studies.
Most often, parents or patients self-diagnose and seek
confirmation from a specialist. Simple motor tics seldom
pose diagnostic uncertainty; complex tics and phonic tics
may be more difficult to discern. Although tics may
resemble chorea or myoclonus in some cases, the pres-
ence of a premonitory sensory urge that is relieved by tics
and the ability to suppress tics voluntarily distinguish tics
from true involuntary movement disorders. Rarely, tics
can be symptomatic of other disorders (Table 4). In
those cases, there usually are other signs or symptoms to
distinguish such secondary tic disorders from primary tic
disorders. A comprehensive history (including family
history of tics and associated problems) and physical
examination can rule out most of these causes. If there is
diagnostic uncertainty, referral to a neurologist is indi-
cated.
Additional screening and ongoing monitoring to as-
sess for the presence of any of the psychosocial, behav-
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
neurology tics & stereotypies
Possible Causes of
Table 4.
Secondary Tics
● Infections and postinfectious sources
● Drugs
● Toxins
● Neurodevelopmental disorders (eg, pervasive
developmental disorders, intellectual disabilities)
● Chromosomal disorders (eg, trisomy 21)
● Stroke
● Neoplasm
● Heredodegenerative disorders
– Huntington disease
– Neuroacanthocytosis
– Pantothenate kinase-associated neurodegeneration
(PKAN)
– Wilson disease
● Neurocutaneous disorders
● Head trauma
● Seizure disorders
● Psychogenic factors
ioral, learning, and developmental comorbid conditions
are essential in providing comprehensive care. When
screening identifies concerns beyond the scope of general
primary care diagnosis or management, specific consul-
tation or referral for more specialized medical, psycho-
social, or psychoeducational evaluations may be indi-
cated. An evaluation by a medical specialist, such as a
developmental/behavioral pediatrician, psychiatrist, or
neurologist, includes an assessment of past medical, fam-
ily, and developmental history; a social and environmen-
tal overview; a review of the onset and course of tics and
related problems; and a complete physical examination
with direct clinical observation. When screening suggests
the presence of obstacles to optimal academic and social
performance, psychosocial and psychoeducational assess-
ments within the school district or the private domain are
indicated.
Management
GENERAL PRINCIPLES. Management starts with edu-
cation about tic disorders as neurologically based, which
often includes reversing long-held misconceptions and
myths. Solicitation of beliefs and concerns from patients
and families can help guide educational approaches. For
example, awareness that tics may be suppressible can
mislead parents or teachers to assume that tics are delib-
erate. Also, attention to the tics may be misplaced or
overemphasized, and when overlooked, comorbidity
may be a much more significant source of functional
Pediatrics in Review Vol.31 No.6 June 2010 227
neurology tics & stereotypies
difficulty. At the same time, comorbid challenging be-
haviors may be regarded as an irremediable part of the
broad TS spectrum and not amenable to disciplinary
guidance, an assumption that is invalid. Comorbid prob-
lems often accompany disruption in family and peer
relations, and effective intervention relies first and fore-
most on successful, positive parenting foundations. Pro-
fessional family, individual, or parenting counseling may
be considered.
EDUCATION. The first approach to managing tic dis-
orders should be providing education and reassurance.
Parents, patients, educators, and peers may benefit from
education about tics and TS. An understanding of how
symptoms of tics and comorbid problems affect the
patient, family, and others can help to prioritize and
establish a sensible direction and degree of intervention.
In recent years, an abundance of new educational re-
sources on TS and related conditions has become avail-
able, owing, in part, to increased awareness and research
as well as to a collaborative agreement between the
Tourette Syndrome Association and the Centers for Dis-
