’s Disorder Essay, Research Paper
Tourette’s Disorder
Table of Contents
Tourette Syndrome And Other Tic Disorders
Definitions of Tic Disorders
Differential Diagnosis
Symptomatology
Associated Behaviors and Cognitive Difficulties
Etiology
Stimulant Medications
Epidemiology and Genetics
Non-Genetic Contributions
Clinical Assessment Of Tourette Syndrome
Treatment Of Tourette Syndrome
Monitoring
Reassurance
Pharmacological Treatment of Tourette Syndrome
Psychodynamic Psychotherapy
Family Treatment
Genetic Counseling
Academic and Occupational Interventions
Bibliography
Today the full-blown case of TS is unlikely to be confused with any other
disorder.
However, only a decade ago TS was frequently misdiagnosed as schizophrenia,
obsessive-compulsive disorder, Sydenham’s chorea, epilepsy, or nervous habits.
The
differentiation of TS from other tic syndromes may be no more than semantic,
especially
since recent genetic evidence links TS with multiple tics. Transient tics of
childhood are
best defined in retrospect. At times it may be difficult to distinguish
children with
extreme attention deficit hyperactivity disorder (ADHD) from TS. Many ADHD
children, on
close examination, have a few phonic or motor tics, grimace, or produce noises
similar to
those of TS. Since at least half of the TS patients also have attention
deficits and
hyperactivity as children, a physician may well be confused. However, the
treating doctor
should be aware of the potential dangers of treating a possible case of TS with
stimulant
medication. On rare occasions the differentiation between TS and a seizure
disorder may be
problematic. The symptoms of TS sometimes occur in a rather sharply separated
paroxysmal
manner and may resemble automatisms. TS patients, however, retain a clear
consciousness
during such paroxysms. If the diagnosis is in doubt, an EEG may be useful. We
have seen TS
in association with a number of developmental and other neurological disorders.
It is
possible that central nervous system injury from trauma or disease may cause a
child to be
vulnerable to the expression of the disorder, particularly if there is a
genetic
predisposition. Autistic and retarded children may display the entire gamut of
TS symptoms,
but whether an autistic or retarded individual requires the additional
diagnosis of TS may
remain an open question until there is a biological or other diagnostic test
specifically
for TS. In older patients, conditions such as Wilson’s disease, tardive
dyskinesia, Meige’s
syndrome, chronic amphetamine abuse, and the stereotypic movements of
schizophrenia must be
considered in the differential diagnosis. The distinction can usually be made
by taking a
good history or by blood tests. Since more physicians are now aware of TS,
there is a
growing danger of overdiagnosis or over-treatment. Prevailing diagnostic
criteria would
require that all children with suppressible multiple motor and phonic tics,
however
minimal, of at least one year, should be diagnosed as having TS. It is up to
the clinician
to consider the effect that the symptoms have on the patient’s ability to
function as well
as the severity of associated symptoms before deciding to treat with medication.
TABLE 1. RANGE OF SYMPTOMS OF TS
Motor
Simple motor tics: fast, darting, and meaningless.
Complex motor tics: slower, may appear purposeful
Vocal
Simple vocal tics: meaningless sounds and noises.
Complex vocal tics: linguistically meaningful utterances such as
words and
phrases (including coprolalia, echolalia, and palilalia).
Behavioral and Developmental
Attention deficit hyperactivity disorder, obsessions and compulsions,
emotional problems, irritability, impulsivity, aggressivity, and self-
injurious
behaviors; various learning disabilities
Symptomatology
The varied symptoms of TS can be divided into motor, vocal, and behavioral
manifestations
(Table 2). Complex motor tics can be virtually any type of movement that the
body can
produce including gyrating, hopping, clapping, tensing arm or neck muscles,
touching people
or things, and obscene gesturing. At some point in the continuum of complex
motor tics, the
term “compulsion” seems appropriate for capturing the organized, ritualistic
character of
the actions. The need to do and then redo or undo the same action a certain
number of times
(e.g., to stretch out an arm ten times
Definitions of Tic Disorders
Tics are involuntary, rapid, repetitive, and stereotyped movements of
individual muscle
groups. They are more easily recognized than precisely defined. Disorders
involving tics
generally are divided into categories according to age of onset, duration of
symptoms, and
the presence of vocal or phonic tics in addition to motor tics. Transient tic
disorders
often begin during the early school years and can occur in up to 15% of all
children.
Common tics include eye blinking, nose puckering, grimacing, and squinting.
Transient
vocalizations are less common and include various throat sounds, humming, or
other noises.
