Handbook of Tourette''''s Syndrome and Related Tic and Behavioral Disorders, Second Edition doc

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5316-X_Kurlan_Prelims_R2_090904

Preface
When the first edition of this book was published in 1993, I commented how
notions regarding Tourette’s syndrome (TS) had undergone recent dramatic
changes. Major shifts in views of the disorder included identification of its
complex spectrum of clinical features (including tics and specific behavioral
disorders, particularly obsessive-compulsive disorder and attention deficit
hyperactivity disorder), establishment of heredity as a major etiological
factor, and recognition that, rather than a rare disorder, it occurs quite
commonly in the population.
Views of TS continue to evolve rapidly, resulting in the need to convey
this important information in a second edition. New data suggest that the TS
behavioral spectrum extends to include anxiety disorders and rage attacks.
More and more complexities in the hereditary transmission pattern of TS are
becoming apparent, posing new challenges in the longstanding research
attempts to identify involved genes. Recent epidemiological studies indicate
that perhaps 1% of all schoolchildren have TS, with up to 25% of children
with school problems demonstrating tics. A novel potential cause of at least
some cases of TS has been proposed, namely, a poststreptococcal autoim-
mune process, which remains highly controversial. There have also been
major advances in the treatment of TS with the availability of atypical
antipsychotic drugs, guanfacine, new long-acting stimulants, and more anti-
obsessional drugs. In short, important developments have occurred in
virtually all the topics covered in the first edition, making the publication of
this second edition important and timely.
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iii
5316-X_Kurlan_Preface_R1_073004
Appreciation and thanks are extended to the authors, who have
provided clear, concise, and up-to-date information. I hope this book will
help you appreciate TS as a fascinating condition that will help us learn about

the most basic aspects of human behavior.
Roger Kurlan
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Prefaceiv
Contents
Preface iii
Contributors ix
I. PHENOMENOLOGY OF THE TIC DISORDER
1. Motor and Vocal Tics 1
Stanley Fahn
2. Premonitory (‘‘ Sensory’’ ) Experiences 15
Tamara M. Pringsheim and Anthony E. Lang
3. The Natural History of Gilles de la Tourette Syndrome 23
Ruth Dowling Bruun and Cathy L. Budman
II. ASSOCIATED BEHAVIORAL DISORDERS
4. Obsessive-Compulsive Disorder and Self-Injurious
Behavior 39
Valsamma Eapen, Jessica W. Yakeley,
and Mary May Robertson
5. New Directions in the Treatment of Comorbid Attention
Deficit Hyperactivity Disorder and Tourette’s Syndrome 89
Donna R. Palumbo
MD: KURLAN, JOB: 03329, PAGE: v
v
5316-X_Kurlan_Contents_R2_090904
6. Anxiety and Other Comorbid Emotional Disorders 109
Barbara J. Coffey, Deborah Frisone,
and Loren Gianini
7. Aggressive Symptoms and Tourette’s Syndrome 127

Cathy L. Budman, Lori Rockmore,
and Ruth Dowling Bruun
III. DIAGNOSIS AND ASSESSMENT
8. Primary Tic Disorders 155
Gerald Erenberg
9. Tics in Other Neurological Disorders 173
Joseph Jankovic and Carolyn Kwak
10. Drug-Induced Tics 195
Karen E. Anderson and William J. Weiner
11. Rating Tic Severity 215
Roger Kurlan and Michael P. McDermott
12. Neuropsychological Function in Tourette’s
Syndrome 237
Peter G. Como
IV. NEUROBIOLOGY
13. Basal Ganglia Circuits and Thalamocortical Outputs 253
Jonathan W. Mink
14. Neurobiological Issues in Tourette’s Syndrome 273
Harvey S. Singer and Karen Minzer
15. Infection and Autoimmune Factors in Tourette’s
and Related Disorders 319
William M. McMahon and Michael Johnson
16. Imaging in Tourette’s Syndrome 351
Andrew Feigin and David Eidelberg
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Contentsvi
V. GENETICS AND EPIDEMIOLOGY
17. The Inheritance Pattern 365
Maria C. Rosario-Campos and David L. Pauls

18. Progress in Gene Localization 379
Cathy L. Barr
19. Epidemiology of Tourette’s Syndrome 399
Caroline M. Tanner
VI. CLINICAL CARE
20. The Treatment of Tics 411
Christopher G. Goetz and Stacy Horn
21. Obsessive-Compulsive Disorder in Tourette’s
Syndrome: Treatment and Other Considerations 427
Robert A. King, Diane Findley,
Lawrence Scahill, Lawrence A. Vitulano,
and James F. Leckman
22. The Treatment of Comorbid Attention-Deficit Disorder
and Tourette’s Syndrome 455
Laurie Brown and Leon S. Dure
23. The Neurosurgical Treatment of Tourette’s
Syndrome 467
Chris van der Linden, Henry Colle, Elisabeth M. J.
Foncke, and Richard Bruggeman
24. Genetic Counseling 475
P. Michael Conneally
VII. SPECIAL TOPICS
25. The Child and Adolescent with Tourette’s Syndrome:
Clinical Perspectives on Phenomenology
and Treatment 481
James F. Leckman and Donald J. Cohen
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Contents vii
26. Tourette’s Syndrome: A Human Condition 505