ease Control and Prevention. Some of these materials are
listed at the end of the article under “DVD Educational
Programming.”
When tics are chronic, anticipatory guidance can help
avert needless interventions. In these cases, the basis of
management focuses on forming an identity separate
from the tic disorder and maintaining and supplement-
ing the development of academic, organizational, and
interpersonal skills with an eye to ensuring a satisfying,
independent adulthood. When tics cause impairment,
specific treatment of the tics may be indicated. It is
important to establish clear treatment goals, with the
expected outcome being the reduction rather than the
eradication of tics. For most, however, ongoing moni-
toring and reassuring periodic reminders that chronic tics
usually improve during adolescence are adequate tic
management.
Children who have special educational needs should
be evaluated in this area and identified needs addressed
accordingly. Such needs may include complications due
to tics. In 2006, the United States Department of Edu-
cation, Office of Special Education Programs, formally
included TS as a condition in the category of “Other
Health Impairment,” eligible for special education ser-
vices under the Individuals with Disabilities Education
Act. Additional information is available through the
United States Department of Education at http://
idea.ed.gov. The “Education/Education Advocacy” link
228 Pediatrics in Review Vol.31 No.6 June 2010
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
on the Tourette Syndrome Association web site provides
a wealth of suggestions and guidance.
BEHAVIORAL APPROACH. Comprehensive behavioral
intervention for tics (CBIT) is a behavior-based strategy
that has shown preliminary evidence of efficacy; addi-
tional investigation is underway. The theoretical princi-
ple behind this strategy relies on the roles that internal
and external environmental influences contribute to pro-
moting tics and assumes that tics are, in part, negatively
reinforced learned behaviors. This interpretation means
that tics are performed to some degree because they
eliminate the negative experience of the premonitory
sensory urge, thereby providing temporary relief to the
TS sufferer, and the repetition of tics and their associated
experiences of relief reinforce the tic behavior. Accord-
ingly, CBIT employs an individualized “competing re-
sponse training” (CRT) with “functional analysis” to
disrupt the urge-tic-relief cycle that, when successful,
seems ultimately to weaken the urge. The nuts and bolts
of CRT involve strengthening the patient’s awareness of
the internal environment (ie, premonitory urge) so as to
anticipate an impending tic and gradually replace it with
a competing tic-incompatible behavior. The functional
analysis piece identifies and modifies reinforcements in
the external environment that promote tics (eg, parents
inappropriately indulging a child’s wish not to do home-
work to avoid anxiety and resultant tic exacerbation) as
well as those that reduce tics (eg, appropriately praising a
child for practicing CRT).
Pharmacotherapy
A variety of psychotropic medications is available and
potentially useful for the amelioration of behavioral and
psychosocial symptoms due to tics and comorbid condi-
tions. Medication management of patients who have TS
usually addresses problems related to tics, ADHD, anxi-
ety (including OCD), or a combination. Although each
diagnostic condition must be considered individually
(“splitting”), contributions of self-esteem, parenting
practices, social milieus of family and community, learn-
ing or organizational barriers, temperament, and other
elements strongly influence functional outcomes in all
aspects of the patient’s illness. Effective interventions rely
on the identification and monitoring of these modifiable
factors, a point that should be underscored and repeated.
TICS. Historically, even mild tics were treated medi-
cally with “typical neuroleptics” (older-generation anti-
psychotics), but today a more conservative approach is
recommended. Features of tic severity, including fre-
 neurology tics & stereotypies