Childhood tics may be bizarre, such as licking the palm or poking and pinching
the
genitals. Transient tics last only weeks or a few months and usually are not
associated
with specific behavioral or school problems. They are especially noticeable
with heightened
excitement or fatigue. As with all tic syndromes, boys are three to four times
more often
afflicted than g! irls. While transient tics by definition do not persist for
more than a
year, it is not uncommon for a child to have series of transient tics over the
course of
several years. Chronic tic disorders are differentiated from those that are
transient not
only by their duration over many years, but by their relatively unchanging
character. While
transient tics come and go – with sniffing replaced by forehead furrowing or
finger
snapping, chronic tics – such as contorting one side of the face or blinking -
may persist
unchanged for years. Chronic multiple tics suggest that an individual has
several chronic
motor tics. It is often not an easy task to draw the lines between transient
tics, chronic
tics, and chronic multiple tics. Tourette Syndrome (TS), first described by
Gilles de la
Tourette, can be the most debilitating tic disorder, and is characterized by
multiform,
frequently changing motor and phonic tics. The prevailing diagnostic criteria
include onset
before the age of 21; recurrent, involuntary, rapid, purposeless motor
movements affecting
multiple muscle groups; one or more vocal tics; variations in the intensity of
the symptoms
over weeks to months (waxing and waning); and a duration of more than one year.
While the
criteria appear basically valid, they are not absolute. First, there have been
rare cases
of TS which have emerged later than age 21. Second, the concept of
“involuntary” may be
hard to define operationally, since some patients experience their tics as
having a
volitional component – a capitulation to an internal urge for motor discharge
accompanied
by psychological tension aefore writing, to even up, or to stand up and push a
chair into
“just the right position”) is compulsive in duality and accompanied by
considerable
internal discomfort. Complex motor tics may greatly impair school work, e.g.,
when a child
must stab at a workbook with a pencil or must go over the same letter so many
times that
the paper is worn thin. Self-destructive behaviors, such as head banging, eye
poking, and
lip biting, also may occur. Vocal tics extend over a similar spectrum of
complexity and
disruption as motor tics ( The most socially distressing complex vocal symptom
is
coprolalia, the explosive utterance of foul or “dirty” words or more elaborate
sexual and
aggressive statements. While coprolalia occurs in only a minority of TS
patients (from
5-40%, depending on the clinical series), it remains the most well known
symptom of TS. It
should be emphasized that a diagnosis of TS does not require that coprolalia is
present.
Some TS patients may have a tendency to imitate what they have just seen
(echopraxia),
heard (echolalia), or said (palilalia). For example, the patient may feel an
impulse to
imitate another’s body movements, to speak with an odd inflection, or to accent
a syllable
just the way it has been pronounced by another person. Such modeling or
repetition may lead
to the onset of new specific symptoms that will wax and wane in the same way as
other TS
symptoms.
TABLE 2. EXAMPLES OF MOTOR SYMPTOMS
Simple motor tics
Eye blinking, grimacing, nose twitching, lip pouting, shoulder shrugging, arm
jerking,
abdominal tensing, kicking, finger movements, jaw snapping, tooth clicking,
frowning,
tensing parts of the body, and rapid jerking of any part of the body.
Complex motor tics
Hopping, clapping, touching objects (or others or self), throwing, arranging,
gyrating,
bending, “dystonic” postures, biting the mouth, the lip, or the arm,
headbanging, arm
thrusting, striking out, picking scabs, writhing movements, rolling eyes
upwards or
side-to-side, making funny expressions, sticking out the tongue, kissing,
pinching,
writing over-and-over the same letter or word, pulling back on a pencil
while writing,
and tearing paper or books.
Copropraxia
“Giving the finger” and other obscene gestures.
Echopraxia
Imitating gestures or movements of other people.
TABLE 3. EXAMPLES OF VOCAL SYMPTOMS
Simple vocal tics
Coughing, spitting, screeching, barking, grunting, gurgling, clacking,
whistling, hissing,
sucking sounds, and syllable sounds such as “uh, uh,” “eee,” and “bu.”
Complex vocal tics
“Oh boy,” “you know,” “shut up,” “you’re fat,” “all right,” and “what’s that.”
or any other understandable word or phrase Rituals
Repeating a phrase until it sounds “just right” and saying something over 3
times.
Speech atypicalities
Unusual rhythms, tone, accents, loudness, and very rapid speech.
Coprolalia
Obscene, aggressive, or otherwise socially unacceptable words or phrases.
Palilalia
Repeating one’s own words or parts of words.
