A personal 35 year perspective on Gilles de la Tourette syndrome: assessment, investigations, and management.

After having examined the definition, clinical phenomenology, comorbidity, psychopathology, and phenotypes in the first paper of this Series, here I discuss the assessment, including neuropsychology, and the effects of Gilles de la Tourette syndrome with studies showing that the quality of life of patients with Tourette's syndrome is reduced and that there is a substantial burden on the family. In this paper, I review my local and collaborative studies investigating causal factors (including genetic vulnerability, prenatal and perinatal difficulties, and neuro-immunological factors). I also present my studies on neuro-imaging, electro-encephalograms, and other special investigations, which are helpful in their own right or to exclude other conditions. Finally, I also review our studies on treatment including medications, transcranial magnetic stimulation, biofeedback, target-specific botulinum toxin injections, biofeedback and, in severe refractory adults, psychosurgery and deep brain stimulation. This Review summarises and highlights selected main findings from my clinic (initially The National Hospital for Neurology and Neurosurgery Queen Square and University College London, UK, and, subsequently, at St George's Hospital, London, UK), and several collaborations since 1980. As in Part 1 of this Series, I address the main controversies in the fields and the research of other groups, and I make suggestions for future research.

Predictors during childhood of future health-related quality of life in adults with Gilles de la Tourette syndrome.

BACKGROUND: Gilles de la Tourette syndrome (GTS) is a chronic neurodevelopmental disorder characterised by multiple motor and phonic tics and behavioural problems. Patients with GTS of all ages often report a poor health-related quality of life (HR-QOL). The diagnosis of GTS is usually established in childhood but little is known about factors that predict the long-term well-being of patients, especially in the presence of co-morbid behavioural problems. AIM: To investigate the childhood predictors of HR-QOL in a cohort of adult patients with GTS. METHODS: Forty-six patients with GTS aged 6-16 years underwent a baseline standardised clinical assessment of both tics and behavioural symptoms at a specialist GTS clinic. The same patients were re-assessed aged 16 years and above, with a mean follow-up period of 13 years (range 3-25 years), when they completed the Gilles de la Tourette Syndrome-Quality of Life Scale (GTS-QOL), a disease-specific measure of HR-QOL. RESULTS: Tic severity, premonitory urges and family history of GTS were identified as predictors during childhood of a poorer HR-QOL in adults with GTS by multiple linear regression analysis. Specifically, tic severity significantly predicted poor outcome across physical, psychological and cognitive domains of the GTS-QOL, reflecting widespread effects on HR-QOL. CONCLUSION: Young patients with severe tics associated with characteristic premonitory urges and a family history of tic disorders appear to be at higher risk for poorer HR-QOL as adults. Further prospective research into HR-QOL in GTS is required in order to inform long-term strategic resource allocation.

Psychological morbidity and caregiver burden in parents of children with Tourette's disorder and psychiatric comorbidity.

OBJECTIVE: To investigate the mental health and caregiver burden in parents of children with Tourette's disorder (TD) compared with parents of children with asthma. METHOD: A cross-sectional cohort survey was conducted at TD and pediatric asthma hospital outpatient clinics over a 6-month period. Main outcome measures were parent mental health (General Health Questionnaire [GHQ]-28) and caregiver burden (Child and Adolescent Impact Assessment) scores. RESULTS: The response rate achieved was 89.7%. Of the parents of children with TD, 76.9% achieved caseness on the GHQ-28 compared with 34.6% of the parents of children with asthma; this effect remained significant after controlling for demographic variables. Parents of children with TD also experienced greater caregiver burden, and this burden was significantly correlated with GHQ caseness. CONCLUSIONS: Parents of children with TD are at risk of psychiatric morbidity; an intervention targeting caregiver burden might be helpful in reducing this.