The Gilles de la Tourette syndrome: the current status.

Gilles de la Tourette syndrome (GTS) is characterised by multiple motor and one or more vocal/phonic tics. GTS was once thought to be rare, but many relatively recent studies suggest that the prevalence is about 1% of the worldwide community, apart from in Sub-Saharan Black Africa. Comorbidity and coexistent psychopathology are common, occurring in about 90% of clinical cohorts and individuals in the community. The most common comorbidities are attention deficit hyperactivity disorder, obsessive-compulsive behaviours, and disorder, and autistic spectrum disorders, while the most common coexisting psychopathologies are depression, anxiety and behavioural disorders such as oppositional defiant and conduct disorder. There has been an increasing amount of evidence to show that the quality of life in young people is reduced when compared with normative data or healthy control populations. It is widely accepted that most cases of GTS are inherited, but the genetic mechanisms appear much more complex than previously understood, as evidenced by many recent studies; indeed, there have been suggestions of 'general neurodevelopmental genes' which affect the brain development after which the 'specific GTS gene(s)' may further affect the phenotype. Other aetiopathogenetic suggestions have included environmental factors such as neuro-immunological factors, infections, prenatal and peri-natal difficulties and androgen influences. Few studies have addressed aetiology and phenotype, but initial results are exciting. The search for endophenotypes has followed subsequently. Intriguing neuroanatomical and brain circuitry abnormalities have now been suggested in GTS; the most evidence is for cortical thinning and a reduction in the size of the caudate nucleus. Thorough assessment is imperative and multidisciplinary management is the ideal. Treatment should be 'symptom targeted', and in mild cases, psycho-education and reassurance for the patient and the family may be sufficient. Behavioural treatments such as Comprehensive Behavioural Intervention for Tics including Habit Reversal Training have been shown to be significantly better than other behavioural/psychological treatments and 'placebo'. Medication is often necessary for moderately affected individuals. In more severe cases, medical treatment is not simple and referral to an expert may be advisable. In general, neuroleptics and clonidine or guanfacine are the medications of choice for the tics. Other treatments which may be needed for loud and severe phonic tics include botulinum toxin. In severe adult GTS patients who are refractory to medication and other therapies, deep brain stimulation looks promising.

European clinical guidelines for Tourette syndrome and other tic disorders. Part I: assessment.

A working group of the European Society for the Study of Tourette Syndrome (ESSTS) has developed the first European assessment guidelines of Tourette Syndrome (TS). The available literature including national guidelines was thoroughly screened and extensively discussed in the expert group of ESSTS members. Detailed clinical assessment guidelines of tic disorders and their comorbidities in both children and adults are presented. Screening methods that might be helpful and necessary for specialists' differential diagnosis process are suggested in order to further analyse cognitive abilities, emotional functions and motor skills. Besides clinical interviews and physical examination, additional specific tools (questionnaires, checklists and neuropsychological tests) are recommended.

The behavioral spectrum of Gilles de la Tourette syndrome.

Gilles de la Tourette syndrome is a neurodevelopmental disorder consisting of multiple motor and one or more vocal/phonic tics. Tourette's syndrome is increasingly recognized as a common neuropsychiatric disorder usually diagnosed in early childhood, and comorbid neuropsychiatric disorders occur in approximately 90% of patients; the most common of these are attention deficit hyperactivity disorder and obsessive-compulsive disorder. Depression is also common, with a lifetime risk of 10% of patients. Moreover, a high prevalence of personality disorders has been reported in preliminary investigations on Tourette's syndrome populations. This paper provides an updated review of the literature on the multifaceted phenotype of Tourette's syndrome, with special attention to the behavioral problems and the relationship between Tourette's syndrome and comorbid neuropsychiatric conditions. The issue of whether Tourette's syndrome should still be considered as a unitary nosological entity is also addressed.

Depression in women with epilepsy: clinical and neurobiological aspects.

Patients affected by epilepsy show a considerably higher incidence of depression compared with the general population. Since women are twice as likely as men to suffer from depression, female gender could be considered a major risk factor for developing this condition. Converging lines of evidence suggest that sex hormones, which are known to contribute to remodelling the hippocampus, play a pivotal role in both epilepsy and depression. In women, the role of sex hormone levels may be more important because of their physiological cyclic fluctuations. Oestrogens, more than other ovarian hormones, show an effect similar to antidepressant drugs by stimulating hippocampal synaptogenesis, thus exerting a protective role against seizures as well. This paper reviews the current knowledge on the neurobiological basis of depression in women with epilepsy. The emerging picture informs therapeutic strategies to improve the clinical management of this common comorbidity.

Gilles de la Tourette Syndrome, Depression, Depressive Illness, and Correlates in a Child and Adolescent Population.