Table 5. Medications Used for Tic Reduction decisions are informed largely by
results from trials of adults and by
Alpha-2-adrenergic Commonly prevailing practice patterns. Devel-
agonists ● Clonidine (0.025 to 0.4 mg/day divided 3 to 4 times/ opment of research units in pediat-
day) ric psychopharmacology has helped
● Guanfacine (0.5 to 4 mg/day divided 1 to 2 times/day)
to rectify these serious limitations,
Typical neuroleptics Commonly
● Haloperidol (0.25 to 4 mg/day divided 2 times/day)* and progress is ongoing but very
● Pimozide (0.5 to 8 mg/day divided 2 times/day)* slow.
Less Commonly The primary indication for treat-
● Fluphenazine (0.5 to 10 mg/day) ment is the extent to which tics
Atypical Commonly
bother the child. Tics of mild sever-
neuroleptics ● Risperidone (0.25 to 3 mg/day divided 2 times/day)
● Ziprasidone (10 to 100 mg/day divided 2 times/day) ity do not require pharmacologic
Less Commonly intervention. Medication may be
● Aripiprazole (2.5 to 20 mg/day divided 2 times/day) indicated for moderate-to-severe
● Olanzapine (2.5 to 12.5 mg/day divided 2 times/day) tics, but reasonable expectations
Benzodiazepine Less Commonly
must be understood. Table 5 pre-
● Clonazepam (0.125 to 1.5 mg/day divided 1 to
3 times/day) sents medications listed by class
Other Less Commonly that are used for tic reduction. Sca-
● Botulinum toxin hill and associates have prepared
*Approved by the United States Food and Drug Administration for use in children who have Tourette more detailed guidelines (see Sug-
syndrome. gested Reading). Some therapeutic
combinations can benefit both tics
and comorbid complications and
quency, functional interference, distraction, intensity, may help guide medication selection. For example,
self-injurious aspects, and social implications, help to alpha-2-adrenergic agonists can assist in tic reduction
guide decisions regarding treatment, and all approaches and may aid some in reducing symptoms of ADHD and
are individualized. No medication has been designed insomnia; atypical neuroleptics can be useful for tic re-
specifically for tic reduction, and none eliminates tics duction and comorbid aggression.
entirely. A reasonable goal is to reduce tic severity to a A conservative medication approach for moderate-to-
state of tolerable functional outcome that has acceptable severe tics begins with an alpha-2-adrenergic agonist.
adverse effects. Because tics tend to increase during times Families should be informed that these drugs may re-
of stress, it may be useful to provide reassurance and quire up to 2 months to achieve benefit. The neuroleptic
monitoring during such periods, rather than initiating or agents are the next option. As evidence grows for their
resuming medication control. In so doing, the child or efficacy in tic reduction in combination with atypical
adolescent may learn to “ride the wave” and recognize neuroleptics, this class commonly now is chosen over the
that successful relief from tics can come with patience, typical neuroleptics because the atypical agents are less
while also helping to build attitudinal tolerance to the likely to cause extrapyramidal effects such as restlessness
natural ebbs and flows of the disorder. It is important to or acute dystonic reactions. In addition, their risk for the
recognize that the waxing and waning pattern persists development of tardive dyskinesia is considered to be
during medication use. Therefore, changes in tic severity substantially less relative to typical neuroleptics. How-
or expression should not be assumed to be a result of the ever, adverse effects, including significant weight gain,
medication. sedation, “foggy” thinking, gynecomastia, and glucose
When it is decided to use medication, the most con- intolerance with the risk for diabetes mellitus, are fre-
sistently effective available tic-reducing agents work by quent and often poorly tolerated. Alternative classes of
blocking dopamine neurotransmission within regions of agents, such as benzodiazepines, are sometimes helpful.
the basal ganglia. Both typical and atypical neuroleptics Botulinum toxin can be useful for an isolated severe
are useful, although adverse effect profiles limit their tic when injected by a neurologist directly into the of-
selection as first-line agents. Research trials of children fending muscle group. Its therapeutic impact is limited
and adolescents investigating the safety and efficacy of to the injected anatomic region and lasts about 3
medications for tic reduction have been very limited, months. Of course, any desired function of the affected
despite a growing array of available treatments. Clinical muscle group also is curtailed by this intervention.