Oliver Sacks
27. The Tourette Syndrome Association, Inc. 511
Sue Levi-Pearl
Index 519
About the Editor 535
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Contentsviii
Contributors
Karen E. Anderson University of Maryland School of Medicine, Baltimore,
Maryland, U.S.A.
Cathy L. Barr University of Toronto, The Toronto Western Hospital Re-
search InstituteandTheHospital for Sick Children, Toronto, Ontario, Canada
Laurie Brown The University of Alabama at Birmingham, Birmingham,
Alabama, U.S.A.
Richard Bruggeman University Hospital Groningen, Groningen, The
Netherlands
Ruth Dowling Bruun New York University School of Medicine, Westhampton
Beach, New York, U.S.A.
Cathy L. Budman New York University School of Medicine, New York and
North Shore University Hospital, Manhasset, New York, U.S.A.
Barbara J. Coffey New York University Child Study Center, New York,
New York, U.S.A.
Donald J. Cohen
yyyyy
Yale University School of Medicine, New Haven, Con-
necticut, U.S.A.
MD: KURLAN, JOB: 03329, PAGE: ix
ix
y

Deceased.
5316-X_Kurlan_Contributors_R2_090904
Henry Colle St. Lucas Hospital Ghent, Ghent, Belgium
Peter G. Como University of Rochester School of Medicine and Dentistry,
Rochester, New York, U.S.A.
P. Michael Conneally Indiana University School of Medicine, Indianapolis,
Indiana, U.S.A.
Leon S. Dure The University of Alabama at Birmingham, Birmingham,
Alabama, U.S.A.
Valsamma Eapen UAE University, United Arab Emirates and University
College London Medical School, London, England
David Eidelberg North Shore-Long Island Jewish Research Institute, North
Shore University Hospital, Manhasset and New York University School of
Medicine, New York, New York, U.S.A.
Gerald Erenberg Cleveland Clinic Foundation, Cleveland, Ohio, U.S.A.
Stanley Fahn Columbia University College of Physicians & Surgeons and
The Neurological Institute of New York, Presbyterian Hospital, New York,
New York, U.S.A.
Andrew Feigin North Shore-Long Island Jewish Research Institute, North
Shore University Hospital, Manhasset and New York University School of
Medicine, New York, New York, U.S.A.
Diane Findley Yale University School of Medicine, New Haven, Connect-
icut, U.S.A.
Elisabeth M. J. Foncke AmsterdamMedicalCenter,Amsterdam,The
Netherlands
Deborah Frisone McLean Hospital, Belmont, Massachusetts, U.S.A.
Loren Gianini Massachusetts General Hospital, Cambridge, Massachu-
setts, U.S.A.
Christopher G. Goetz Rush University, Chicago, Illinois, U.S.A.
Stacy Horn Rush University, Chicago, Illinois, U.S.A.

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Contributorsx
Joseph Jankovic Baylor College of Medicine, Houston, Texas, U.S.A.
Michael Johnson University of Utah, Salt Lake City, Utah, U.S.A.
Robert A. King Yale University School of Medicine, New Haven, Connecticut,
U.S.A.
Roger Kurlan University of Rochester Medical Center, Rochester, New
York, U.S.A.
Carolyn Kwak Baylor College of Medicine, Houston, Texas, U.S.A.
Anthony E. Lang Toronto Western Hospital, Toronto, Ontario, Canada
James F. Leckman Yale University School of Medicine, New Haven,
Connecticut, U.S.A.
Sue Levi-Pearl Tourette Syndrome Association, Inc., Bayside, New York,
U.S.A.
Michael P. McDermott University of Rochester Medical Center, Rochester,
New York, U.S.A.
William M. McMahon University of Utah, Salt Lake City, Utah, U.S.A.
Jonathan W. Mink University of Rochester School of Medicine and Den-
tistry, Rochester, New York, U.S.A.
Karen Minzer Johns Hopkins University School of Medicine, Baltimore,
Maryland, U.S.A.
Donna R. Palumbo University of Rochester School of Medicine and Den-
tistry, Rochester, New York, U.S.A.
David L. Pauls Massachusetts General Hospital and Harvard University
School of Medicine, Boston, Massachusetts, U.S.A.
Tamara M. Pringsheim Toronto Western Hospital, Toronto, Ontario, Canada
Mary May Robertson The National Hospital for Neurology and Neurosur-
gery and University College London Medical School, London, England
5316-X_Kurlan_Contributors_R2_090904

