Tourette's Disorder Diagnostic Criteria
The
Diagnostic and Statistical Manual of Mental Disorders , 4th
ed (DSM-IV-TR). for Tourette syndrome is as follows for making
the diagnosis:
Diagnostic
Criteria for 307.23 Tourette's Disorder;
•
both multiple motor tics and one or more vocal tics must
be present at the same time, although not necessarily concurrently;
•
the tics must occur many times a day (usually in bouts)
nearly every day or intermittently over more than 1 year,
during which time there must not have been a tic-free period
of more than 3 consecutive months;
•
the age at onset must be less than 18 years;
•
the disturbance must not be due to the direct physiological
effects of a substance (e.g. stimulants) or a general medical
condition (e.g. Huntington's disease or postviral encephalitis).
*Comments
from the editor:
DSM-IV
used to have another
qualification that was removed in this latest revision, which
I personally have concluded helpful to some and maybe not to
others depending on the circumstances.
The
item removed was:
-
The disturbance causes marked distress or significant impairment
in social, occupational, or other important areas of functioning.
For
obvious reasons, many Touretters would not want to be labled
this way and having this item removed is a blessing, especially
if in a job or other profession that having this on your resume
would not seem fair or responsible. Most people with Tourette
Syndrome can function just fine.
I know of several people who have TS in a mild form that would
not in any way altar their life much at all. Though this
does not rule out at times that Tourette's Disorder can come
in and out of our lives and cause a severe type of marked disturbance,
especially under times of sever stress, including but not limited
to, physical or emotional stress. i.e. Divorce or loss of a
loved one.
Why
the Changes in DSM-IV
James Morrison, M. D.
With
no completely new DSM in the offing until 2006 or later, in
2000 the American Psychiatric Association revised the text of
DSM-IV to include new research information that had been developed
since first publication in 1994.
This
text revision (TR) included a very few changes in the criteria,
designed mainly to correct what were perceived as errors in
the original text.
Here
is the essence of these changes:
•
Personality
Change Due to General Medical Condition is now allowed in
a patient who has dementia, such as Alzheimer’s dementia.
•
Tourette’s
Disorder and all of the Tic Disorders no longer require that
symptoms cause distress or impair functioning.
•
Several
of the Paraphilias (exhibitionism, frotteurism, pedophilia,
sexual sadism, and voyeurism) can now be diagnosed if they
are acted upon, even though they may not cause the person
whose behavior is in question distress or impaired functioning
as a result.
The
essential features of Tourette's Disorder are multiple motor
tics and one or more vocal tics (Criterion A). These may appear
simultaneously or at different periods during the illness. The
tics occur many times a day, recurrently throughout a period
of more than 1 year. During this period, there is never a tic-free
period of more than 3 consecutive months. (Criterion B). The
onset of the disorder is before age 18 years (Criterion C).
The tics are not due to the direct physiological effects of
a substance (e.g., stimulants) or a general medical condition
(e.g., Huntington's disease or postviral encephalitis) (Criterion
D).
The
anatopical location, number, frequency, complexity, and severity
of the tics change over time. The tics typically involve the
head and, frequently, other parts of the body, such as the torso
and upper and lower limbs. The vocal tics include various words
or sounds such as clicks, grunts, yelps, barks, sniffs, snorts,
and coughs. Coprolalia, a complex vocal tic involving the uttering
of obscenities, is present in a few individuals (less than 10%)
with this disorder. Complex motor tics involving touching, squatting,
deep knee bends, retracing steps, and twirling when walking
may be present.
In
approximately one-half the individuals with this disorder, the
first symptoms to appear are bouts of a single tic, most frequently
eye blinking, less frequently tics involving another part of
the face or the body. Initial symptoms can also include tongue
protrusion, squatting, sniffing, hopping, skipping, throat clearing,
stuttering, uttering sounds or words, and coprolalia. The other
cases begin with multiple symptoms.
Associated
Features and Disorders
The most common associated symptoms of Tourette's Disorder are
obsessions and compulsions. Hyperactivity, distractibility,
and impulsivity are also relatively common. Social discomfort,
shame, self-consciousness, and depressed mood frequently occur.
Social, academic, and occupational functioning may be impaired
because of rejection by others or anxiety about having tics
in social situations. In sever cases of Tourette's Disorder,
the tics may directly interfere with daily activities (e.g.,
reading or writing). Rare complications of Tourette's Disorder
include physical injury, such as blindness due to retinal detachment
(from head banging or striking oneself), orthopedic problems
(from knee bending, neck jerking, or head turning), and skin
problems (from picking). The severity of the tics may be exacerbated
by administration of central nervous system stimulants, which
may be a dose-related phenomenon. Obsessive-Compulsive disorder,
Attention-Deficit/Hyperactivity Disorder, and Learning Disorders
may be associated with Tourette's Disorder.
