Tics emergencies and malignant tourette syndrome: Assessment and management.

Tourette syndrome (TS) is a complex neurodevelopmental disorder characterized by the presence of tics, frequently accompanied by a variety of neuropsychiatric comorbidities. A subset of patients with TS present with severe and disabling symptoms, requiring prompt therapeutic intervention. Some of these manifestations may result in medical emergencies when severe motor or phonic tics lead to damage of anatomical structures closely related to the tic. Examples include myelopathy or radiculopathy following severe neck ("whiplash") jerks or a variety of self-inflicted injuries. In addition to self-aggression or, less commonly, allo-aggression, some patients exhibit highly inappropriate behavior, suicidal tendencies, and rage attacks which increase the burden of the disease and are important components of "malignant TS". This subset of TS is frequently associated with comorbid obsessive-compulsive disorder. Therapeutic measures include intensive behavioral therapy, optimization of oral pharmacotherapy, botulinum toxin injections, and deep brain stimulation.

Responsive deep brain stimulation for the treatment of Tourette syndrome.

To report the results of 'responsive' deep brain stimulation (DBS) for Tourette syndrome (TS) in a National Institutes of Health funded experimental cohort. The use of 'brain derived physiology' as a method to trigger DBS devices to deliver trains of electrical stimulation is a proposed approach to address the paroxysmal motor and vocal tic symptoms which appear as part of TS. Ten subjects underwent bilateral staged DBS surgery and each was implanted with bilateral centromedian thalamic (CM) region DBS leads and bilateral M1 region cortical strips. A series of identical experiments and data collections were conducted on three groups of consecutively recruited subjects. Group 1 (n = 2) underwent acute responsive DBS using deep and superficial leads. Group 2 (n = 4) underwent chronic responsive DBS using deep and superficial leads. Group 3 (n = 4) underwent responsive DBS using only the deep leads. The primary outcome measure for each of the 8 subjects with chronic responsive DBS was calculated as the pre-operative baseline Yale Global Tic Severity Scale (YGTSS) motor subscore compared to the 6 month embedded responsive DBS setting. A responder for the study was defined as any subject manifesting a ≥ 30 points improvement on the YGTSS motor subscale. The videotaped Modified Rush Tic Rating Scale (MRVTRS) was a secondary outcome. Outcomes were collected at 6 months across three different device states: no stimulation, conventional open-loop stimulation, and embedded responsive stimulation. The experience programming each of the groups and the methods applied for programming were captured. There were 10 medication refractory TS subjects enrolled in the study (5 male and 5 female) and 4/8 (50%) in the chronic responsive eligible cohort met the primary outcome manifesting a reduction of the YGTSS motor scale of ≥ 30% when on responsive DBS settings. Proof of concept for the use of responsive stimulation was observed in all three groups (acute responsive, cortically triggered and deep DBS leads only). The responsive approach was safe and well tolerated. TS power spectral changes associated with tics occurred consistently in the low frequency 2-10 Hz delta-theta-low alpha oscillation range. The study highlighted the variety of programming strategies which were employed to achieve responsive DBS and those used to overcome stimulation induced artifacts. Proof of concept was also established for a single DBS lead triggering bi-hemispheric delivery of therapeutic stimulation. Responsive DBS was applied to treat TS related motor and vocal tics through the application of three different experimental paradigms. The approach was safe and effective in a subset of individuals. The use of different devices in this study was not aimed at making between device comparisons, but rather, the study was adapted to the current state of the art in technology. Overall, four of the chronic responsive eligible subjects met the primary outcome variable for clinical effectiveness. Cortical physiology was used to trigger responsive DBS when therapy was limited by stimulation induced artifacts.

Efficacy and safety of combined deep brain stimulation with capsulotomy for comorbid motor and psychiatric symptoms in Tourette's syndrome: Experience and evidence.

