Second edition

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whispering. Some patients may find improvement with
singing, or even with shouting.
Course
After a period of progression of variable duration, the dysphonia
generally remains chronic.
Etiology
Spasmodic dysphonia is considered one of the primary
dystonias; the etiology is not known.
Differential diagnosis
Dysphonia may result from lesions in the central or peripheral
nervous system or in the larynx itself. Dysphonia has
been noted with a putaminal lesion (Lee et al. 1996), lesions
of the nucleus ambiguus or the vagus or recurrent laryngeal
nerves, and with various intrinsic laryngeal lesions.
Dysphonia has also been reported as a side-effect of valproic
acid (Oh et al. 2004) and gabapentin (Reeves et al.
1996), and has also occurred as part of tardive dyskinesia
(Lieberman and Reife 1989).
Treatment
Botulinum injection of the laryngeal musculature generally
provides relief. In treatment-resistant cases, consideration
may be given to lesioning the recurrent laryngeal nerve.
8.25 TOURETTE’S SYNDROME
Tourette’s syndrome, first describe by the French neurologist
Georges Gilles de la Tourette in 1885 (de la Tourette
1885), is the classic cause of chronic tics; this is a not
uncommon disorder, with a lifetime prevalence of about
0.05 percent, and is about three times more common in
males than females. Synonyms for this disorder include
maladie des tics and tic convulsiv.
Clinical features
The onset of Tourette’s syndrome is typically with a simple
tic, more often motor than verbal, and more often on the
head or face than elsewhere. Although the age of onset
may be anywhere from infancy to the early adult years
(Marneros 1983), most patients fall ill in childhood,
around the age of 7 years.
In its fully developed form, both motor and verbal tics
are present, and these may be either simple or complex
(Cardoso et al. 1996; Lang et al. 1993; Lees et al. 1984;
8.25 Tourette’s syndrome 369
Nee et al. 1980; Regeur et al. 1986); it has also become
apparent that sensory tics, once thought to be unusual, are
also present in most patients.
Motor tics are usually the first to appear. Simple motor
tics include blinking, brow wrinkling, grimacing, and
shoulder shrugging; complex motor tics may include
touching, smelling, hopping, throwing, clapping, bending
over, squatting, or even such very complex acts as
echopraxia or copropraxia (wherein patients make obscene
gestures). Motor tics usually appear first in the face or head
and then spread in a caudal direction. In most cases, before
having a tic, patients first experience an urge to tic (Lang
1991), an urge that may at times be resisted, albeit with difficulty.
Furthermore, some patients are able to abort a
motor tic with a geste antagoniste, such as placing a hand
under the chin to prevent the emergence of a tic of the head
(Wojcieszek and Lang 1995).
Vocal tics, like motor tics, may also be simple or complex.
Simple vocal tics include snorting, hissing, coughing,
throat-clearing, grunting, and, classically, barking.
Complex vocal tics include the utterance of words, simple
phrases, or entire sentences. Echolalia or palilalia may
occur and, in a minority, classic coprolalia, or involuntary
swearing, may be seen.
Sensory tics occur in the majority of patients and appear
to exist in two forms. In one there is simply the experience
of an itch or a tingle (Chee and Sachdev 1997), and this is
seen in perhaps one-quarter of patients. In the other the
sensory tic appears more as a premonitory urge to a motor
tic (Cohen and Leckman 1992; Leckman et al. 1993), and
this has been reported in over 90 percent of cases
(Leckman et al. 1993). Remarkably, in one case a premonitory
urge to itch was experienced by a patient as residing
in another person, whom the patient then proceeded to
scratch (Karp and Hallett 1996).
Rarely, dystonic movements, especially cervical or facial
dystonias, may appear in the course of Tourette’s syndrome,
but not until 10–38 years have passed (Stone and
Jankovic 1991).
Obsessions and compulsions are common in Tourette’s
syndrome, eventually appearing in nearly one-half of all
patients (Frankel et al. 1986; Robertson et al. 1988); they
typically begin to appear about 5 years into the course.
Interestingly, compulsions experienced by patients with
Tourette’s syndrome often center on getting things ‘just
right’ (Leckman et al. 1994).
Attention deficit/hyperactivity disorder (ADHD) very
commonly accompanies Tourette’s syndrome and, in
those cases of Tourette’s syndrome in which it does occur,
the hyperactivity usually precedes the tics by a little over a
year (Cardoso et al. 1996).
Course
In most cases, symptoms gradually worsen over a matter of
a few years, peaking in severity around the age of 10 years;