Specific
Culture and Gender Features
Tourette's
Disorder has been widely reported in diverse racial and ethnic
groups. The disorder is approximately 1.5-3 times more common
in males than in females.
Prevalence
Tourette's Disorder occurs in approximately 4-5 individuals
per 10,000.
The
age at onset of Tourette's Disorder may be as early as age 2
years, is usually during childhood or early adolescence, and
is by definition before age 18 years. The median age at onset
for motor tics is 7 years. The duration of the disorder is usually
lifelong, though periods of remission lasting from weeks to
years may occur. In most cases, the severity, frequency, and
variability of the symptoms diminish during adolescence and
adulthood. In other cases, the symptoms disappear entirely,
usually by early adulthood.
The
vulnerability to Tourette's Disorder and related disorders is
transmitted in an autosomal dominant pattern. "Vulnerability"
implies that the child receives the genetic or constitutional
basis for developing a Tic Disorder; the precise type or severity
of disorder may be different from one generation to another.
Not everyone who inherits the genetic vulnerability will express
symptoms of a Tic Disorder. Penetrance in female gene carriers
is about 70%; penetrance in male gene carriers is about 99%.
The range of forms in which the vulnerability may be expressed
includes full-blown Tourette's Disorder, Chronic Motor or Vocal
Tic Disorder, some forms of Obsessive-Compulsive Disorder, and,
perhaps, Attention-Deficit/Hyperactivity Disorder. In about
10% of those with Tourette's Disorder, there is no evidence
of a familial pattern. Individuals with these "nongenetic"
forms of Tourette's Disorder or another tic disorder often have
another mental disorder (e.g., Pervasive Developmental Disorder)
or a general medical condition (e.g., a seizure disorder).
Refer
to the "Differential Diagnosis" section for Tic Disorders
(p. 100 From Page 100: Tic
Disorders must be distinguished from other types of abnormal
movements that may accompany general medical conditions (e.g.,
Huntington's disease, stroke, Lesch-Nyhan syndrome, Wilson's
disease, Sydenham's chorea, multiple sclerosis, postviral encephalitis,
head injury) or may be due to the direct effects of a substance
(e.g., a neuroleptic medication). Choreiform movements are dancing,
random, irregular, nonrepetitive movements. Dystonic movements
are slower, twisting movements interspersed with prolonged states
of muscular tension. Athetoid movements are slow, irregular,
writhing movements, most frequently in the fingers and toes,
but often involving the face and neck. Myoclonic movements are
brief, shocklike muscle contractions that may affect parts of
muscles or muscle groups but not synergistically. Hemiballismic
movements are intermittent, coarse, large-amplitude, unilateral
movements of the limbs. Spasms are stereotypic, slower, and
more prolonged than tics and involve groups of muscles. Hemifacial
spasm consists of irregular, repetitive, unilateral jerks of
facial muscles. Synkinesis involves an involuntary movement
accompanying a voluntary one (e.g., movement of the corner of
the mouth when the person intends to close the eye.) This differentiation
is further facilitated by considering the presence of features
of the underlying general medical condition (e.g., characteristic
family history in Huntington's disease) or a history of medication
use.
When
tics are a direct physiological consequence of medication use,
a Medication-Induced Movement Disorder Not Otherwise Specified
would be diagnosed instead of a Tic Disorder. In some cases,
certain medications (e.g., methylphenidate) may exacerbate a
preexisting Tic Disorder, in which case no additional diagnosis
of a medication-induced disorder is necessary.
Tics
must also be distinguished from stereotyped movements seen in
Stereotypic Movement Disorder and Pervasive Developmental Disorders.
Differentiating simple tics (e.g., eye blinking) from the complex
movements characteristic of stereotyped movements is relatively
straightforward. The distinction between complex motor tics
and stereotyped movements is less clear-cut. In general, stereotyped
movements appear to be more driven and intentional, whereas
tics have a more involuntary quality and are not rhythmic. Tics
must be distinguished from compulsions (as in Obsessive-Compulsive
Disorder). Compulsions are typically quite complex and are performed
in response to an obsession or according to rules that must
be applied rigidly. In contrast to a compulsion, tics are typically
less complex and are not aimed at neutralizing the anxiety resulting
from an obsession. Some individuals manifest symptoms of both
Obsessive-Compulsive Disorder and a Tic Disorder (especially
Tourette's Disorder), so that both diagnoses may be warranted.