OBJECTIVES: To evaluate the efficacy and safety of combined deep brain stimulation (DBS) with capsulotomy for comorbid motor and psychiatric symptoms in patients with Tourette's syndrome (TS). METHODS: This retrospective cohort study consecutively enrolled TS patients with comorbid motor and psychiatric symptoms who were treated with combined DBS and anterior capsulotomy at our center. Longitudinal motor, psychiatric, and cognitive outcomes and quality of life were assessed. In addition, a systematic review and meta-analysis were performed to summarize the current experience with the available evidence. RESULTS: In total, 5 eligible patients in our cohort and 26 summarized patients in 6 cohorts were included. After a mean 18-month follow-up, our cohort reported that motor symptoms significantly improved by 62.4 % (P = 0.005); psychiatric symptoms of obsessive-compulsive disorder (OCD) and anxiety significantly improved by 87.7 % (P < 0.001) and 78.4 % (P = 0.009); quality of life significantly improved by 61.9 % (P = 0.011); and no significant difference was found in cognitive function (all P > 0.05). Combined surgery resulted in greater improvements in psychiatric outcomes and quality of life than DBS alone. The synthesized findings suggested significant improvements in tics (MD: 57.92, 95 % CI: 41.28-74.56, P < 0.001), OCD (MD: 21.91, 95 % CI: 18.67-25.15, P < 0.001), depression (MD: 18.32, 95 % CI: 13.26-23.38, P < 0.001), anxiety (MD: 13.83, 95 % CI: 11.90-15.76, P < 0.001), and quality of life (MD: 48.22, 95 % CI: 43.68-52.77, P < 0.001). Individual analysis revealed that the pooled treatment effects on motor symptoms, psychiatric symptoms, and quality of life were 78.6 %, 84.5-87.9 %, and 83.0 %, respectively. The overall pooled rate of adverse events was 50.0 %, and all of these adverse events were resolved or alleviated with favorable outcomes. CONCLUSIONS: Combined DBS with capsulotomy is effective for relieving motor and psychiatric symptoms in TS patients, and its safety is acceptable. However, the optimal candidate should be considered, and additional experience is still necessary.

Parent-Report Sleep Disturbances and Everyday Executive Functioning Difficulties in Children with Tourette Syndrome.

There is an increasing need to identify and treat sleep disturbances in Tourette syndrome (TS), a neurodevelopmental condition characterized by tics. This study explored sleep, tics, and executive functioning in children with TS (n=136) and neurotypical controls (n=101) through parent-report scales and open-ended questions. 85% of children with TS scored in the clinical range for a sleep disorder. Higher tic severity predicted increased sleep disturbances and executive difficulties. Qualitative insights indicated a bidirectional link between sleep and tics, which warrants consideration in clinical settings. Further research is needed to explore causal links.

Advancements in the phenomenology, assessment, and treatment of Tourette syndrome.

PURPOSE OF REVIEW: Tourette syndrome (TS) and chronic tic disorders, collectively referred to as TS, are relatively common childhood onset neuropsychiatric conditions associated with functional impairment and distress. Over the past several years, clinical research has contributed to the advancement of the field's understanding of mechanisms and clinical correlates of TS. This progress has led to the development of key assessment tools and the implementation of novel interventions for individuals with TS. RECENT FINDINGS: This article provides a review of innovative TS research focusing on four key themes: investigation of clinical phenomenology of TS; validation of assessment tools for TS; dissemination of current evidence-based treatments for TS; and exploration of new intervention programs. SUMMARY: Cumulatively, this growing body of work presents considerable progress and provides a path forward to improve the assessment and treatment of TS.

[Formula: see text] Beyond the rubicon: a continuum approach to investigating the impact of ADHD like characteristics on everyday executive function in children with Tourette Syndrome.

Across a range of neurodevelopmental conditions, a diagnosis of attention deficit hyperactivity disorder (ADHD) has been shown to be associated with executive function impairment. However, the DSM-V emphasis upon viewing psychological characteristics as existing on a continuous distributed quantitative dimension has enabled the opportunity to consider the influence of sub-diagnostic or sub-referral levels of these psychological characteristics upon cognitive function. This study adopted a continuum approach to the consideration of this ADHD influence and examined the extent to which the difference in parental reported executive functions between children with Tourette syndrome (TS) or typically developing children could be mediated by a concurrent group difference in the possession of sub-referral levels of ADHD-like characteristics. A total of 146 children, 58 with reported TS diagnosis, participated. Parental report measures of ecological executive functioning, the Child Executive Functioning Inventory, and the Vanderbilt ADHD Diagnostic Parent Rating Scale were employed. The analyses with a full sample and a sub-referral sample revealed significant group differences in most of the key measures. In addition, these measures were highly correlated even when controlling for age and gender. A series of mediation analyses indicated that in all models, the ADHD-like measures significantly mediated the group difference in executive function. These results suggest that sub-referrals levels of ADHD-like characteristics continue to contribute to executive challenges in TS. Future intervention research targeting these executive functions should consider the presence of ADHD-like characteristics at sub-referral levels of possession.

