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Comorbid Down's syndrome, Tourette syndrome and intellectual disability: registry prevalence and developmental course.

The co-occurrence of Tourette syndrome (TS) and Down's syndrome (DS) has been previously reported in the literature. In the present study, a retrospective record review was conducted using the North Dakota TS registry in order to ascertain the number of cases of TS and DS, and to develop case descriptions. We identified five cases from North Dakota. Two of these patients were simply comorbid for TS and DS. One was additionally comorbid for bipolar disorder, another for childhood disintegrative disorder and a third had a D/G group translocation. The association between DS and TS occured in 2% of TS patients. Contrary to the situation in patients with pervasive developmental disorders, the presence of TS in DS may be a negative prognostic indicator.

Adolescent↗

A complete genome screen in sib pairs affected by Gilles de la Tourette syndrome. The Tourette Syndrome Association International Consortium for Genetics.

Gilles de la Tourette syndrome is a neuropsychiatric disorder characterized by waxing and waning multiple motor and phonic tics with a complex mode of inheritance. Previous attempts, which used large multigenerational families to localize susceptibility loci, have been unsuccessful. In this report, the results of the first systematic genome scan, using 76 affected-sib-pair families with a total of 110 sib pairs, are summarized. While no results reached acceptable statistical significance, the multipoint maximum-likelihood scores (MLS) for two regions (4q and 8p) were suggestive (MLS > 2.0). Four additional genomic regions also gave multipoint MLS scores between 1.0 and 2.0.

Female↗

Effects of medications on regulation of body temperature of patients with Tourette syndrome.

Tourette syndrome is defined and characterized mainly by the presence of motor and phonic tics. Frequently, other medical, psychologic, and psychiatric symptoms coincide with Tourette syndrome. Despite extensive efforts extended over many years of research, the etiology leading to Tourette syndrome remains obscure. A number of hypotheses have been offered in the past to resolve the pathophysiology of Tourette syndrome. Based on the existence of an abnormal body temperature profile in patients with Tourette syndrome, an idiopathic hypothalamic disorder has been proposed to be involved in this ailment. When monitoring the effects of medications employed in the consensus for Tourette syndrome treatments, it became evident that medications ameliorate neurologic tics and rectify the patient's periodic hypothermal body temperature. Correlating the neurologic status to hypothalamic dysregulation, the data presented in this study strengthen the hypothesis of an idiopathic hypothalamic disorder underlying the Tourette syndrome cascade.

Adolescent↗

Height distribution in children with Tourette syndrome.

Tourette syndrome is characterized by motor and vocal tics, frequently accompanied by attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder, coprolalia, aggressive or self-injurious behavior, and learning disabilities. We investigated the height distribution and clinical characteristics of 38 consecutive patients with Tourette syndrome. Seventeen patients with Tourette syndrome (44.7%) were in the lower height quartile versus 25% from a control group of 44 patients with ADHD (P <.05). The mean standard deviation score differential (patient height - [target height]) was -1.12 +/- 0.82 for patients in the first quartile (group A) compared with 0.42 +/- 0.63 in taller patients with Tourette syndrome (group B) (P < .001). The mean birthweight of boys in group A versus group B was 3023 +/- 351 g and 3363 +/- 486 g, respectively (P <.02); birthweight correlated with standard deviation score (r=.43). Current weight relative to height was normal. Conduct disorder and/or self-injurious behavior were more common in group A patients (P < .05). Relative short stature appears common in Tourette syndrome, and its presence with other features may implicate a neurotransmitter system that interacts with neuroendocrine pathways, controlling height.

Adult↗

Development of behavioral and emotional problems in Tourette syndrome.

Tourette syndrome is a movement disorder with multiple neuropsychiatric features. Using the parent form of the Child Behavior Checklist by Achenbach and Edelbrock, we identified behavioral and emotional difficulties in 78 males, 6-16 years of age, with Tourette syndrome. Symptoms most often identified included obsessive-compulsive behavior, aggressiveness, hyperactivity, immaturity, withdrawal, and somatic complaints. Abnormal checklist scores were most prevalent in adolescents with Tourette syndrome. Tic severity was not a statistically significant predictor of behavioral disturbance, although a suggestive relationship between tic severity and behavioral disturbance was observed in the 12- to 16-year-old group. Dividing Tourette syndrome patients into those with or without hyperactivity failed to identify whether hyperactivity had a major impact on abnormal behaviors. Our results illustrate the relative frequency of psychopathology in Tourette syndrome and emphasize the need for a comprehensive approach to this syndrome.

Adolescent↗

The genetics of Tourette syndrome.