MD: KURLAN, JOB: 03329, PAGE: xi
Contributors xi
Lori Rockmore Mount Sinai School of Medicine, New York, New York, U.S.A.
Maria C. Rosario-Campos Yale University School of Medicine, New Ha-
ven, Connecticut, U.S.A.
Oliver Sacks Albert Einstein College of Medicine, Bronx, New York,
U.S.A.
Lawrence Scahill Yale University School of Medicine, New Haven, Con-
necticut, U.S.A.
Harvey S. Singer Johns Hopkins University School of Medicine, Baltimore,
Maryland, U.S.A.
Caroline M. Tanner Parkinson’s Institute, Sunnyvale, California, U.S.A.
Chris van der Linden St. Lucas Hospital Ghent, Ghent, Belgium
Lawrence A. Vitulano Yale University School of Medicine, New Haven,
Connecticut, U.S.A.
William J. Weiner University of Maryland School of Medicine, Baltimore,
Maryland, U.S.A.
Jessica W. Yakeley Maudsley Hospital, London, England
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Contributorsxii
1
Motor and Vocal Tics
Stanley Fahn
Columbia University College of Physicians & Surgeons
and The Neurological Institute of New York
Presbyterian Hospital
New York, New York, U.S.A.
INTRODUCTION
Historically, the French used the term tic,ortique, for centuries to denote an

‘‘ unpleasant gesture’’ (1). The term was first used to describe certain trick
movements in horses in 1665 (2). Then it was used to refer to distasteful motor
acts in humans, but in 1756, the term tic douloureux was coined (cited in Ref.
2) (trigeminal neuralgia in today’s lexicon), thereby setting up the different,
confusing meanings for the word tic. It seems preferable to remain with the
original usage, namely, that tic refers to certain unwanted motor acts. The
term convulsive tic (tic convulsif), as used by Charcot in Goetz (3) and Gilles
de la Tourette (4), connotes the ‘‘ abruptness and momentariness’’ of the
abnormal movement (5).
Fahn (6) defined motor tics as consisting of patterned sequences of co-
ordinated involuntary movements. Interestingly, the classic treatise on tics
by Meige and Feindel (7) in 1907 and the earlier publication by Guinon (8) had
a similar definition of tics—systematized, involuntary, coordinated move-
ments. These definitions apply best for complex tics; for simple tics, a single
myoclonic-like contraction is the common feature, and these are not coordi-
nated movements, although they can be repetitive.
Because complex tics resemble stereotypies, the question arises as to
whether tics should be listed as a subcategory of stereotypies rather than
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1
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an independent category of hyperkinetic disorders. Meige and Feindel (9)
distinguished between tics and stereotypies by describing the former as
acts that are impelling but not impossible to resist, whereas the latter, al-
though illogical, are without an irresistible urge. Shapiro et al. (10) prefer to
reserve stereotypy for those movements seen in schizophrenia, autism, and
mental deficiency. I also prefer to list tics in its own hyperkinetic dyskinesia
category.
Although a complex tic often recurs, and thereby has features of a

stereotypy, simple tics more closely resemble myoclonic jerks. Therefore,
analogously, if complex motor tics are to be classified as a stereotypy, then
simple motor tics should be classified as part of the myoclonias. It is this
diversity of motor tics that sets their phenomenology apart from all others.
Furthermore, as will be pointed out below, tics have many other features that
aid in their diagnosis, such as their suppressibility, their accompaniment by an
underlying urge or compulsion to make the movement, their variability, their
migration from one body part to another, their abruptness, their brevity, and
the repetitiveness, rather than randomness, of the particular body part
affected by the movements.
As will be discussed below, tics as occurring in Gilles de la Tourette
syndrome [usually referred to as Tourette’s syndrome (TS)] can be somatic
motor phenomena (motor tics) or phonic phenomena (equivalently referred
to as phonic or vocal tics). These are sounds produced by moving air through
the nose, mouth, or throat. In contrast to many other movement disorders,
the movements and sounds are not constantly present (except when extreme-
ly severe), but occur out of a background of normal motor activity; hence,
there is a paroxysmal pattern to them. Motor and vocal tics can be simple or
complex. Meige and Feindel (11) emphasized motor tics as being either clonic
or tonic. The more common clonic tics are rapid and brief in duration; the
less common tonic (or dystonic) tics are contractions that are longer in
duration.
PHENOMENOLOGY OF TICS
Motor Tics
Simple Motor Tics
Motor tics can be simple or complex (Table 1). Simple motor tics are abrupt,
sudden, and usually brief movements. The most simple and brief would be an
isolated jerk resembling a myoclonic jerk in speed (i.e., lightning-like). More
often appearing as a single, isolated jerk, there would be a repetitive run of
these fast movements, making their distinction from myoclonus easier. In