Co-morbid tics and stereotypies: a systematic literature review.

BACKGROUND: Tics and stereotypies are childhood-onset repetitive behaviours that can pose significant diagnostic challenges in clinical practice. Both tics and stereotypies are characterised by a complex co-morbidity profile, however little is known about the co-occurrence of these hyperkinetic disorders in the same patient population. OBJECTIVE: This review aimed to assess the relationship between tics and stereotypies when these conditions present in co-morbidity. METHODS: We conducted a systematic literature review of original studies on co-morbid tics and stereotypies, according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: Our literature search identified six studies of suitable sample size (n ≥ 40) presenting data on the association between tics and stereotypies in otherwise typically developing patients. A considerable proportion (23%) of patients diagnosed with stereotypic movement disorder present with co-morbid tics (range 18-43%). Likewise, the prevalence of primary stereotypies is increased in patients with tic disorders such as Tourette syndrome (8%, range 6-12%). DISCUSSION: Tics and stereotypies can often develop in co-morbidity. The association of tics and stereotypies in the same patient has practical implications, in consideration of the different treatment approaches. Future research should focus on the assessment and management of both conditions, particularly in special populations (e.g. patients with pervasive developmental disorders).

Effectiveness of speech therapy in treating vocal blocking tics in children with Tourette syndrome: Two case reports.

Tourette syndrome is characterized by at least two motor tics and one vocal tic, which persist for over a year. Infrequently, tics can manifest as blocking tics in speech when they prevent a person from starting to speak or interrupt their speech flow. Vocal blocking tics (VBTs) resemble stuttering, and they can be difficult to differentiate from each other. A previous report described two patients with severe VBTs who did not benefit from stuttering-therapy-based speech therapy and were treated effectively with cannabis-based medicine. Here, we present the cases of two patients, seven- and nine-year-old boys, who benefited from speech therapy in which stuttering therapy techniques were used. Detailed descriptions of the interventions are included. Further research is needed to test the effectiveness of speech therapy in treating VBTs in a larger group of children with Tourette syndrome.

Sleep and daytime functioning in children with tourette syndrome: A two-week case-control study with actigraphy and cognitive assessments.

There is increasing recognition of the high prevalence of sleep issues in children with Tourette syndrome (TS), a condition characterised by motor and vocal tics. Overnight polysomnography (PSG) has been the primary mode of sleep assessment in the TS literature, despite the extensive use of actigraphy in other neurodevelopmental populations. As a result, there are existing research gaps surrounding day-to-day variability of sleep in TS and links to daytime functioning. This study adopts a naturalistic, intensive longitudinal design to examine sleep in children with TS while considering potential links to tic severity and daytime functioning. Participants were 34 children aged between 8 and 12 years (12 with TS, 22 neurotypical controls). Wrist actigraphs tracked sleep-wake cycles across two weeks and a battery of scales and cognitive assessments measured sleep disturbances and daytime functioning. Mixed models using N = 476 nights of actigraphy data found that relative to controls, children with TS had significantly increased time in bed, increased sleep onset latency, reduced sleep efficiency, lower subjective sleep quality, but comparable actual sleep time. Higher self-report tic severity at bedtime did not predict increased sleep onset latency. In the sleep disturbance scale, 83.33 % of children with TS met the clinical cut-off for a sleep disorder. Parent-report emotional, behavioural, and executive difficulties were greater in the TS group relative to controls, but performance on cognitive tasks was comparable between groups. Together, findings highlight sleep disturbances as an important clinical factor to consider in the management of TS, though further research is required to substantiate findings in larger-scale studies. This study demonstrates the feasibility of assessing sleep via actigraphy in children with TS, supporting more widespread use in the future.

Images: Polysomnographic artifact in a patient with Tourette syndrome.

Patients with Tourette syndrome frequently have sleep disturbances that may require polysomnographic testing. The use of deep brain stimulators is increasing with expanding indications including children with medically intractable Tourette syndrome. It is important to understand the effect the stimulator can have on polysomnographic monitoring. Herein we present an interesting case of an adolescent with medically intractable Tourette syndrome with a deep brain stimulator implant who underwent a polysomnogram demonstrating rhythmic, monomorphic artifact. CITATION: Bindra T, Ingram DG. Images: Polysomnographic artifact in a patient with Tourette syndrome. J Clin Sleep Med. 2023;19(12):2149-2151.

Tourette-like syndrome secondary to Kleefstra syndrome 1 with a de novo microdeletion in the EHMT1 gene.