Certain vocal or motor tics (e.g., barking, echolalia, palilalia)
must be distinguished from disorganized or catatonic behavior
in Schizophrenia.
The
Tic Disorders can be distinguished from one another based on
duration and variety of tics and age at onset. Transient Tic
Disorder includes motor and/or vocal tics lasting for at least
4 weeks but for no longer than 12 consecutive months. Tourette's
Disorder and Chronic Motor or Vocal Tic Disorder each have a
duration of more than 12 months but are distinguished by the
requirement for Tourette's Disorder that there be multiple motor
tics and at least one vocal tic. Tic Disorder Not Otherwise
Specified would be appropriate for clinically significant presentations
lasting less than 4 weeks, for presentations with an age at
onset above age 18 years, and for the unusual case of an individual
with only one motor tic and only one vocal tic.
The
ICD-10 Classification of Mental and Behavioural Disorders (European
Description)
The
ICD-10 calls the disorder `combined vocal and multiple motor
tic disorder (de la Tourette's syndrome)' and classifies it
as one of the tic disorders. The ICD-10 diagnostic criteria
are less strict about the age of onset than the DSM-IV-TR criteria.
F95
Tic Disorders
The
predominant manifestation in these syndromes is some form of
tic. A tic is an involuntary, rapid, recurrent, non-rhythmic
motor movement (usually involving circumscribed muscle groups),
or vocal production, that is of sudden onset and serves no apparent
purpose. Tics tend to be experienced as irresistible but they
can usually be suppressed for varying periods of time. Both
motor and vocal tics may be classified as either simple or complex,
although the boundaries are not well defined. Common simple
motor tics include eye-blinking, neck-jerking, shoulder-shrugging,
and facial grimacing. Common simple vocal tics include throat-clearing,
barking, sniffing, and hissing. Common complex tics include
hitting one's self, jumping, and hopping. Common complex vocal
tics include the repetition of particular words, and sometimes
the use of socially unacceptable (often obscene) words (coprolalia),
and the repetition of one's own sounds or words (palilalia).
There
is immense variation in the severity of tics. At the one extreme
the phenomenon is near-normal, with perhaps 1 in 5 to 1 in 10
children showing transient tics at some time. At the other extreme,
Tourette's syndrome is an uncommon, chronic, incapacitating
disorder. There is uncertainty about whether these extremes
represent different conditions or are opposite ends of the same
continuum; many authorities regard the latter as more likely.
Tic disorders are substantially more frequent in boys than in
girls and a family history of tics is common.
Diagnostic
Guidelines
The
major features distinguishing tics from other motor disorders
are the sudden, rapid, transient, and circumscribed nature of
the movements, together with the lack of evidence of underlying
neurological disorder; their repetitiveness; (usually) their
disappearance during sleep; and the ease with which they may
be voluntarily reproduced or suppressed. The lack of rhythmicity
differentiates tics from the stereotyped repetitive movements
seen in some cases of autism or of mental retardation. Manneristic
motor activities seen in the same disorders tend to comprise
more complex and variable movements than those usually seen
with tics. Obsessive compulsive activities sometimes resemble
complex tics but differ in that their form tends to be defined
by their purpose (such as touching some object or turning a
number of times) rather than by the muscle groups involved;
however, the differentiation is sometimes difficult.
Tics
often occur as an isolated phenomenon but not infrequently they
are associated with a wide variety of emotional disturbances,
especially, perhaps, obsessional and hypochondriacal phenomena.
However, specific developmental delays are also associated with
tics.
There
is no clear dividing line between tic disorder with some associated
emotional disturbance and an emotional disorder with some associated
tics. However, the diagnosis should represent the major type
of abnormality.
F95.2
Combined vocal and multiple motor tic disorder [de la Tourette's
syndrome]
A
form of tic disorder in which there are, or have been, multiple
motor tics and one or more vocal tics, although these need not
have occurred concurrently. Onset is almost always in childhood
or adolescence. A history of motor tics before development of
vocal tics is common; the symptoms frequently worsen during adolescence,
and it is common for the disorder to persist into adult life.
The
vocal tics are often multiple with explosive repetitive vocalizations,
throat-clearing, and grunting, and there may be the use of obscene
words or phrases. Sometimes there is associated gestural echopraxia,
which also may be of an obscene nature (copropraxia). As with
motor tics, the vocal tics may be voluntarily suppressed for
short periods, be exacerbated by stress, and disappear during
sleep.
Tics
are often hardly observable at the first appointment, and information
obtained by interview is decisive for making the diagnosis.
A full medical and psychosocial history should be obtained.
