Diagnosis
Before the 1980s, only people with the most severe
and clini-cally obvious tics were diagnosed with Tourette’s disorder. The
majority of these patients were adults who pursued care and were correctly
diagnosed only when their tic symptoms were disabling and when classic symptoms
such as coprolalia were present. Adults with milder tics generally did not
pursue care and may have been stigmatized without the awareness of the cause of
their movements. Children with tics were not identified at all or were
identified as having other behavioral or psychiatric difficulties. Increasingly,
as medical professionals and the public became more knowledgeable about tic
disorders, psychiatrists began to see children at younger ages and with milder
symptoms. Today, psychiatrists sometimes become involved even when the tics
themselves are not obvious or even disabling. In today’s clinical practice, the
challenge is often not the treatment of the tics but identification of
cooccurring and often more disabling psychiat-ric, behavioral, family and
school problems.
More than half of families who finally pursue
expert con-sultation find out about tic disorders from news articles or
televi-sion. Many parents describe as their worst fear that their child’s mild
tic disorder is the beginning of a permanent neuropsychiatric disorder with a
deteriorating course. Other children are identified during evaluation for other
problems, such as ADHD. When the diagnosis of Tourette’s disorder is made as
part of an evaluation for other problems, it can be particularly difficult for
the fam-ily and the patient to cope with the additional and unexpected
diagnosis. Clearly, at the time of the evaluation, the patient and family are
often frightened and require considerable psychologi-cal support.
Some children with tics, who present directly to a
neurolo-gist or a psychiatrist for an evaluation, may have a parent who has
been diagnosed with a tic disorder. In this context, children can present early
in the course of their disorder, often before a clear diagnosis can be made.
The parents of these children were often diagnosed with tics late in their life
or experienced significant duress from their symptoms and want their child to
have a better experience.
Clinical assessment of the tic disorders begins
with identifica-tion of the specific movements and sounds. It is also important
to identify the severity of and impairment caused by the tics. A number of
structured and semistructured instruments are avail-able for the identification
of tics and the rating of tic. Knowledge of the basic clinical parameters of
tics and the course of illness dictates the evaluation. Questioning patients
and their families about the presence of simple and complex movements in muscle
groups from head to toe is a good beginning. Because vocal tics usually follow
the development of motor tics, questions about the presence of simple sounds is
next. Inquiring about the presence of complex vocal tics completes the tic
inventory. It is helpful to elu-cidate other aspects of tic severity, such as
the absolute number of tics; the frequency, forcefulness and intrusiveness of
the symp-toms; the ability of the patient successfully to suppress the tics;
and how noticeable the tics are to others. It is also important to know whether
premonitory sensory or cognitive experiences are a component of specific tics
because these intrusive experiences may disrupt functioning more than the tics
themselves. Although the waxing and waning nature of the tics and the
replacement of one tic with another do not directly affect severity, identify-ing
the characteristic course of illness is important for diagnostic confidence.
Last, it is
important to assess the impairment due to the tics themselves. Whereas tic
severity is frequently correlated with overall impairment, it is not uncommon
to identify patients in whom tic severity and impairment are not correlated.
Patients who experience more impairment than their tic symptoms ap-parently
warrant are a particular clinical challenge. A number of clinical features of
tics are associated with impairment:
·
Large, disruptive, or painful motor movements;
·
Vocalizations that call attention to the patient;
·
Premonitory sensations or cognitions that intrude
into consciousness;
·
Tics that are socially unacceptable.
Whereas tic severity and impairment are often
correlated, many patients with mild tics are most impaired by the comorbid
condi-tions ADHD, OCD and Learning Disorders. An adequate assess-ment of these
conditions is part of any comprehensive evaluation. The assessment of
tic-related obsessive–compulsive symptoms, for example, touching, tapping,
rubbing, “evening up”, repeating actions, stereotypical self-mutilation,
staring, echolalia and pali-lalia, although often omitted from the traditional
psychiatric and neurological review of symptoms, should always be part of the
routine evaluation of patients with tics, OCD, or ADHD.
Psychosocial issues can play a role in tic severity
and in over-all adaptation and impairment. Assessment of family, peer and
school support for the youngster (adequate protection) along with assessment
for the presence of opportunities to be intellec-tually, physically and
socially challenged is important. The bal-ance between protection and challenge
in children is critical for long-term development. An environment that is too
protective decreases opportunities for building skills. An environment that is too
challenging can lead to frustration, anger and maladaptive coping.
Tic assessment requires a careful evaluation of
observable tic symptoms. Interestingly, the absence of tic symptoms during an
evaluation, in spite of parent’s or patient’s report, is not uncommon and
should not necessarily lead to clinical doubt. Occasionally, an additional
clinical observer (e.g. nurse or medi-cal student) may identify tics more
readily than the psychiatrist conducting the evaluation. Other than the
observation of tics in the interview, there are no pathognomonic physical
examina-tion findings. Patients with Tourette’s disorder have been noted to
have nonfocal and nonspecific subtle neurological findings (“soft” signs). If
tic suppression the with neuroleptic
agents is con-sidered, a more structured method of documenting the complex
movements that are part of the pretreatment baseline evaluation is useful for
following the progression of the disease and for sub-sequent assessment for
neuroleptic-induced movements.
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