Pediatrics in Review Vol.31 No.6 June 2010 229

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
neurology tics & stereotypies
COMORBID ADHD. When present, ADHD often im-
poses more severe functional interference than do tics.
Stimulant medications are the first-line agents in the
treatment of ADHD, even when comorbid with tic dis-
orders. Until recently, the presence of TS contraindi-
cated the use of stimulants for the treatment of ADHD in
clinical practice, and the Physicians’ Desk Reference con-
tinues to list motor tics and family history or diagnosis of
TS among their contraindications. The assumptions of
tic induction or exacerbation with stimulant use have
been reinforced by case reports dating to the 1970s as
well as by the natural time courses for the emergence of
symptoms of ADHD (prior to age 7 years) and tics
(around age 7 years), respectively. As such, the concom-
itant introduction of stimulant medication in a patient at
approximately the time that she or he might otherwise
have been destined to develop tics or perhaps during a
natural waxing phase reinforces the impression of causal-
ity.
In addition, stimulant medications work by increasing
dopamine neurotransmission, and because dopamine-
blocking agents decrease tics, it is reasonable to assume
that stimulants increase tics. However, data from recent
placebo-controlled studies simply do not support this
prohibition for most patients. Nevertheless, it is a sensi-
ble and safer practice to alert families to the possibility of
tic appearance or exacerbation in children who have tics.
For some children, tics increase and may not decrease, as
can be the nature of tic disorders. Families must under-
stand this, and counseling should emphasize that stimu-
lants do not cause tics de novo or permanently increase
tic severity. Reminding families of the natural waxing and
waning feature of tics as well as raising awareness of
predictable environmental influences that may increase
tics, such as stresses imposed by excitement, a holiday, or
the beginning of a school year, may help to assuage
apprehensions during inevitable upsurges.
Standard stimulant dosing schedules and customary
medical precautions apply. Nonstimulant medications
also are available for the treatment of ADHD, including
alpha-2-adrenergic agonists, tricyclic antidepressants,
and newer antidepressants. For some patients, combined
treatment with a stimulant and alpha-2-adrenergic ago-
nist may provide improved outcomes relative to either
agent alone. There now is some evidence that treating
ADHD with the selective norepinephrine reuptake in-
hibitor atomoxetine may reduce tic severity.
OCD. The selective serotonin reuptake inhibitors
(SSRIs) are the first-line agents for the treatment of
comorbid OCD. Currently, no specific SSRI shows
230 Pediatrics in Review Vol.31 No.6 June 2010
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
unique advantages. Their effectiveness may be enhanced
when used in combination with CBIT, particularly expo-
sure and response prevention. Second-line agents, such
as clomipramine, may be chosen when patients fail
two SSRI trials. Families must be made aware of the
United States Food and Drug Administration “black
box” warning regarding the use of antidepressants in
children and adolescents. Useful support for families
and practitioners has been prepared by The American
Psychiatric Association and the American Academy of
Child and Adolescent Psychiatry and is available at http:
//ParentsMedGuide.org.
Prognosis
Long-term prognosis in TS has not been well studied, in
large part because the condition was considered rare until
very recently. However, available data and clinical expe-
rience reveal that chronic tic disorders wax and wane
regardless of intervention and most often improve sub-
stantially or resolve entirely in mid-to-late adolescence.
The likelihood of the patient experiencing ongoing se-
vere tics in adulthood is low. The greater prognostic risks
for continued challenges and disability are seen not in tics
but rather in comorbid conditions, such as ADHD,
OCD, LD, anxiety, depression, and poor anger control.
Competent interpersonal and coping skills with effective
behavioral and emotional self-control afford tremendous
support toward a positive psychosocial outcome, irre-
spective of disease severity or course. Therefore, identi-
fying and building these qualities should form the back-
bone of any management design.
Stereotypies
Stereotypies are patterned, repetitive, purposeless, invol-
untary movements that also are rhythmic and continual
and tend to change little over time. In contrast, tics are
nonrhythmic and discrete, typically change in location
and type over time, and wax and wane in frequency and
severity. Examples of stereotypies include body rocking,
head nodding, walking in circles, hand flapping or clap-
ping, finger wiggling, and facial grimacing. Some stereo-
typed movements may occur in the setting of object
manipulations, including spinning or twirling items, but
manipulation of objects is not required for stereotypies to
be identified as such. Other terms have been used to
describe stereotypies, including “rhythmic habit pat-
terns,” “gratification phenomena,” “self-stimulation,”
and “motor rhythmias.”
Stereotypies are associated most commonly with au-
tism and other developmental disabilities. Indeed, ste-
reotypies are present in most autistic children. Stereotyp-

