Definitions of Tic Disorders
by Ruth Dowling Bruun, M.D., Donald J. Cohen, M.D., James F. Leckman, M.D.
Tics are involuntary, rapid, repetitive and stereotyped movements of
individual muscle groups. They are more easily recognized than precisely
defined. Tic disorders are generally categorized according to age of onset,
duration of symptoms, severity of symptoms and the presence of vocal and/or
motor tics.
Transient tic disorders often begin during the early school years and
can occur in up to 18% 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 - palm licking, poking and/or pinching the genitals are examples.
Transient tics last only a few weeks or months and are usually not associated
with specific behavioral or school problems. They are especially noticeable
during times of heightened excitement or fatigue. As with all tic syndromes,
boys are three to four times more often affected than girls. While transient
tics by definition do not persist for more than a year, it is not uncommon for a
child to have recurrent episodes of transient tics over the course of several
years.
Chronic tic disorders are differentiated from transient tic disorders
not only by their duration over many years, but by their relatively unchanging
character. While transient tics come and go (sniffing may be replaced by
forehead furrowing and the furrowing may become finger snapping), chronic tics -
such as facial contortions or blinking - may persist unchanged for years.
Chronic multiple tics suggest that an individual has several chronic
motor tics (or, in rare cases, several chronic vocal tics). Often it is not an
easy task to draw distinctions between transient tics, chronic tics, and chronic
multiple tics.
Tourette Syndrome (Tourette Syndrome), 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 current diagnostic criteria, as
defined by the Diagnostic and Statistical Manual of Mental Disorders IV
are as follows:
- Both multiple motor and one or more vocal tics have been present at some
time during the illness, although not necessarily concurrently.
- The tics occur many times a day (usually in bouts) nearly every day or
intermittently throughout a period of more than 1 year, and during this
period there was never a tic-free period of more than 3 consecutive months.
- The disturbance causes marked distress or significant impairment in
social, occupational, or other important areas of functioning.
- The onset is before age 18.
- The disturbance is 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).
While the criteria appear basically valid, they are not absolute. First,
there have been rare cases of Tourette Syndrome which have emerged later than age 18. Second,
the concept of "involuntary" may be hard to define operationally, since many
patients experience their tics as having a volitional component - either a
capitulation to an internal sensory urge for motor discharge, or a more
generalized psychological tension and anxiety, or both. Finally, the diagnostic
criteria do not adequately portray the full range of behavioral difficulties
that are commonly observed in patients with Tourette Syndrome, such as attention problems,
compulsions, and obsessions.
Today, the full-blown case of Tourette Syndrome is unlikely to be confused with any other
disorder. In the past, however, Tourette Syndrome was frequently misdiagnosed or undiagnosed.
The differentiation of Tourette Syndrome from other tic syndromes may be no more than
semantic, especially since recent genetic evidence links Tourette Syndrome with multiple and
transient tics of childhood and can only be defined in retrospect.
At times it may be difficult to distinguish children with extreme
attention
deficit hyperactivity disorder (ADHD) from those with Tourette Syndrome. On close examination,
many ADHD children have a few phonic or motor tics, grimace, or produce noises
similar to those with Tourette Syndrome. Since at least half of patients with
Tourette Syndrome also have had
attention deficits and hyperactivity as children, a physician may well be
confused. However, the treating doctor should be aware of the potential
complications of
treating a possible case of Tourette Syndrome with stimulant medication.
On rare occasions, the differentiation between Tourette Syndrome and a seizure disorder may
be difficult. The symptoms of Tourette Syndrome sometimes occur in a rather sharply separated
paroxysmal manner and may resemble automatisms. Patients with Tourette Syndrome, however,
retain a clear consciousness during such paroxysms. If the diagnosis is in
doubt, an EEG may be useful.
Tourette Syndrome has been seen 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 Tourette Syndrome symptoms. Whether an
autistic or retarded individual requires the additional diagnosis of Tourette
Syndrome may
remain an open question until testing (biological or otherwise) is available for
definitive diagnosis of Tourette Syndrome.
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 test.
Since more physicians are now aware of Tourette Syndrome, there is a growing danger of
over-diagnosis or over-treatment. 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 or
other approaches.
The Authors
Tourette Syndrome Association
Ruth Dowling Bruun, M.D. is Clinical Associate Professor of Pyschiatry, Cornell University Medical School, New York, N.Y.
Donald J. Cohen, M.D. is Director and Irving B. Harris Professor of Child Psychiatry, Pediatrics and Psychology, Yale Child Study Center, New Haven, CT.
James F. Leckman, M.D. is Neison Harris Associate Professor of Child Psychiatry and Pediatrics, Yale Child Study Center, New Haven, CT.
More information can be obtained from:
Tourette Syndrome Association
42-40 Bell Boulevard
Bayside, New York 11361
718/224-2999
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Reviewed: 09/2006
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