BACKGROUND: Gills de la Tourette syndrome (TS) is a childhood-onset neurodevelopmental disorder manifested by motor and vocal tics. Kleefstra syndrome 1 (KS1), a rare genetic disorder, is caused by haploinsufficiency of the EHMT1 gene and is characterized by intellectual disability (ID), childhood hypotonia, and distinctive facial features. Tourette-like syndrome in KS1 has rarely been reported. CASE PRESENTATION: Here we describe a 7-year-old girl presenting involuntary motor and vocal tics, intellectual disability, childhood hypotonia, and dysmorphic craniofacial appearances, as well as comorbidities including attention deficit-hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), and self-injurious behavior (SIB). The patient's CNV-seq testing revealed a de novo 320-kb deletion in the 9q34.3 region encompassing the EHMT1 gene. CONCLUSIONS: This is the first case reporting Tourette-like syndrome secondary to KS1 with a de novo microdeletion in the EHMT1 gene. Our case suggests TS with ID and facial anomalies indicate a genetic cause and broadens the phenotypic and genotypic spectrum of both TS and KS1.

Developmental stuttering, physical concomitants associated with stuttering, and Tourette syndrome: A scoping review.

BACKGROUND AND PURPOSE: Developmental stuttering and Tourette syndrome (TS) are common neurodevelopmental disorders. Although disfluencies may co-occur in TS, their type and frequency do not always represent pure stuttering. Conversely, core symptoms of stuttering may be accompanied by physical concomitants (PCs) that can be confused for tics. This scoping review aimed to explore the similarities and differences between stuttering and tics in terms of epidemiology, comorbidities, phenomenology, evolution, physiopathology, and treatment. We also described the nature of PCs in stuttering and disfluencies in TS. METHODS: A literature search on Medline, Embase and PsycInfo was executed in March 2022. From 426 studies screened, 122 were included in the review (a majority being narrative reviews and case reports). RESULTS: TS and stuttering have several epidemiological, phenomenological, comorbidity, and management similarities suggesting shared risk factors and physiopathology (involving the basal ganglia and their connections with speech and motor control cortical regions). PCs in stuttering commonly involve the face (eyelids, jaw/mouth/lip movements) and sometimes the head, trunk and limbs. PCs can be present from early stages of stuttering and vary over time and within individuals. The function of PCs is unknown. Some individuals with TS have a distinct disfluency pattern, composed of a majority of typical disfluencies (mostly between-word disfluencies), and a mix of cluttering-like behaviors, complex phonic tics (e.g. speech-blocking tics, echolalia, palilalia), and rarely, atypical disfluencies. CONCLUSION: Future investigations are warranted to better understand the complex relationships between tics and stuttering and address the management of disfluencies in TS and PCs in stuttering.

Group-based comprehensive behavioral intervention for tics (CBIT) for adults with Tourette syndrome or chronic tic disorders: A pilot study.

Comprehensive behavioral intervention for tics (CBIT) administered individually is an effective treatment for tics. However, the effectiveness of CBIT administered in groups for adults with Tourette syndrome and chronic tic disorders has not been investigated yet. This pilot study examined the effectiveness of group-based CBIT with respect to reduction of tic severity and tic-related impairment, as well as improvement of tic-related quality of life. Data from 26 patients were included in the intention-to-treat analyses. The Yale Global Tic Severity Scale was used to assess total tic severity and tic-related impairment. The Gilles de la Tourette - Quality of Life Scale was used to assess tic-related quality of life. These measures were administered at three points in time: at pretreatment, posttreatment, and 1-year follow-up. The results showed a significant reduction of total tic severity from pretreatment to 1-year follow-up, with larges effect sizes. Tic-related impairment and tic-related quality of life also improved significantly, although the effect sizes were smaller. Motor tics showed a stronger reduction than vocal tics. Additional analysis revealed that all change was achieved during treatment and that this effect was maintained from posttreatment to 1-year follow-up. The results of this study indicate that group-based CBIT is a promising treatment for tics.

Symptoms compatible with long COVID in an Italian pediatric cohort of Tourette patients with and without SARS­CoV­2 infection: a short-term follow-up assessment.

