A clinical study of Gilles de la Tourette syndrome in the United Kingdom.

Abstract

SUMMARY The clinical features of 53 British-born patients with Gilles de la Tourette syndrome are described. The mean age at onset of body tics was seven years and for vocalisations 11 years. Coprolalia was present in 39%, copropraxia in 21%, echolalia in 46% and echopraxia in 21%. Complicated antics and mannerisms were also common, often involving the compulsive touching of objects or self-injurious behaviour. Forty-six per cent of cases had a family history of tics in a single close relative and in two individuals a further member of the family had Gilles de la Tourette syndrome. Focal dystonia was present in four patients who had never received neuroleptics drugs and chorea was seen in two other untreated patients. In three patients acoustic startle consistently induced brief eye blink followed by a whole body jerk or jump. Rapid repetitive movements of the hands increased the frequency and severity of tics in 13 patients, but the performance of mental arithmetic under time pressure had a much more unpredictable effect. Electroencephalographic abnormalities occurred in eight (13%) but no definite CT brain scan abnormalities were detected. The incidence of left handedness did not differ from that in the general population and no evidence to suggest organic impairment was found on neuropsychological testing. This study provides no support for the notion that Gilles de la Tourette syndrome is a degenerative disorder of the central nervous system but provides some evidence for heterogeneity.Tics are abrupt variable, usually repetitive coordinated muscular jerks which are relatively easy to imitate and can be voluntarily suppressed for considerable periods of time. Their pathophysiology remains obscure but they have in the past generally been considered to be environmentally determined-as Charcot put it "a psychical disorder in a physical guise". However, recent neurophysiological studies suggest that tics are not generated through the motor pathways used for normal self-paced voluntary movement' and there has been a recent swing towards considering this phenomenon to have an underlying neurogenic cause.2 Tic disorders range in severity from the single acute transient tic, which affects up to 10% of children, to the uncommon and sometimes socially

Pathophysiology of tics and Tourette syndrome

“…Many patients are able to exert some voluntary control over their tics. Motor tics can be suppressed by an effort of will or concentration and reappear when the patient is relaxed [21,29].…”

“…Motor tics accompanied by vocal tics and speech or behavioral disorders, with an onset between ages of 5 and 15 years, and a tendency to wax and wane in severity over time, fulfill the criteria for a diagnosis of the Gilles de la Tourette syndrome (TS) [21,29,38]. Among the behavioral disorders that develop in many patients are obsessive-compulsive disorders and attention deficit hyperactivity disorders, impulsive and self-destructive behavior, sleep abnormalities and alterations in mood and sexual behavior.…”

‘An international perspective on Tourette syndrome’

“…4,5,6,7 This also applies to the prevalence of self-injurious behaviours (SIB). This is given as 14% also (compared with other studies estimating between 33% and 48%).…”

Tic-reducing effects of music in patients with Tourette’s syndrome: Self-reported and objective analysis

“…Tics can be triggered not only by emotional stimuli like anxiety [11][12][13], anger [10], aggression [10] and excitement [11,12,14] but also by boredom [11] or fatigue [11,15,16]. Stress has also been to be a factor worsening tics [11,13,[15][16][17]. The influence of stress, however, is currently a matter of debate.…”

“…Moreover, a large number of patients report to have premonitory urges (sensations) [2][3][4][5][6][7][8][9], also called sensory tics [10], that are perceived directly prior to the execution of a motor or vocal tic. Tics can be triggered not only by emotional stimuli like anxiety [11][12][13], anger [10], aggression [10] and excitement [11,12,14] but also by boredom [11] or fatigue [11,15,16]. Stress has also been to be a factor worsening tics [11,13,[15][16][17].…”