addition, rather than the tic repeating in the same site, a different body part
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Fahn2
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Table 1 Phenomenological Characteristics of Tics
Motor tics
(1) Simple motor tics
(a) Clonic tics
(i) Isolated, single movement
Examples: eyeblinking, shrug, eye movement, nose flare
Differential diagnosis: myoclonus, chorea
(ii) Run of simple movements
Examples: repetitive eyeblinking, arm-jerking
Differential diagnosis: blepharospasm
(b) Dystonic tics
(i) Isolated, single movements
Examples: prolonged oculogyric deviation, head deviation, mouth-opening
Differential diagnosis: focal and segmental torsion dystonia, oculogyric
crisis
(2) Complex motor tics
(a) Clonic tics
(i) Nonpurposeful, appearing purposeful, acts
Examples: tossing head, touching body, rubbing, spitting
Differential diagnosis: stereotypies, mannerisms, akathitic movements,
hyperekplexia
(ii) Acts not appearing purposeful
Examples: head-shaking, trunk-bending, series of different facial twitchings
Differential diagnosis: stereotypies, mannerisms, akathitic movements,

hyperekplexia, and other exaggerated startle syndromes
(b) Dystonic tics
Examples: bruxism with sustained head tilt
Differential diagnosis: segmental or generalized torsion dystonia
Vocal tics
(1) Simple vocal tics
Examples: throat-clearing, sniffing, grunting, barking, yelping, squeaking, clicking
Differential diagnosis: moaning with akathisia, parkinsonism; squeaking and
grunting with oromandibular dystonia and Huntington’s disease; humming with
blepharospasm and Meige syndrome
(2) Complex vocal tics
Examples: whistling, belching, coprolalia, echolalia, palilalia
Differential diagnosis: palilalia with stuttering; echolalia with exaggerated startle
syndromes; coprolalia with encephalitis; spitting with Huntington’s disease and
neuroacanthocytosis
Motor and Vocal Tics 3
may be involved with the next tic. This variation in location also helps dis-
tinguish simple tics from myoclonic jerks.
Examples of a simple motor tic that appears as an isolated movement
are an eyeblink, shrug, head jerk, dart of the eyes, twitch of the nose or flare of
the nostril, mouth-opening, and tongue protrusion. A run of any of these
would result in, for example, several eyeblinks in a row, a series of arm jerks,
or a run of facial twitches.
The single, isolated simple motor tic could also be difficult to distinguish
from choreic jerks, which are slightly slower than myoclonus. Choreic
movements do not tend to repeat immediately in the same site, but appear
randomly in different muscles. Tics may repeat in the same site before ap-
pearing elsewhere. A run of the tic movements would be particularly helpful
to distinguish simple motor tics from chorea.
Another hyperkinetic disorder that needs to be differentiated from tics is

dystonia, particularly from dystonic tics and from eye-blinking tics. Repet-
itive blinking is seen in mild idiopathic blepharospasm, which is considered a
form of focal dystonia (12). Fortunately, the blinking from tics and blepha-
rospasm can usually be differentiated from each other by the presence of
either other tics or dystonic movements at other sites. In addition, tics almost
always begin in childhood, whereas blepharospasm is predominantly a
disorder of the older adult population (13).
Dystonic tics are more difficult to distinguish from torsion dystonia. As
emphasized by Meige and Feindel (11), tics not only may be rapid jerks (clonic
tics) but may also present as sustained contractions. Sustained contraction is a
characteristic feature of dystonic movements. Meige and Feindel called tics
with sustained contractions tonic tics. The concept of dystonia had not yet
been defined in those days, but today, these tics of sustained contractions are
more commonly called dystonic tics. Examples of dystonic tics are sustained
tilt of the head, sustained elevation of the shoulder, sustained abduction of the
shoulder, sustained flexion of the trunk, and sustained opening of the mouth.
A major differential feature is that torsion dystonia is a continual hyperkine-
sia, usually twisting in pattern, that can result in sustained, continuous
abnormal postures. Dystonic tics, in contrast, are abrupt bursts of movement
that are sustained in a posture, but usually for a relatively short duration.
They do not tend to be continuous. The presence of the more typical clonic tics
in other body regions indicates that the sustained contractions are probably
dystonic tics rather than torsion dystonia.
Although dystonic tics are less common than clonic tics, Jankovic and
Stone (14) reported that they occurred in 57% of 156 patients with TS, 100%
of whom had clonic tics. The most common dystonic tics in this population
were oculogyric deviations (43 patients), sustained closure of eyelids (23
patients), and neck-posturing (11 patients).
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Fahn4
Perhaps the most helpful feature to differentiate simple tics from myoc-
lonus, chorea, and dystonia is the fact that complex motor and vocal tics
may also be present in the patient with simple tics, thereby allowing one to
establish the diagnosis by ‘‘ the company it keeps.’’ However, occasional
patients with dystonia may also have tics (15,16), and this association can
make it especially difficult to discern simple dystonic tics from dystonia in
some patients presenting with known tics and in others presenting with
known dystonia.
Another important motor feature that helps differentiate tics from
other dyskinesias is ocular deviation, if present. Ocular movements such as
a jerk of the eyes (clonic motor tic) or a more sustained eye deviation (dystonic
motor tic) may occur in patients with tics (18). When these ocular movements
are present, they often suggest that the correct diagnosis is tics because few
other dyskinesias involve ocular movements. The exceptions are: (1) opso-
clonus (‘‘ dancing eyes’’ ), which is a form of myoclonus; (2) ocular myoclonus
(rhythmic vertical oscillations at a rate of approximately 2 Hz), which often
accompanies palatal myoclonus and is considered a rhythmical segmental
myoclonus; and (3) oculogyric spasms (a sustained deviation of the eyes),
most often associated with neuroleptics or as a consequence of encephalitis
lethargica.
Complex Motor Tics
Complex tics are abrupt, distinct, coordinated patterns of sequential move-
ments. They may appear purposeful, as if performing a voluntary motor act,
but they serve no purpose (save the relief of an urge or unpleasant sensation;
see below). Examples of complex tics that appear purposeful include such acts
as tossing the head as if to move hair off the face, touching the nose, touching
other people, smelling objects, spitting, neck-cracking, rubbing, jumping, and
copropraxia (obscene gestures). Examples of complex tics that appear non-
purposeful include head-shaking associated with shrugging, repetitive kick-