BACKGROUND: Tourette Syndrome (TS) is a childhood-onset neurodevelopmental disorder with a worldwide prevalence of about 0.3-1% of the population. During the pandemic caused by SARS-CoV-2 infection, the impact on the mental health of children and adolescents was very important. The persistence of symptoms in the post-acute phase of the disease has been termed Long COVID. The neuropsychiatric symptoms seem to be the most common impairment in children and adolescents with long COVID. OBJECTIVES: Considering the impact of pandemic on mental health, in this study we analyzed the long-term effects of SARS-CoV-2 infection in children and adolescents affected by TS. METHODS: We conducted an online questionnaire covering socio-demographic and clinical data among 158 patients affected by TS or chronic tic disorders (CTD), of which 78 participants reported a positive SARS-CoV-2 infection. Data were collected to investigate tic severity and both the comorbidities, as well as lockdown-related changes to daily life activities and, in case of infection of SARS-CoV-2, possible symptoms of acute infection and long COVID. Markers of systemic inflammation including C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), ferritin, iron, electrolytes, white blood cell counts, platelet cell counts levels, markers of liver, kidney and thyroid function were analyzed. First, all patients were screened with the Schedule for affective disorders and Schizophrenia for School age children-present and lifetime (Kiddie-SADS-PL) to rule out primary psychiatric disorders considered as criteria of exclusion. Then, all patients were clinically assessed at baseline (T0), and after three months (T1) through the administration of Yale Global Tic Severity Rating Scale (YGTSS), Multidimensional Anxiety Scale for Children (MASC), Child Depression Inventory (CDI) and Child Behavior Checklist (CBCL). RESULTS: Among the cohort of TS patients that contracted SARS-CoV-2 infection, 84.6% (n = 66) experienced any acute symptoms, and long COVID symptoms occurred in 38.5% (n = 30). A worsening of clinical symptoms of tics and eventually associated comorbidities occurred in 34.6% (n = 27) of TS patients that contracted SARS-CoV-2 infection. TS patients with or without SARS-CoV-2 infection showed an increase in the severity of tics and also behavioral, depressive and anxious symptoms. Instead, this increase was more evident in patients who contracted the infection than in patients who did not contract it. CONCLUSIONS: SARS-CoV-2 infection may have a role in the increase of tics and associated comorbidities in TS patients. Despite of these preliminary results, further investigations are necessary to improve knowledge about the acute and long-term impact of SARS-CoV-2 in TS patients.

Adolescent Gender Differences in Tic- and Non-Tic-Related Impairments in Tourette Syndrome.

We aimed to compare tic- and non-tic-related impairment experienced by adolescent girls and boys (ages 13 through 17) with Tourette syndrome and associations with age. We extracted from the electronic health record child and parental responses to the mini-Child Tourette Syndrome Impairment Scale (mini-CTIM) and other questionnaire data reflective of tic- and non-tic-related impairment of adolescents with Tourette syndrome presenting to our clinic over a 12-month period. We identified a total of 132 (49 female, 83 male) unique adolescent encounters. Mini-CTIM scores did not differ significantly between genders. Tic- and non-tic-related impairment were lower in older boys, but not older girls. Obsessive-compulsive symptoms correlated with parent-reported non-tic-related impairment experienced by adolescent girls but not boys. During adolescence, tic- and non-tic-related impairments may be less likely to improve with age in girls. Future longitudinal studies are needed to confirm this finding.

New-onset functional tics during the COVID-19 pandemic: Clinical characteristics of 105 cases from a single centre.

BACKGROUND AND PURPOSE: The COVID-19 pandemic has been associated amongst other things with a sharp increase in adolescents and young adults presenting acutely with functional tics. Initial reports have suggested clinically relevant differences between functional tics and neurodevelopmental tics seen in primary tic disorders such as Tourette syndrome. We aimed to provide confirmatory findings from the largest single-centre cohort to date. METHODS: In the present study we present data from 105 consecutive patients who developed functional tics during a 3-year period overlapping with the COVID-19 pandemic (April 2020-March 2023). All patients underwent a comprehensive neuropsychiatric assessment at a single specialist centre for tic disorders. RESULTS: Female adolescents and young adults accounted for 69% of our sample. Functional tics had an acute/subacute onset in most cases (75% with a peak of severity within 1 month). We found a disproportionately high frequency of complex movements (81%) and vocalizations (75%). A subset of patients (23%) had a pre-existing primary tic disorder (Tourette syndrome with functional overlay). The most common psychiatric co-morbidities were anxiety (70%) and affective disorders (40%). Moreover, 41% of patients had at least one functional neurological disorder in addition to functional tics. Exposure to tic-related social media content was reported by half of the patients. CONCLUSIONS: Our findings confirm substantial clinical differences between functional tics developed during the pandemic and neurodevelopmental tics. Both patient- and tic-related red flags support the differential diagnostic process and inform ongoing monitoring in the post-pandemic era.