Echolalia
Repeating sounds, words, or parts of words of others.
The symptoms of TS can be characterized as mild, moderate, or severe by their
frequency,
their complexity, and the degree to which they cause impairment or disruption
of the
patient’s ongoingctivities and daily life. For example, extremely frequent tics
that occur
20-30 times a minute, such as blinking, nodding, or arm flexion, may be less
disruptive
than an infrequent tic that occurs several times an hour, such as loud barking,
coprolalic
utterances, or touching tics. There may be tremendous variability over short
and long
periods of time in symptomatology, frequency, and severity. Patients may be
able to inhibit
or not feel a great need to emit their symptoms while at school or work. When
they arrive
home, however, the tics may erupt with violence and remain at a distressing
level
throughout the remainder of the day. It is not unusual for patients to “lose”
their tics as
they enter the doctor’s office. Parents may plead with a child to “show the
doctor what you
do at home,” only to be told that the youngster “just doesn’t feel like doing
them” or
“can’t do them” on command. Adults will say “I only wish you could see me
outside of your
office,” and family members will heartily agree. A patient with minimal
symptoms may
display more usual severe tics when the examination is over. Thus, for example,
the doctor
often sees a nearly symptom-free patient leave the office who begins to hop,
flail, or bark
as soon as the street or even the bathroom is reached. In addition to the
moment-to-moment
or short-term changes in symptom intensity, many patients have oscillations in
severity
over the course of weeks and months. The waxing and waning of severity may be
triggered by
changes in the patient’s life; for example, around the time of holidays,
children may
develop exacerbations that take weeks to subside. Other patients report that
their symptoms
show seasonal fluctuation. However, there are no rigorous data on whether life
events,
stresses, or seasons, in fact, do influence the onset or offset of a period of
exacerbation. Once a patient enters a phase of waxing symptomatology, a process
seems to be
triggered that will run its course – usually within 1-3 months. In its most
severe forms,
patients may have uncountable motor and vocal tics during all their waking
hours with
paroxysms of full-body movements, shouting, or self-mutilation. Despite that,
many patients
with severe tics achieve adequate social adjustment in adult life, although
usually with
considerable emotional pain. The factors that appear to be of importance with
regard to
social adaptation include the seriousness of attentional problems, intelligence,
the degree
of family acceptance and support, and ego strength more than the severity of
motor and
vocal tics. In adolescence and early adulthood, TS patients frequently come to
feel that
their social isolation, vocational and academic failure, and painful and
disfiguring
symptoms are more than they can bear. At times, a small number may consider and
attempt
suicide. Conversely, some patients with the most bizarre and disruptive
symptomatology may
achieve excellent social, academic, and vocational adjustments.
Associated Behaviors and Cognitive Difficulties
As well as tics, there are a variety of behavioral and psychological
difficulties that are
experienced by many, though not all, patients with TS. Those behavioral
features have
placed TS on the border between neurology and psychiatry, and require an
understanding of
both disciplines to comprehend the complex problems faced by many TS patients.
The most
frequently reported behavioral problems are attentional deficits, obsessions,
compulsions,
impulsivity, irritability, aggressivity, immaturity, self-injurious behaviors,
and
depression. Some of the behaviors (e.g., obsessive compulsive behavior) may be
an integral
part of TS, while others may be more common in TS patients because of certain
biological
vulnerabilities (e.g., ADHD). Still others may represent responses to the
social stresses
associated with a multiple tic disorder or a combination of biological and
psychological
reactions.
Obsessions and Compulsions
Although TS may present itself purely as a disorder of multiple motor and vocal
tics, many
TS patients also have obsessive-compulsive (OC) symptoms that may be as
disruptive to their
lives as the tics – sometimes even more so. There is recent evidence that
obsessive-compulsive symptomatology may actually be another expression of the
TS gene and,
therefore, an integral part of the disorder. Whether this is true or not, it
has been well
documented that a high percentage of TS patients have OC symptoms, that those
symptoms tend
to appear somewhat later than the tics, and that they may be seriously
impairing. The
nature of OC symptoms in TS patients is quite variable. Conventionally,
obsessions are
defined as thoughts, images, or impulses that intrude on consciousness, are
involuntary and
distressful, and while perceived as silly or excessive, cannot be abolished.
Compulsions
consist of the actual behaviors carried out in response to the obsessions or in
an effort
to ward them off. Typical OC behaviors include rituals of counting, checking
things over
and over, and washing or cleaning excessively. While many TS patients do have
such
behaviors, there are other symptoms typical of TS patients that seem to