Tourette syndrome has significant genetic determinants. The mode of transmission, while mildly controversial, generally is thought to be due to a single major locus inherited either as an autosomal dominant trait with reduced penetrance, or as a trait with intermediate inheritance in which some heterozygotes manifest the disorder. These is evidence for a Tourette syndrome spectrum of symptoms that includes obsessive-compulsive disorder. Systematic genome linkage studies of Tourette syndrome are progressing, but to date there are no significant linkage findings, although the search has included many neurologically relevant candidate genes.

Diseases in Twins↗

Helping school-age children cope with Tourette syndrome.

Tourette Syndrome, a neurological "tic" disorder characterized by involuntary muscular movements and vocal sounds, can be psychologically devastating and unbearable for the school-age child. Through the help and support of educational and medical professionals, children and their families can learn to accept and cope with the uncontrollable, bizarre behavior of the Tourette Syndrome.

Adaptation, Psychological↗

[Early developmental aspects of Tourette syndrome].

Tourette syndrome is a neuropsychiatric disorder characterized by a combination of multiple motor and vocal tics. It is frequently associated with other manifestations which are typical, though not obligatory for the diagnosis. Some of these manifestations may appear in early childhood and cause developmental disorders. Our observations in 2 families, in which an early developmental symptom was later found to be an associated manifestation of Tourette syndrome, led us to investigate our other patients with this syndrome. In all, 16 patients were studied. Early developmental disabilities, such as speech and language disorder, learning disabilities, attention deficit hyperactivity disorder, motor clumsiness or behavioral problems were found in 8. An epidemiological survey is needed to determine the prevalence of the association of Tourette syndrome with developmental manifestations in early childhood.

Adolescent↗

Helping your patient cope with Tourette syndrome.

Tourette Syndrome is a neurological disorder of unknown origin. The individual suffering from Tourette Syndrome encounters tics and often additional behaviors such as speech, sleep, and learning difficulties, as well as social isolation.

Child↗

Tourette syndrome.

Tourette syndrome is not rare. At an early age, persons with this disorder show involuntary, rapid and purposeless movements of multiple muscle groups. They also develop vocal tics. Both motor and vocal tics vary in intensity and can worsen with stress. The syndrome may be confused with, or associated with, a variety of psychiatric disorders. Although some cases appear to be familial, the etiology of the syndrome is generally unknown. Haloperidol is effective in controlling symptoms in many patients. The Tourette Syndrome Association provides a valuable support system.

Child↗

Mental health needs associated with Tourette syndrome.

Tourette Syndrome (TS) is an uncommon developmental disability characterized by repetitive and involuntary verbal and motor tics. A survey of all known affiliates of the Tourette Syndrome Association of Ohio was conducted. A total of 431 questionnaires was returned, and behavioral problems were found to be pervasive in Tourette people. Some 53.1 per cent of the sample had sought some form of counseling for these problems, but this counseling was generally not perceived as being very helpful. Medication was not reported to ameliorate behavioral problems. When compared to a normal population sample, self-ratings of mental health status were low among Tourette persons. Problems experienced by TS persons were aggregated into a Behavioral Problem Scale, which successfully discriminated between levels of need in TS persons.

Adolescent↗

[Tourette syndrome].

Tourette Syndrome is not as rare as many health professionals may think. On the contrary it is very common, especially among the Afrikaans population. It is possibly the most common inherited condition. Tourette Syndrome is a neurological variation characterized by "tics" or mannerisms, which can also be combined with one or all of the following: frontal lobe involvement particularly the limbic system; learning problems; anxiety; obsessive-compulsive behaviour; sexual deviations. Most authorities regard TS as a dominant inherited condition although Comings describes it as semi-dominant--semi-recessive.

Humans↗

Nicotine attenuates DOI-induced head-twitch response in mice: implications for Tourette syndrome.

Tourette syndrome (TS), a chronic neuropsychiatric disorder, is characterized by motor and vocal tics. Preliminary clinical studies indicate possible therapeutic benefits of nicotine in the treatment of Tourette's syndrome (TS). It has been proposed that twitches of the head in mice or twitches of head and shoulders in rats following administration of the selective 5HT(2A/C) agonist DOI (1-)2,5-dimethoxy-4-iodophenyl-2-aminopropane, can serve as an animal model of tics in TS. In this study, the effects of acute and chronic administration of nicotine on DOI-induced head twitch response (HTR) in male albino ICR mice were evaluated. Both acute and chronic nicotine (daily injections for 10 days) reduced the DOI-induced HTR. Moreover, chronic administration of DOI (1 mg/kg/day for 10 days) resulted in 65% increase in [125I]alpha-bungarotoxin binding in cerebellum and 41% increase in striatal [3H]cytisine binding. However, the acute inhibitory effects of nicotine were not blocked by pretreatment with the nicotinic antagonist, mecamylamine. Indeed, at higher doses, mecamylamine also reduced the DOI-induced HTR. The data suggest that both nicotine and mecamylamine may be of therapeutic potential in the treatment of some symptoms of TS.

