Blocking Phenomena in Gilles de la Tourette Syndrome.
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ABSTRACT:
SUBMITTER: Ganos C
PROVIDER: S-EPMC6353507 | biostudies-literature | 2015 Dec
REPOSITORIES: biostudies-literature
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Blocking Phenomena in Gilles de la Tourette Syndrome.
Ganos Christos C Müller-Vahl Kirsten K Bhatia Kailash P KP
Movement disorders clinical practice 20150630 4
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Project description:Introduction: Patients with Gilles de la Tourette syndrome (GTS) may experience blocking tics (BTs) defined as recurrent, brief cessations of motor acts. The aim of this study was to assess the prevalence, age of onset, and clinical correlates of BTs in GTS patients. Materials and Methods: We performed a one-time registration study in a cohort of 195 consecutive GTS patients aged 5-66 years (mean age: 15.0 ± 9.2; 47 females, 24.1%). All patients were personally interviewed and examined. Results: At least one BT occurred at some point in the lifetime of 73 patients (37.4%) with a mean age of onset of 10.4 ± 5.9 years. BTs occurred an average of 4.8 ± 5.3 years after tic onset. The most common BT was cessation of walking (n = 59, 80.8%), followed by speech (n = 19, 26.0%), running (n = 18, 24.7%), and writing (n = 9, 12.3%). Most of the patients (n = 52, 71.2%) reported cessation of only one activity. Clinical associations of BTs included more severe tics, overall greater number of tics, and, to a lesser extent, higher age at evaluation and comorbid obsessive-compulsive disorder. Conclusions: BTs represent complex tics, early and common symptoms of GTS, and are associated with a more severe form of GTS.
| S-EPMC8201401 | biostudies-literature
Project description:Gilles de la Tourette syndrome (TS) is a complex neurodevelopmental disorder characterized by the presence of motor and vocal tics as well as psychiatric comorbidities such as attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), depression, and anxiety. The underlying cause of the disease is still unknown, but several lines of evidence suggest a paramount role of the dopaminergic system. Based on the clinical observation that cannabis-based medicine including cannabis and delta-9-tetrahydrocannabinol (THC, dronabinol) may improve TS, alternatively, an involvement of the endocannabinoid system (ECS) has been suggested. In this study we measured cerebrospinal fluid (CSF) levels of the two most important endocannabinoids "N"-arachidonoylethanolamine (AEA, anandamide) and 2-arachidonoylglycerol (2-AG), the endocannabinoid-like molecule palmitoyl ethanolamide (PEA), and the lipid arachidonic acid (AA) in a sample of adult patients with TS (n = 20) compared with controls (n = 19) using liquid-liquid lipid extraction and simultaneous quantification by liquid chromatography multiple reaction monitoring (LC/MRM). CSF levels of AEA (p = 0.0018), 2-AG (p = 0.0003), PEA (p = 0.02), and AA (p < 0.0001) were significantly increased in TS compared with controls. Levels of 2-AG correlated with the severity of comorbid ADHD (p < 0.01). This is the first study, demonstrating alterations in the ECS suggesting an involvement of this system in the pathophysiology of TS. It can be speculated that elevated endocannabinoid levels either represent secondary changes in order to compensate for alterations in other neurotransmitter systems such as the dopaminergic system, are simply an epiphenomenon or, alternatively, represent the primary cause of TS.
| S-EPMC7297729 | biostudies-literature
Project description:Heat has been reported to exert variable effects on people with Gilles de la Tourette syndrome (TS). At age 24 years, a 32-year-old right-handed man with TS experienced a marked reduction in tics for two years after undergoing dehydration by entering a hot tub at 103°F (39.4°C) to 104°F (40.0°C) for 3 to 4 hours. On the Yale Global Tic Severity Scale (YGTSS) he scored 55 seven months before dehydration and 13 one month after dehydration. An intense heat exposure and dehydration led to an apparent remission in tics. The remission continued without the use of prescribed or nonprescribed medications or substances for two years until tics returned in the worst ever exacerbation after a tetanus immunization. The heat exposure may have altered at least temporarily his thermostat for normal heat-loss mechanisms through dopaminergic pathways from the anterior hypothalamus to the basal ganglia and the substantia nigra. Whether or not that mechanism or some other mechanism relevant to the heat exposure and/or dehydration is at play, the sudden and marked improvement in his tics needs further attention. Prospective testing of the heat and dehydration effect on tics should be pursued.
| S-EPMC6078428 | biostudies-literature
Project description:The novel coronavirus disease (COVID-19) was identified as the cause of an outbreak of respiratory disease in China at the end of 2019. It then spread with enormous rapidity and by mid-March 2020 was declared a world pandemic. Gilles de la Tourette Syndrome (GTS) is a childhood-onset neurodevelopmental disorder with a worldwide prevalence of about 1% of the population. The clinical symptoms include multiple motor and one or more phonic (vocal) tics. Germane to this communication is that 85% of patients with GTS have associated psychiatric co-morbidities, many of which are being exacerbated in the current global health crisis. In addition, several symptoms of GTS may mimic COVID-19, such as a dry cough and sniffing (phonic tics), while other symptoms such as spitting, inappropriate touching of others and "non-obscene socially inappropriate symptoms" can potentially get patients with GTS into trouble with the law. We suggest that a clear explanation of the COVID-19 illness and GTS is important to enable colleagues of various specialities who tend to patients with GTS. It is important to acknowledge at the outset that the information available on the COVID-19 pandemic changes daily, including cases infected, deaths reported, and how various national health systems are planning and or coping or not. It is fair to say that having read the current medical and lay press we conclude that it is not easy to reassure our patients with absolute certainty. However, notwithstanding that, we hope our documentation is of some assistance.
