Tic Disorders

Michael H. Bloch

James F. Leckman, M.D

Tic disorders are transient or chronic conditions associated with difficulties in self-esteem, family life, social acceptance or school or job performance that are directly related to the presence of motor and/or phonic tics. Tic disorders have been noted since antiquity. The first identified case of TS in historical literature was recounted in Malleus Maleficarium (The Witches’ Hammer), a 1482 treatise on recognizing and curing demonic possession. Sprenger and Kraemer, the authors of Malleus Maleficarium, provide a detailed description of a young priest with motor and vocal tics, who is saved from a fiery death at the stake only by successful treatment with exorcism (1). In later French historical archives, there is a chilling account of one Prince de Conde, a seventeenth century French nobleman in the court of Louis XIV, who resorted to stuffing objects in his mouth to prevent an involuntary bark when in the presence of his royal highness (2).

Although reported since antiquity, recognition of TS as a distinct neuropsychiatric syndrome, and systematic study of individuals with tic disorders, began only with the reports of French neurologists Itard (1825) and Gilles de la Tourette (1885) in the nineteenth century. Gilles de la Tourette, in his classic study of 1885, described nine cases characterized by motor “incoordinations” or tics, “inarticulate shouts accompanied by articulated words with echolalia and coprolalia (3).” In addition to identifying the cardinal features of severe tic disorders, his report noted an association between tic disorders and obsessive-compulsive symptoms, as well as the hereditary nature of the syndrome in some families.

Since the case series presented by Gilles de la Tourette, we have learned that TS typically has a much more benign course than initially suggested in his account. We have also developed some effective pharmacological, psychological, and now, even surgical, treatments for individuals with TS. With increasing longitudinal assessment of children with tic disorders we have learned that many present or develop a broad array of behavioral difficulties, including disinhibited speech or conduct, impulsivity, distractibility, motoric hyperactivity, and obsessive compulsive symptoms (4). In this chapter, a presentation of the phenomenology and classification of tic disorders precedes a review of the epidemiology, clinical course, neurobiological substrates, assessment and management of tic disorders and their associated comorbidities.

Definition

Tics are sudden, repetitive movements, gestures, or phonic productions that typically mimic some aspect of normal behavior. Usually of brief duration, individual tics rarely last more than a second. Many tics tend to occur in bouts, with brief inter-tic intervals of less than one second (5). Individual tics can occur singly or together in an orchestrated pattern. They vary in their intensity or forcefulness. Motor tics, which can be viewed as disinhibited fragments of normal movement, can vary from simple, abrupt movements such as eye blinking, nose twitching, head or arm jerks, or shoulder shrugs to more complex movements that appear to have a purpose, such as facial or hand gestures or sustained looks. These two phenotypic extremes of motor tics are classified as simple and complex motor tics respectively. Similarly, phonic tics can be classified into simple and complex categories. Simple vocal tics are sudden, meaningless sounds such as throat clearing, coughing, sniffing, spitting, or grunting. Complex phonic tics are more protracted, meaningful utterances, which vary from prolonged throat clearing to syllables, words or phrases, to even more complex behaviors such as repeating one’s own words (palalalia) or those of others (echolalia) and, in rare cases, the utterance of obscenities (coprolalia) (6). Clinicians typically characterize tics by their anatomical location, number, frequency, duration, forcefulness and complexity as outlined above. Each of these elements has been incorporated into clinician rating scales that have proven to be useful in monitoring tic severity (7).

Many individuals with tics, especially those above the age of ten, are aware of premonitory urges that may either be experienced as a focal perception in a particular body region where the tic is about to occur (like an itch or a tickling sensation) or as a mental awareness (8,9). A majority of patients also report a fleeting sense of relief after a bout of tics has occurred, and most individuals are able to suppress their tics for short intervals of time (9,10).

Diagnostic Classification

The currently accepted diagnostic criteria for TS as defined in the Diagnostic and Statistical Manual of Mental Disorders Text-Revision (DSM-IV-TR) include: 1) presence of multiple motor tics, 2) presence of one or more vocal tics, 3) onset before age 18, 4) tics that may appear many times a day, either everyday or intermittently, 5) presence of tics for a period longer than 1 year, 6) change in anatomic location and character of tics over time and 7) occurrence of tics not attributable to CNS disease (Huntington disease or postviral encephalopathies) or psychoactive medication or substance usage (Table 5.6.1).

Tics that appear in childhood are often ephemeral. When a patient exhibits motor and/or vocal tics for less than a year, a diagnosis of a transient tic disorder is made, regardless of tic frequency or severity. If either motor or phonic tics (but not both) are present for a year or more, then a diagnosis of chronic motor or phonic tic disorder, respectively, can be made, according to DSM-IV-TR. Chronic tics are viewed by experts
as a milder phenotypic expression of TS, while transient tic disorders are generally viewed as a separate entity (4).

TABLE 5.6.1 DSM-IV-TR TIC DISORDER CLASSIFICATION

Diagnostic criteria for 307.23 TS

Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently. (A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization.)
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 onset is before age 18 years.
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).

Diagnostic criteria for 307.22 Chronic Motor or Vocal Tic Disorder

Single or multiple motor or vocal tics (sudden, rapid, recurrent, nonrhythmic, stereotyped motor movements or vocalizations), but not both, have been present at some time during the illness.
The tics occur many times a day 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 onset is before age 18 years.
The disturbance is not due to the direct physiological effects of a substance (stimulants) or a general medical condition (Huntington’s disease or postviral encephalitis).
Criteria have never been met for Tourette’s Disorder.

Diagnostic criteria for 307.21 Transient Tic Disorder

Single or multiple motor and/or vocal tics (sudden, rapid, recurrent, nonrhythmic, stereotyped motor movements or vocalizations).
The tics occur many times a day, nearly every day for at least 4 weeks, but for no longer than 12 consecutive months.
The onset is before age 18 years.
The disturbance is not due to the direct physiological effects of a substance (stimulants) or a general medical condition (Huntington’s disease or postviral encephalitis).
Criteria have never been met for Tourette’s Disorder or Chronic Motor or Vocal Tic Disorder.

Diagnostic criteria for 307.20 Tic Disorder Not Otherwise Specified
This category is for disorders characterized by tics that do not meet criteria for a specific Tic Disorder. Examples include tics lasting less than 4 weeks or tics with an onset after age 18 years.

(From the American Psychiatric Association: Diagnostic and Statistical Manual, Text Revision (DSM-IV-TR, Washington DC, 2000.)

Clinical Course of TS

The onset of TS is usually characterized by the appearance of simple, transient motor tics that affect the face (typically eye blinking) around the age of 5–7 (11). Over time these simple motor tics generally progress in a rostrocaudal direction affecting other areas of the face, followed by the head, neck, arms and last and less frequently, the lower extremities (12

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