In his now famous 1885 publication, George Gilles de la Tourette described the illness that now bears his name. He reported nine patients with motor and vocal tics, some of whom had echophenomena and coprolalia (vide infra).1 Up to the past few decades Tourette syndrome (TS) was viewed as a rare and disabling condition with bizarre symptoms and unknown etiology. Although our understanding of the disorder is still incomplete, it has improved considerably as a result of the body of clinical, neuroscience, imaging, and genetic data that has accumulated since. This new knowledge has led to a paradigm shift in the thinking about TS, and to more informed and effective approaches to the illness. This research and resulting evolution in our clinical approaches to patients with TS are the foci of this chapter.
Tics and the Diagnosis of TS
Tics are the primary motor abnormality and diagnostic signature of TS, and the reason for which the syndrome is sometimes referred to as a “movement disorders.” Tics are recurrent, nonrhythmic, stereotyped movements (motor tics) or sounds (phonic tics). Complex phonations involving words or phrases or the use of language are termed vocal tics. In contrast to some other involuntary movements, tics are not constant (except when severe), and occur in a background of otherwise normal motor function. Motor and phonic/vocal tics may take a variety of forms and can be divided conceptually into simple and complex (see Table 39–1). Motor tics can affect any part of the body but they typically begin in the eyelids or face, and may later involve other muscle groups with an apparent rostrocaudal migration during development with constant waxing and wanning.2
Table 39–1. Clinical Heterogeneity of TS
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Table 39–1. Clinical Heterogeneity of TS
|I. The Tic Disorder|
|A. Tic types|
|1. Simple motor tics|
|2. Simple phonic or vocal tics|
|3. Complex motor tics|
|4. Complex vocal tics|
|5. Tic variants|
|a. Dystonic tics|
|b. Sensory tics|
|B. Tic Disorder Syndromes|
|1. Tourette syndrome|
|2. Chronic tic disorder (motor or vocal)|
|3. Transient tic disorder|
|C. Tic severity|
|II. The Behavioral Disorder|
|A. Obsessive compulsive behavior/disorders (OCD)|
|B. Attention defi cit hyperactivity disorder (ADHD)|
|C. Anxiety disorders|
|D. Impulse control disorders|
|E. Conduct disorder and rage attacks|
|F. Other behavioral disturbances|
Simple motor tics are sudden, brief, isolated movements such as an eye blink, a shoulder shrug, or a head jerk. Although most simple motor tics are fast and abrupt, some comprise sustained, tonic movements (e.g., neck twisting, abdominal or buttock tightening) and are termed “dystonic tics”. They include painless oculogyric eye movements, blepharospasm, and dystonic neck and back movements (see Table 39–2).3,4
Table 39–2. Tic Phenomenology
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