Animals↗

Association between 7q31 markers and Tourette syndrome.

Tourette syndrome (TS) is a complex neuropychiatric disorder with a strong genetic basis. Although no specific susceptibility genes have been identified for TS, cytogenetic studies in selected cases suggest the existence of a predisposing gene located in the 7q31 chromosomal region. In order to test the hypothesis of a possible relationship between this region and TS at the population level, we undertook a family based association study in a sample of French Canadian patients from Quebec. For this purpose, markers D7S522, D7S523, and D7S1516 were tested using the extended transmission disequilibrium test (e-TDT). Marker D7S522 showed a biased transmission of alleles from heterozygote parents to their TS offsprings (allele-wise TDT chi(2) = 12.61, 4 df, P = 0.013, genotype-wise TDT chi(2) = 15.49, 7 df, P = 0.030). When the analysis was restricted to patients without ADHD or OCD comorbidity, similar results were observed both allele and genotype-wise (chi(2) = 10.68, 4 df, P = 0.03 and chi(2) = 12.55, 5 df, P = 0.028, respectively). In addition, marker D7S523 was also associated (allele-wise TDT chi(2) = 18.37, 7 df, P = 0.01 and genotype-wise TDT chi(2) = 46.26, 17 df, P = 0.00016), and showed a tendency for association in the comorbidity-free subgroup (genotype-wise TDT chi(2) = 18.7, 10 df, P = 0.044). Finally, marker D7S1516, contained in the inner mitochondrial membrane peptidase 2 like (IMMP2L) gene, also showed a tendency for association (genotype-wise TDT chi(2) = 32.87, 21 df, P = 0.048). These results may reflect the proximity of markers D7S522, D7S523, and possibly D7S1516 to a gene or regulatory region relevant to TS predisposition.

Adult↗

Hypothesis III. Tourette syndrome is only one of several causes of a developmental basal ganglia syndrome.

Tourette syndrome (TS) is a hereditary condition characterized by the presence of chronic, multiple motor tics and is commonly associated with obessive-compulsive behavior, attentional and executive dysfunction, and aggressive behavior. A wide range of other genetic and environmental conditions that interfere with normal basal ganglia developmental processes can produce a clinical syndrome that we call the developmental basal ganglia syndrome (DBGS), and which closely resembles TS. Failure to distinguish TS from other causes of DBGS has probably hampered research efforts aimed at clarifying the neurobiological mechanisms, localizing the genetic defect, assessing therapy, and establishing accurate prevalence rates for TS.

Aggression↗

Novel surgical therapies for Tourette syndrome.

Tourette syndrome is a common neurologic disorder with close ties to obsessive-compulsive disorder and attention-deficit hyperactivity disorder (ADHD). The majority of patients improve spontaneously as they near adulthood. Most others are effectively treated with pharmacologic agents, although these therapies fail for an unfortunate few. For patients debilitated by the disease despite medical therapy, surgery can become an important option.

Brain↗

Clinical predictors of psychopathology in children and adolescents with Tourette Syndrome.

Tourette Syndrome (TS) is a neuropsychiatric disorder of childhood onset characterized by vocal and motor tics and associated psychopathologies. The current study was undertaken to explore the associations between tic symptomatology, related clinical variables and behavioral dysfunction within a cohort of TS subjects. Ninety-two child and adolescent TS subjects were rated through self-measure, and by parents on measures of tic symptomatology, OC characteristics, and dysfunctional behaviors including learning difficulties and attention deficits. Statistical modeling revealed associations among tic clusters, clinical items and behavioral measures, which were unique for the child and adolescent subgroups.

Adolescent↗

Eye movement abnormalities in a case of Tourette syndrome.

Tourette syndrome (TS) is a neuropsychiatric disorder that is characterised by the presence of multiple vocal, facial, and motor tics which change with time, and a number of other behavioural phenomena. Previous studies have not revealed any ocular-motor abnormalities. We report the eye movement studies of a patient with TS, using electrooculography and simultaneous video recording. Intrusive saccades occurred during smooth pursuit and optokinetic nystagmus. Reflexive and voluntary saccades were dysmetric and there was a complete failure of antisaccades. These abnormalities are characteristic of disease of the frontal lobes and basal ganglia. We review the literature with respect to the eye movement abnormalities associated with TS.

Adolescent↗