ies also may be seen in children who have sensory
impairments, specifically in blind or deaf children. Fur-
thermore, stereotypies may occur in children subjected
to severe environmental deprivation or restriction. How-
ever, stereotypies also occur in children who have no
developmental disabilities, sensory impairments, or social
deprivation.
Natural History
Unfortunately, few data exist on the natural history of
stereotypies. Stereotyped, repetitive, rhythmic move-
ments are common and, indeed, characteristic of infants
and toddlers. Particular types of stereotypy develop in
correlation with motor development. In most children,
such movements decrease and ultimately cease. Stereo-
typies typically are present before 3 years of age in both
autistic and nonautistic children. As children get older,
stereotypies may diminish but can persist into adulthood
in both autistic and nonautistic children.
Neurobiology
The neurobiologic mechanisms underlying stereotypies
are not known. However, there are reasons to think that
like most other involuntary movements, they arise from
the basal ganglia or from cortical-basal ganglia-
thalamocortical circuits. There may be important roles
for dopamine and serotonin in the maintenance of ste-
reotyped repetitive behaviors as well.
Physiologic Stereotypies
Stereotypies are associated most commonly with devel-
opmental disabilities. However, it has been estimated
that stereotypies occur in up to 7% of nondisabled chil-
dren. The term “physiologic stereotypies” has been sug-
gested when the movements occur in otherwise healthy
children. Typical stereotypies include repeated, recurrent
raising and lowering of the arms, flapping, waving, wrist
rotation, and finger wiggling. Such motions often are
accompanied by facial movements or grimacing. Physio-
logic stereotypies may be present in any setting but occur
most commonly when the child is excited, mentally
engaged, stressed, or bored and may increase with fa-
tigue. A hallmark of stereotypies is that they usually cease
when the child is distracted or engaged in a new activity.
Most children appear to be unaware of the stereotypies.
The typical age of onset for physiologic stereotypies is
younger than 2 years. These motions are more common
in boys (almost 2:1). Unlike tics, stereotypies tend not to
change in anatomic location or complexity over time.
Stereotypies are not preceded by an urge or thought, as is
common with tics or compulsions. Physiologic stereo-
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
neurology tics & stereotypies
typies last for many years in most children. In some
children, they disappear over time; in others, they persist
into adulthood. There may be an increased incidence of
ADHD or LD among children who have stereotypies.
Treatment
When approaching the treatment of stereotypies in any
child, the first question should be whether to treat. If the
behaviors are not causing discomfort, injury, or social
impairment, it may be preferable not to treat them. If
they are causing difficulty, treatment may be indicated.
Treatments include pharmacologic and behavioral ap-
proaches.
Many pharmacologic treatments have been reported
in single cases or in small series. However, most are small,
open-label designs subject to the usual problems of such
studies, that is, placebo effect and observer bias. Clomi-
pramine, risperidone, and fluoxetine have been shown to
reduce repetitive behaviors in children and adolescents
who have autism. However, there has been no systematic
study of pharmacologic therapy in nonautistic children.
Our clinical experience is that pharmacotherapy with
available agents is of limited use in the treatment of
stereotypies. The evidence for behavioral treatment with
habit reversal therapy and differential reinforcement of
other behaviors is a bit stronger, but still is preliminary
(see Miller and associates in Suggested Reading).
Summary
● Tics and stereotypies are among the most commonly
seen movement disorders in childhood.
● Tics and stereotypies share several features, including
stereotyped appearance of each movement, repetitive
nature, and involuntary production.
● Important distinguishing features include typical age
of onset, presence or absence of premonitory “urge,”
association with other symptoms, and response to
medication.
● For both types of disorders, education and
reassurance may be sufficient. For tics, effective
pharmacologic and behavioral therapy is available.
For stereotypies, consistently effective treatment has
not been described.
● Transient tics are the most common form of tic
disorder.
● Tourette syndrome is characterized by the chronic
presence of motor and phonic tics.
● In general, tic disorders and stereotypies improve as
individuals enter adulthood, but this is not a uniform
occurrence.
Pediatrics in Review Vol.31 No.6 June 2010 231
neurology tics & stereotypies
Suggested Reading
Mahone EM, Bridges D, Prahme C, Singer HS. Repetitive arm and
hand movements (complex motor stereotypies) in children.
J Pediatr. 2004;145:391–395
Miller JM, Singer HS, Bridges DD, Waranch HR. Behavioral
therapy for treatment of stereotypic movements in nonautistic
children. J Child Neurol. 2006;21:119 –125
Scahill L, Erenberg G, Berlin CM, et al. Contemporary assessment
and pharmacotherapy of Tourette syndrome. NeuroRx. 2006;
3:192–206
Woods DW, Piacentini JC, Walkup JT. Treating Tourette Syndrome
and Tic Disorders – A Guide for Practitioners. New York, NY:
The Guilford Press; 2007
DVD Educational Programming
Home Box Office, Inc. and the Tourette Syndrome Association. I
Have Tourette’s But Tourette’s Doesn’t Have Me. 2005. Available
from: The Tourette Syndrome Association, 42-40 Bell Boule-
vard, Bayside, NY 11361-2820, Phone: (718) 224-2999, Fax:
(718) 279-9596, Web site: http://www.tsa-usa.org
232 Pediatrics in Review Vol.31 No.6 June 2010
Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
Tourette Syndrome Association and the National Center on Birth
Defects and Developmental Disabilities at the Centers for Dis-
ease Control and Prevention. Diagnosing and Treating Tourette
Syndome. 2004. Two-disc set available from: The Tourette
Syndrome Association, 42-40 Bell Boulevard, Bayside, NY
11361-2820, Phone: (718) 224-2999, Fax: (718) 279-9596,
Web site: http://www.tsa-usa.org
Tourette Syndrome Association and the National Center on Birth
Defects and Developmental Disabilities at the Centers for Dis-
ease Control and Prevention. TSA Youth Ambassador Program.
2006. Available from: The Tourette Syndrome Association,
42-40 Bell Boulevard, Bayside, NY 11361-2820, Phone:
(718) 224-2999, Fax: (718) 279-9596, Web site: http://
www.tsa-usa.org
Internet Resources
Parents Med Guide. http://ParentsMedGuide.org
Tourette Syndrome Association, 42-40 Bell Boulevard, Bayside,
NY 11361-2820, Phone: (718) 224-2999, Fax: (718) 279-
9596, Web site: http://www.tsa-usa.org
 neurology tics & stereotypies