ing, sequential display of a variety of facial movements, trunk-bending, and
echopraxia (mimicking movements performed by others). A run of simple
motor tics could be considered at the borderline between simple and complex
motor acts.
Other movement disorders that present with clonic-like complex move-
ments but are commonly considered distinct from tics are: (1) the repetitive
complex movements, known as stereotypies, of patients with hyperactivity,
mental retardation, or psychosis; (2) hyperekplexia, which is an excessive
startle syndrome; (3) akathitic movements (initiated to overcome a feeling of
inner restlessness); (4) the rituals of the obsessive–compulsive; and (5) the
‘‘ hand-caressing’’ movements in Rett’s syndrome (a syndrome in young girls
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Motor and Vocal Tics 5
with autism, dementia, and motor difficulties). All these other complex move-
ments can sometimes be difficult to distinguish from complex motor tics, and
the correct diagnosis of tics is usually made by exclusion of these or by finding
an association with simple motor tics or with vocal tics.
Dystonic tics can also manifest themselves as complex acts. Examples
would include a combination of bruxism (sustained jaw-clenching with teeth-
gnashing) and sustained head-tilting, sustained trunk-bending with arm or leg
deviation, and sustained facial distortion plus sustained trunk-twisting.
Complex dystonic tics are rather uncommon, but an example can be seen
on the videotape publication of Jankovic and Fahn (17). Dystonic complex
tics could be mistaken for segmental or generalized torsion dystonia, or
possibly paroxysmal dystonic choreoathetosis.
Vocal Tics
Simple Vocal Tics
Vocal tics, also referred to as phonic tics, are sounds produced by moving
air through the nose, mouth, or throat. These sounds range from simple

throat-clearing sounds and sniffing to grunts to verbalizations of syllables
and words. Like somatic motor tics, vocal tics can also be divided into sim-
ple and complex tics. Single sounds, such as throat-clearing, barking, grunt-
ing, yelping, squeaking, snorting, clicking, and sniffing, represent simple
vocal tics.
Complex Vocal Tics
When vocal tics are more complicated, such as whistling, panting, belching, or
hiccupping, or include words, they can be considered complex vocal tics.
Verbalizations (the expression of words) can be complete words, but more
commonly are partial words or unintelligible words. Utterances of inappro-
priate, undesired statements or obscenities (more often than profanities) are
known as coprolalia. Echolalia (repeating the words of others) and palilalia
(repeating one’s own words) are fairly common complex vocal tics.
Involuntary and voluntary phonations occur in only a few other
neurological disorders. These include moaning in akathisia, severe parkin-
sonism, and progressive supranuclear palsy, and from levodopa toxicity; brief
sounds in oromandibular dystonia, Huntington’s disease, neuroacanthocy-
tosis, and tardive dyskinesia; and the sniffing and spitting occasionally
encountered in Huntington’s disease and neuroacanthocytosis. The humming
and coughing encountered in some patients with blepharospasm or Meige
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Fahn6
syndrome should be considered voluntary because they are used as tricks to
suppress the dystonic movements.
Tics Status or Status Tics
Rarely, motor and vocal tics can be quite prolonged, usually as a series of
clonic tics or dystonic tics that are continual, lasting tens of minutes. These
episodes appear to have received little comment in the literature on tics. They
are not continuous tics because there may be short periods of normal behavior