OBJECTIVE: Gilles de la Tourette syndrome (GTS) and depression are both common disorders. It has been suggested that depression occurs in 13%-76% GTS patients. Despite this, there are few studies into the specific relationships and correlates between the two disorders. There is only some consensus as to the precise relationship between the two disorders. MATERIALS AND METHODS: We undertook the study to investigate the relationship between depressive symptomatology and the core clinical features of GTS in a well-characterized clinical population of youth with this disorder. Our aim was to verify the association between depression and comorbid obsessive-compulsive disorder and explore further other potential associations highlighted in some, but not all, of the studies focused on this topic. RESULTS: Our results demonstrated that (1) the GTS patients were significantly older than the controls, (2) the GTS patients were significantly more depressed than controls, (3) depression was associated with tic severity, (4) the Diagnostic Confidence Index scores were higher in GTS patients without depression, (5) anxiety, attention-deficit/hyperactivity disorder (ADHD), conduct disorder (CD), and behavioral problems were significantly associated with depression, and (6) finally, patients with GTS and depression have a positive family history of depression. However, obsessionality (CY-BOCS) did not differentiate between depressed and not depressed GTS patients. CONCLUSIONS: Depression is common in patients with GTS and occurs significantly more in GTS than in controls. Depression is significantly associated with GTS factors such as tic severity, comorbidity with ADHD, and the presence of coexistent anxiety, CDs, and behavior problems. Depression is importantly significantly associated with a positive family history of depression. Intriguingly, depression in our sample was not related to obsessionality.

Mood disorders and Gilles de la Tourette's syndrome: An update on prevalence, etiology, comorbidity, clinical associations, and implications.

Gilles de la Tourette's syndrome (GTS) consists of multiple motor tics and one or more phonic tics. Psychopathology occurs in approximately 90% of GTS patients, with attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD) being common. Depression is common, with a lifetime risk of 10% and a prevalence of between 1.8% and 8.9%. Depression and depressive symptoms are found to occur in 13% and 76% of GTS patients attending specialist clinics, respectively. In controlled studies embracing over 700 GTS patients, the patients were significantly more depressed than controls in all but one instance. In community and epidemiological studies, depression in GTS individuals was evident in two of five investigations. Clinical correlates of depression in people with GTS appear to be: tic severity and duration, the presence of echophenomena and coprophenomena, premonitory sensations, sleep disturbances, obsessive-compulsive behaviors/OCD, self-injurious behaviors, aggression, conduct disorder (CD) in childhood, and, possibly, ADHD. Depression in people with GTS has been shown to result in a lower quality of life, potentially leading to hospitalization and suicide. The etiology of depression appears to be multifactorial. Bipolar affective disorder (BAD) and GTS may be related in some individuals. However, it is noted that sample sizes in most of these studies were small, and it is unclear at the present time as to why BAD may be overrepresented among GTS patients.

The role of clinical phenotypes in understanding the genetics of obsessive-compulsive disorder.

Studies have shown that genetic factors are significant in predisposing individuals to obsessive-compulsive disorder (OCD). Family studies have demonstrated significantly higher rates of OCD in parents and siblings of OCD probands with an age-corrected morbid risk ranging from approximately 10% to 35% in first-degree relatives. Twin studies suggest that this familiality is, in part, due to genetic factors, and results from complex segregation analyses imply the existence of genes that have major effects on the transmission of OCD. However, not all cases of OCD seem to be familial. Furthermore, it appears that even in the familial form, there are clinical and genetic heterogeneities. Thus, future studies should either adjust the prevalence rates used in genetic analyses to account for nonfamilial cases or perform separate analyses of those families with a demonstrably familial form of OCD. Furthermore, in complex psychiatric disorders such as OCD, a single genetic locus may influence only a small part of phenotypic variance, and other genetic and environmental factors may interact in determining clinical phenotype. The implications of this finding on clinical and genetic heterogeneity in OCD are discussed.

Tourette's: syndrome, disorder or spectrum? Classificatory challenges and an appraisal of the DSM criteria.

The fifth version of the Diagnostic and statistical manual of mental disorders (DSM-5) was released in May 2013 after 14 years of development and almost two decades after the last edition DSM-IV was published in 1994. We review the DSM journey with regards to Tourette Syndrome from the original publication of DSM 1 in 1952 till date. In terms of changes in DSM 5, the major shift has come in the placement of Tourette Syndrome under the 'Neurodevelopmental Disorders' alongside other disorders with a developmental origin. This review provides an overview of the changes in DSM-5 highlighting key points for clinical practice and research along with a snap shot of the current use of DSM as a classificatory system in different parts of the world and suggestions for improving the subtyping and the diagnostic confidence.

The international prevalence, epidemiology, and clinical phenomenology of Tourette syndrome: a cross-cultural perspective.

The overall international prevalence of Tourette syndrome (TS) is 1% in the majority of cultures of the world. Both TS and tics are certainly more obvious and may be more common in younger people. Moreover, TS is seen less frequently in some cultures. However, in all cultures where it has been reported, the phenomenology is similar, highlighting the biological underpinnings of the disorder. This article reviews the international prevalence, epidemiology, and clinical phenomenology of TS, from a cross-cultural perspective.