PIR Quiz
Quiz also available online at: http://pedsinreview.aappublications.org.

1. A 9-year-old boy presents for evaluation of stereotypic movements noted over the past year by his teacher.
Among the following, the most common chronic tic disorder is:
A. Coprolalia.
B. Copropraxia.
C. Eye blinking.
D. Sniffing.
E. Tongue clicking.

2. All of the following are commonly comorbid with chronic tics except:
A. Anxiety disorder.
B. Attention-deficit/hyperactivity disorder.
C. Hypersomnia.
D. Obsessive-compulsive disorder.
E. Rage attacks.

3. A mother seeks treatment for her 9-year-old son, who is bothered by tics that are moderately disruptive in
the classroom. Of the following, the most appropriate first-line pharmacologic approach is:
A. Aripiprazole.
B. Clonazepam.
C. Guanfacine.
D. Haloperidol.
E. Risperidone.

4. In counseling the father of a boy who has chronic tics and attention-deficit/hyperactivity disorder, the
most appropriate statement is that:
A. Atomoxetine should be avoided.
B. Combined treatment with methylphenidate and clonidine may improve outcome.
C. Selective serotonin reuptake inhibitors should be considered.
D. Stimulant medications can increase tic severity permanently.
E. Waxing and waning of tics is rare.

5. The mother of a 3-year-old boy who has autism seeks evaluation for the boy’s repetitive, purposeless, and
rhythmic movements. Of the following, the movement most likely to represent a stereotypy is:
A. Blinking.
B. Hand flapping.
C. Masturbating.
D. Spitting.
E. Trichotillomania.

Pediatrics in Review Vol.31 No.6 June 2010 233

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012
 Movement Disorders I: Tics and Stereotypies
Samuel H. Zinner and Jonathan W. Mink
Pediatrics in Review 2010;31;223
DOI: 10.1542/pir.31-6-223

Updated Information & including high resolution figures, can be found at:
Services http://pedsinreview.aappublications.org/content/31/6/223
References This article cites 3 articles, 1 of which you can access for free at:

http://pedsinreview.aappublications.org/content/31/6/223#BIBL
Permissions & Licensing Information about reproducing this article in parts (figures,
tables) or in its entirety can be found online at:
/site/misc/Permissions.xhtml
Reprints Information about ordering reprints can be found online:
/site/misc/reprints.xhtml

Downloaded from http://pedsinreview.aappublications.org/ by AAP AAP on January 23, 2012