between the episodes of ticking, rather than minutes of uninterrupted tics. The
tics can be simple or complex, but the patient is unable to suppress them
during these attacks. It is reasonable to consider them severe attacks of tics,
with prolongation of continual tics. In analogy with status epilepticus, I refer
to them as tics status or status tics.
Blocking Tics
Another type of tic phenomenology that is rarely, if ever, discussed in the
literature is the feature described here as blocking tics. This is a motor phe-
nomenon seen in some patients with tics in which there is a brief interfer-
ence of social discourse and contact. There is no loss of consciousness and,
although the patient does not speak during these episodes, the patient is fully
aware of what has been spoken. These occurrences have the abruptness and
duration of dystonic tics or a series of clonic tics, but they do not always occur
during an episode of an obvious motor tic. I present them here as appearing in
two situations: as an accompanying feature of some prolonged tics, including
tics status, and as a specific tic phenomenon in the absence of accompanying
obvious motor or vocal tics.
For the former, an example would be a burst of tics that is severe enough
to interrupt ongoing motor acts, including speech (Refs. 19 and 20; published
videotape with Ref. 17), so that the patient is not able to perform other
activities. We may wish to consider these episodes ‘‘ intrusions’’ because the
interruption of activities is due to a positive motor phenomenon (i.e., severe,
somewhat prolonged, motor tics).
As for the latter (i.e., inhibition of other ongoing motor activities
without obvious ‘‘ active’’ tics), we can consider these ‘‘ blocking’’ tics. These
events should be differentiated from seizures or other paroxysmal episodes of
loss of awareness. There is never loss of awareness with blocking tics.
Individuals with intrusions and blocking recognize that they have these
interruptions of normal activity, and are fully aware of the environment
around them, even if they are unable to speak at that time.

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Motor and Vocal Tics 7
OTHER FEATURES OF TICS
Paroxysmal Nature
As mentioned in the introduction, the word convulsif applied to tics represents
the abruptness and momentariness of the motor or vocal tic, out of a
background of normal motor behavior. This paroxysmal nature of tics is
characteristic, and only in severe states of tics, such as prolonged tics or tics
status, are the tics continual. This paroxysmal feature is a most helpful clinical
feature to distinguish tics from most other hyperkinetic movement disorders
(Table 2).
Other paroxysmal dyskinesias usually present no difficulty in diagnosis.
Paroxysmal kinesigenic choreoathetosis (or dystonia) is triggered by sudden
movement or startle, lasts seconds to a few minutes, and is suppressed with
anticonvulsant therapy. Paroxysmal dystonic choreoathetosis is induced by
stress, prolonged exercise, alcohol, or caffeine, and lasts minutes to hours, too
long to be confused with brief bursts of tics. Paroxysmal ataxia and parox-
ysmal tremor have the typical features of ataxia and tremor, respectively,
which are easily distinguished from tics. Stereotypies, which can occur as
bursts or can be more continual, could give the most difficulty in being
discriminated from tics. As mentioned in the introduction, we are listing
stereotypies as nonimpelling acts that would not be irresistible from being
suppressed, and stating that these repetitive complex behaviors are associated
with schizophrenia, autism, and mental retardation.
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Table 2 Differential Diagnosis of Paroxysmal and
Nonparoxysmal Hyperkinesias
Paroxysmal

hyperkinesias
Continual
hyperkinesias
Tics Athetosis
PKC
a
Ballism
PDC
b
Chorea
Paroxysmal ataxia Dystonia
Paroxysmal tremor Myoclonus
Stereotypies Tardive dyskinesia
Tremors
a
Paroxysmal kinesigenic choreoathetosis.
b
Paroxysmal dystonic choreoathetosis.
Fahn8
Variability
A characteristic feature of tics is their variability in time, place, severity, and
frequency of appearance. Patients with tics usually have remissions (and also
exacerbations). This variability in time distinguishes tics from most other
hyperkinetic movement disorders except for other paroxysmal dyskinesias
(Table 2). Tics can move from one part of the body to another, which also
distinguishes tics from other hyperkinetic movement disorders. Tics can wax
and wane in severity, and have remissions and exacerbations. Severity can be
best judged by the impact that tics have on the patient’s daily functioning and
how much effort is needed to try to overcome the tics. Tics may occur from
very few per day to many times a minute, and this variability in frequency is