There is often a family history of tics, attention disorder
or obsessive-compulsive disorder. There is usually no focal
neurological finding on examination. Investigations to rule
out other possible causes of the tic may need to be performed,
as appropriate.
Significant
loss of self-esteem and the development of associated social
problems commonly complicate Tourette syndrome, especially if
it has gone undiagnosed. School or employment are often issues
of major importance, depending on the degree of abnormal behavior.
Stress on the family is also often a problem.
Most
conditions that may be confused with Tourette syndrome are on
the same spectrum (e.g. transient tic disorder, chronic tic
disorder), and differ from Tourette syndrome with respect to
either the duration of tics or the absence of both motor and
vocal tics.
Conditions
in which tics and Tourette-like behaviors may occur include:
tardive dyskinesia; autism; mental retardation; wilson's diseas;
Sydenham's chorea; Huntington's diseas.
The
aims of treatment are:
to
identify what symptoms, if any, require therapy; the diagnosis
alone is not sufficient to warrant pharmacological treatment;
to
exhaust every effort to discern the problematic features for
the individual patient and to address these features appropriately.
No
particular dietary modifications are recommended.
Stress
and anxiety clearly contribute to exacerbations of tics, and
patients should be encouraged to develop strategies to avoid
their stressors.
Pharmacological
treatment
Accurate
identification of the most troublesome symptoms guides the therapeutic
interventions. Useful approaches for mild tics include:
alpha-2
adrenergic agonists (e.g. clonidine**, or guanfacine** (US));
mild blockers of dopamine-2-receptors.
Moderate
to severe tics are treated by means of antipsychotic agents that
are potent blockers of dopamine-2 receptors (e.g. pimozide, or
risperidone**).
**Off-label
indication standard dosages
The
dosage of all drugs must be individually titrated in children;
in all patients, it is advisable to start at a low dose.
Contraindications
to the use of alpha-2 adrenergic agonists include:
occlusive
peripheral vascular disease (including Raynaud's syndrome);
a history of depression.
Contraindications
to the use of antipsychotic agents include:
previous
sensitivity to neuroleptics (e.g. neuroleptic malignant syndrome);
a history of tardive dyskinesia.
Pimozide
may prolong QT interval on electrocardiography, and it is
not recommended in patients with arrhythmogenic conditions.
The
risk or presence of side effects must always be weighed against
the severity of the symptoms.
The
main side effects of alpha-2 adrenergic agonists include:
lethargy;
somnolence;
headache;
dizziness.
The
main side effects of the antipsychotic agents include:
lethargy;
dysphoria;
dystonic reactions;
akathisia; tardive
dyskinesia - although the risk is small in children, this possible
side effect must be discussed with the patient and the family.
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Other
treatment options
Relaxation
therapies have been tried, but no consistent responses have
been reported. For persisting and circumscript tics, habit reversal
and exposure response prevention techniques seem to offer treatment
benefits.
Follow-up
and management
Patients
and families should be well informed about the condition, and
the education of peers and of school or employment personnel
is very important. If
pharmacological intervention is undertaken, patients should
be monitored for side effects and treatment responses. Periodic
attempts to taper medications should be made.
Etiology
Possible
etiologies include: a
genetic component, as indicated by the high frequency of a positive
family history in patients with Tourette syndrome, although
no marker has been identified;
abnormalities of brain dopamine and serotonin neurochemistry,
as suggested by the empiric response to pharmacological manipulation
of these neurotransmitter systems;
head trauma - very rarely, cases of Tourette syndrome have been
described as developing after head trauma.
Epidemiology
The
worldwide prevalence is estimated to be between 0.1 and 1.0
per 1000 of the population. Expression is influenced by sex,
with males more likely to exhibit tics and females more prone
to obsessive-compulsive behavior.
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Diagnostic
and Statistical Manual of Mental Disorders , 4th-ed (DSM-IV-TR).
Washington DC: American Psychiatric Association 2000; 111-114.
The
ICD-10 Classification of Mental and Behavioural Disorders:
clinical descriptions and diagnostic guidelines (ICD-10). Geneva:
World
Health Organization 1992; 284-286.
editor@psychiatrymatters.md
(2003) psychiatrymatters.md,
(June 7, 2003)
Neurobiological
issues in Tourette syndrome Singer HS. Brain Dev 1994;
16: 353-364.
Pharmacologic
treatment of tic disorders Scahill L, Chappell PB,
King RA, Leckman JF Child Adolesc Clin North Am 2000; 9: 99-118.
Complete
DSM-IV Criteria for Mental Disorders, The Clinicians
Guide to Diagnosis, James Morrison, M. D.
http://www.geocities.com/morrison94/index.htm
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