| S-EPMC7195896 | biostudies-literature
Project description:Gilles de la Tourette syndrome (GTS) is a childhood onset neuropsychiatric disorder characterized by the presence of motor and vocal tics. The clinical spectrum of GTS is heterogeneous and varies from mild cases that do not require any medical attention to cases that are refractory to standard treatments. One of the unresolved issues is the definition of what constitutes treatment-refractory GTS. While for some other neuropsychiatric disorders, such as obsessive-compulsive disorder (OCD), a clear definition has been established, there is still no consensus with regard to GTS. One important issue is that many individuals with GTS also meet criteria for one or more other neurodevelopmental and neuropsychiatric disorders. In many individuals, the severity of these comorbid conditions contributes to the degree to which GTS is treatment refractory. The scope of this paper is to present the current state-of-the-art regarding refractory GTS and indicate possible approaches to define it. In closing, we discuss promising approaches to the treatment of individuals with refractory GTS.
| S-EPMC7775596 | biostudies-literature
Project description:Motor symptoms in Gilles de la Tourette syndrome (GTS) have been related to changes in frontostriatal brain networks. These changes may also give rise to alterations in cognitive flexibility. However, conclusive evidence for altered cognitive flexibility in patients with GTS is still lacking. Here, we meta-analyzed data from 20 neuropsychological studies that investigated cognitive flexibility in GTS using the Wisconsin Card Sorting Test (WCST). Results revealed medium-sized GTS-related performance deficits, which were significantly modulated by age: Whilst being substantial in children and adolescents with GTS, WCST deficits seem to dissolve in adult patients with GTS. This age-related normalization of WCST performance might result from the compensatory recruitment of cognitive control in adult patients with GTS. We addressed this possibility by examining neural correlates of proactive and reactive cognitive control in an event-related potential (ERP) study. We analyzed cue- and target-locked ERPs from 23 adult patients with GTS and 26 matched controls who completed a computerized version of the WCST. Compared to controls, patients with GTS showed a marked increase in parietal cue-locked P3 activity, indicating enhanced proactive cognitive control. We conclude that the additional recruitment of proactive cognitive control might ensure flexible cognitive functioning in adult patients with GTS.
| S-EPMC6987949 | biostudies-literature
Project description:BackgroundGilles de la Tourette Syndrome (GTS) is a complex neuropsychiatric disorder, characterized by chronic motor and vocal tics, associated in 50-90% of cases with psychiatric comorbidities. Patients with moderate and severe clinical picture are treated with psychotherapy and pharmacological therapy. Deep brain stimulation (DBS) is reserved for pharmacological refractory GTS patients. As GTS tends to improve with time and potentially resolves in the second decade of life, the major concern of DBS in GTS is the age at which the patient undergoes surgical procedure. Some authors suggest performing DBS after 18 years, others after 25 years of age.Case descriptionWe present a 25-year-old patient with GTS, who was aged 17 years and was treated with thalamic DBS. DBS resulted in progressive and sustained improvement of tics and co-morbidities. After 6 years of DBS treatment, it was noted that the clinical improvement was maintained also in OFF stimulation setting, so it was decided to keep it off. After 2 years in off-setting and stable clinical picture the entire DBS device was removed. Six months after DBS device removal the patient remained symptom-free.ConclusionsDBS is a therapeutic option reserved for severe and refractory GTS cases. In our opinion DBS might be considered as a temporary application in GTS.
| S-EPMC4521224 | biostudies-literature
Project description:Genomic Studies of Gilles de la Tourette Syndrome
| PRJNA390461 | ENA
Project description:Introduction: Stimulus-bound tics (SBTs) belong to stimulus-induced behaviors and are defined as tics that occur in response to internal or external stimuli. The aim of the study was to assess the prevalence and associations of SBTs with other stimulus-triggered behaviors, premonitory urges and stimulus sensitization in Gilles de la Tourette syndrome (GTS). Methods: We performed a prospective, one-registration study in a cohort of 140 consecutive patients with GTS. Duration of GTS was 10.6 ± 8.7 years (range: 0-39 years). SBTs were diagnosed during the interview. Results: SBTs occurred at some point in the lifetime of 20.7% of patients. The presence of SBTs in adults was four times as frequent as in children (35.5% vs. 9.0%) with the most frequent onset in adolescence (58.8%) and adulthood (29.4%). These tics started 9.1 ± 4.7 years after the onset of tics. One stimulus and mental stimulus preceded tics most frequently, 44.8 and 33.3%, respectively. There was no established pattern of tics triggered by stimuli. Multivariate logistic regression analysis showed significant associations of SBTs with age at evaluation, tic severity, and palilalia but not with any co-morbid psychiatric disorders. 80% of patients showed at least one stimulus-triggered behavior. Premonitory urges and stimulus sensitization were reported by 60.0 and 40.7% of patients, respectively. No significant correlations between SBTs, premonitory urges and stimulus sensitization were found. Conclusion: SBTs are a part of the tic spectrum and should be taken into account by clinicians who deal with GTS patients. These tics fall at the tic end of the continuum of stimulus-induced behaviors.
| S-EPMC6024546 | biostudies-literature
Project description: Not available
| S-EPMC6183241 | biostudies-literature