another feature limited to paroxysmal disorders (Table 2).
On the other hand, for a period of time, a patient’s tics will usually recur
in the same parts of the body, and multiple regions of the body can be involved
in this fashion. This pattern of repetition, until they change sites or remit
again, can last from weeks to months or years, and has been referred to as
‘‘ systematic’’ by Charcot [in Goetz (3), p. 57] and ‘‘ systematized’’ by Meige
and Feindel (7) and Guinon (8). The pattern of tics reappearing again and
again in the same manner resembles the characteristic feature of stereotypies.
One feature of variability over the day is that it is quite common for
individuals with tics to manifest more of them in the safety of their homes,
especially at night, when relaxed while watching television. This may be
because tics can be voluntarily suppressed for short periods of time (vide
infra), and the patient makes this effort when in public.
Distribution
Tics occur predominantly in the upper part of the body. Data from Shapiro et
al. (21) reveal that tics in the upper face (around the eyes and eyelids) occurred
at least once in 80% of patients with TS. Reported frequencies of involvement
of other body regions included the neck (69%), upper limbs (55%), lower
limbs (26%), and torso (24%).
Premonitory Sensation and Urge
An inner feeling of a need to make a movement or a sound is experienced by
the majority of patients with tics; executing the movement or sound relieves
this urge. This psychic feature has long been recognized as part of the tic
phenomenology (9,22,23). Wilson (22) has said that no feature of tics ‘‘ is more
prominent than its irresistibility. The strain in holding back is as great as the
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Motor and Vocal Tics 9
relief in letting go.’’ The need to make the movement is as great as the need to
scratch an itch.

More recently, the term sensory tic was composed by Shapiro et al. (24)
to refer to ‘‘ somatic sensations in joints, bones, muscles, or other parts of the
body,’’ which are relieved by the performance of a motor tic in that particular
body part. These recurrent somatic sensations are those of pressure, tickle,
warmth, cold, pain, or other dysphoric sensations in localized regions (24).
How different and specific these focal sensations are compared to the
generalized discomfort or urge often preceding a tic is not certain.
The use of the term sensory tics was accepted by Kurlan et al. (25). But
Lang (26) argues against the use of this term, and I agree with him. As stated
above, tics should apply to a motor phenomenon, not withstanding the in-
appropriate term tic douloureux. Although the term sensory tics was meant to
describe motor tics that occur in response to a localized premonitory sensa-
tion, the terminology lends itself to ambiguity, and it could be mistaken to
represent a pure sensory phenomenon without a motor component. More-
over, Shapiro et al. (24) initially thought these focal sensations were an un-
common circumstance, leading them to coin the term. However, Kurlan et al.
(25) subsequently reported that 41% of their patients experienced a localiz-
able sensation, and that 76% described any sensation or feeling preceding
the tics.
Lang (26) specifically queried 170 patients with tics and other types of
hyperkinesias. He found that 41 of 60 patients with tic disorders stated that all
their motor and vocal tics were intentionally produced to relieve some
sensation, whereas only 8 of 110 patients with other hyperkinetic disorders
thought that (these eight patients had akathisia, an inner feeling of restless-
ness; it is characteristic of akathisia that moving about eliminates this
unpleasant sensation).
Very few other movement disorders are preceded by sensory com-
plaints. Akathitic movements are a response to akathisia, which most com-
monly occurs as a complication of neuroleptic medication. The restless legs
syndrome is a disorder in which a crawling sensation occurs in the legs when

the patient is sitting or lying down at night, to be relieved by the person
walking about. Rarely, some myoclonic jerks in patients with essential myo-
clonus have an electrical-like sensation in the same body part that contains
the myoclonus.
Suppressibility
Unless the disorder is very severe, most individuals with tics can voluntarily
suppress them for varying periods of time (27). This is in contrast to other
hyperkinetic movement disorders, which can be suppressed for only very
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Fahn10
short durations, if at all (27). But when tics are purposefully suppressed, an
inner tension of discomfort builds up, which is relieved only by an increased
burst of tics. This tension is the increase of the inner urge described above. It is
a generalized or focal uncomfortable feeling that otherwise would be relieved
by executing the movement or sound. Suppressibility is one aspect to be
considered in rating the severity of tics (Table 3).
Voluntary, Involuntary, and ‘‘ Unvoluntary’’
Lang (26) discussed the terminology of tic movements and sounds as to
whether they should be labeled ‘‘ voluntary’’ or ‘‘ involuntary.’’ He pointed
out that tics are typically listed as an involuntary movement disorder. Such a
classification was probably originally based on the need to differentiate this
neurological disorder from psychiatric disorders. However, one could argue
today that psychogenic movement disorders, particularly those due to
conversion reaction (28), are not truly voluntary. Therefore, labeling tics
involuntary should be reevaluated, as Lang (26) has proposed.
With the knowledge that premonitory sensations and urges precede tics,
that executing the tic relieves these unpleasant sensations, and that the
majority of patients with tics state that the movements and sounds are
voluntary, we should redefine the traditional concept that tics are equivalent

to the classic abnormal ‘‘ involuntary’’ movements, such as tremor, myoclo-
nus, chorea, ballism, athetosis, and dystonia.
One concept is that the motor or vocal action in response to a
premonitory sensation is ‘‘ voluntary,’’ and that the motor or vocal action
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Table 3 Spectrum of Severity of Tics
Feature Mild
Y

a
Severe
Duration Acute, brief Intermediate length Tics status
Motor tics Simple Complex Copropraxia, echopraxia,
self-mutilation
Vocal tics None Poorly audible
noises
Loud noises, coprolalia
Variety of tics Few Multiple Many
Suppressible Easily With concentrated
volition
No
Interference with
life’s activities
No disruption Mildly disruptible Highly disruptible
Source: Ref. 6.
Motor and Vocal Tics 11
relieving the generalized discomfort or an urge is ‘‘ involuntary’’ (24,25). But
perhaps the term unvoluntary may be better used to describe most motor and
vocal tics, meaning that the movement or sound is a response to relieve either

an unpleasant sensation or uncomfortable urge. By definition, we should
consider unvoluntary to mean automatic without conscious effort, as implied
from studies of the Bereitschaftpotential in patients with TS (31). This
premovement EEG potential does not preface the movements of simple tics.
Furthermore, because patients have no ability to ‘‘ will’’ away the tics, which
are ‘‘ irresistible’’ in the words of Wilson (22), neither voluntary nor involun-
tary seems entirely appropriate. Hence, an intermediate word such as
unvoluntary, or its equivalent, can be used to better describe tics.
ASSOCIATED CLINICAL FEATURES IN PATIENTS WITH TICS
It has long been recognized that patients with tics, especially patients with TS,
tend to be obsessive or compulsive (Refs. 22 and 29; Ref. 3, p. 58). Discussions
of obsessive–compulsive disorder and attention deficit disorder are found
elsewhere in this volume. For now, we need to note the association of
compulsive personality in patients with tics for the purpose of aiding the
differential diagnosis of tics from other movement disorders. As mentioned
above, Meige and Feindel (9) used this feature to differentiate tics from
stereotypies. We should also ask whether the need to respond to inner or
somatic sensory feelings could be a feature of compulsive behavior. This
question is beyond the scope of this chapter.
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Table 4 Etiological Classification of Tics
(I) Idiopathic
(A) Hereditary (TS)
(B) Sporadic
(II) Symptomatic
(A) Neuroacanthocytosis
(B) Postencephalitic
(C) Head injury
(D) Carbon monoxide intoxication

(E) Vascular
(F) Drug-induced
(1) Stimulants (amphetamine, methylphenidate, pemoline, levodopa)
(2) Tardive tics (neuroleptics)
Fahn12
ETIOLOGICAL CLASSIFICATION OF TICS
Tics are usually considered pathological, whereas mannerisms and habit
spasms are physiological (6). In this chapter, stereotypies have been consid-
ered a separate entity.
Etiologically, tic disorders can be classified as: (1) idiopathic, including
hereditary (and including TS, which is considered hereditary) and (2) symp-
tomatic (Table 4). Whether transient tic disorder and chronic motor or
phonic tics should be considered entities distinct from TS can eventually be
answered once the gene for TS is discovered. For now, I will not label these
separate etiological entities, distinct from TS, although for research pur-
poses, they should be so considered (30,31) until a final status can be assigned
to these conditions. Tics due to brain insults are listed in Table 4 as symp-
tomatic tics.
By far, the most common condition causing tics is TS, the focus of this
monograph.
REFERENCES
1. Wilson SAK. Neurology 1940; Vol. II:. Baltimore: Williams and Wilkins,
1940:1629.
2. Meige H, Feindel E. Tics and Their Treatment (translated from French by SAK
Wilson). London: Appleton, 1907:26.
3. Goetz CG. Charcot, the Clinician: The Tuesday Lessons. Excerpts from Nine
Case Presentations on General Neurology Delivered at the Salpetriere Hospital
in 1887–88 by Jean-Martin Charcot, Translated with Commentary. New York:
Raven Press, 1987:56–70.
4. Gilles de la Tourette G. La maladie de tics convulsifs. Sem Med 1899; 19:153–

156.
5. Meige H, Feindel E. Tics and Their Treatment (translated from French by SAK
Wilson). London: Appleton, 1907:31.
6. Fahn S. The clinical spectrum of motor tics. Adv Neurol 1982; 35:341–344.
7. Meige H, Feindel E. Tics and Their Treatment (translated from French by SAK
Wilson). London: Appleton, 1907:45.
8. Guinon G. Sur la maladie des tics convulsifs. Rev Med 1886; 6:50–80.
9. Meige H, Feindel E. Tics and Their Treatment (translated from French by SAK
Wilson). London: Appleton, 1907:57–58.
10. Shapiro AK, Shapiro ES, Young JG, Feinberg TE. Gilles de la Tourette
Syndrome. 2nd ed. New York: Raven Press, 1988:345.
11. Meige H, Feindel E. Tics and Their Treatment (translated from French by SAK
Wilson). London: Appleton, 1907:118–124.
12. Fahn S. Blepharospasm: a form of focal dystonia. Adv Neurol 1988; 49:125–
133.
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Motor and Vocal Tics 13