The impact of the COVID-19 pandemic on sleep in children with Tourette syndrome in Ireland and the United Kingdom.

STUDY OBJECTIVES: The aim of this mixed-methods study was to gain an insight through qualitative and quantitative means into the impact of the coronavirus disease 2019 (COVID-19) pandemic on children with and without Tourette syndrome (TS). METHODS: Parents/guardians of children and adolescents with TS (n = 95; mean age = 11.2 years, standard deviation = 2.68 years) and typically developing controls (n = 86; mean age = 10.7 years, standard deviation = 2.8 years) in the United Kingdom and Ireland completed an online questionnaire examining sleep, with open-ended questions pertaining to their perceived impact of COVID-19 on the sleep of their children. Nine items from the Sleep Disturbance Scale for Children were used to supplement qualitative data. RESULTS: A negative impact of the pandemic on the sleep of both groups was observed, including exacerbated tics, sleep deprivation, and anxiety, with particular disruption for children with TS. Parents of children with TS reported poorer sleep patterns than parents of typically developing children on the Sleep Disturbance Scale for Children. Analyses showed that group and age predicted 43.8% of variance in sleep duration: F (4, 176) = 34.2, P < .001. CONCLUSIONS: Findings suggest that sleep patterns of children with TS may be more impacted by the pandemic than the average child. Given that there are generally more sleep issues reported in children with TS, further research is warranted in relation to the sleep health of children with TS in a postpandemic era. By identifying sleep issues potentially persisting after COVID-19, the true impact of the pandemic on the sleep of children and adolescents with Tourette syndrome may be ascertained. CITATION: Colreavy E, Keenan L, Downes M. The impact of the COVID-19 pandemic on sleep in children with Tourette syndrome in Ireland and the United Kingdom. J Clin Sleep Med. 2023;19(8):1485-1493.

Dimensional Assessment of Depression and Anxiety in a Clinical Sample of Adults With Chronic Tic Disorder.

OBJECTIVE: Among adults with Tourette syndrome, depression and anxiety symptoms are widely prevalent and consistently associated with poor quality of life. Important knowledge gaps remain regarding mood and anxiety dimensions of the adult Tourette syndrome phenotype. Taking a dimensional approach, this study sought to determine the prevalence, severity, and clinical correlates of depression and anxiety symptoms in a clinical sample of adults with Tourette syndrome and other chronic tic disorders. METHODS: A retrospective chart review was conducted of all adults with a chronic tic disorder presenting to a tertiary care Tourette syndrome clinic between December 2020 and July 2022. Information extracted during chart review included data from scales administered as part of routine care: Quality of Life in Neurological Disorders (Neuro-QoL) Depression Short Form, Neuro-QoL Anxiety Short Form, Adult Attention-Deficit/Hyperactivity Disorder Self-Report Screening Scale, Dimensional Obsessive-Compulsive Scale, and Yale Global Tic Severity Scale. Relationships between variables were examined by conducting between-group, correlation, and multivariable regression analyses. RESULTS: Data from 120 adult patients with a chronic tic disorder (77 men and 43 women) were analyzed. Neuro-QoL Anxiety scores were elevated in 66% of the cohort; Neuro-QoL Depression scores were elevated in 26%. Neuro-QoL Anxiety scores were significantly higher than general population norms, whereas Neuro-QoL Depression scores were not. After adjustment for covariates, depressive and anxiety symptom severity scores were significantly associated with each other and with obsessive-compulsive disorder symptom severity but not with tic severity. Sex-based differences emerged in the analyses. CONCLUSIONS: Among adults with chronic tic disorder, anxiety symptoms were more prevalent and severe than depressive symptoms, co-occurring psychiatric symptoms were more tightly linked with each other than with tic severity, and sex-based differences were evident.

Precision Mapping of Thalamic Deep Brain Stimulation Lead Positions Associated With the Microlesion Effect in Tourette Syndrome.

BACKGROUND: The microlesion effect refers to the improvement of clinical symptoms after deep brain stimulation (DBS) lead placement and is suggested to indicate optimal lead placement. Very few studies have reported its implications in neuropsychiatric disorders. OBJECTIVE: To evaluate the magnitude of the microlesion effect in Tourette syndrome and the relationship between the microlesion effect and the anatomic location of implanted DBS leads. METHODS: Six male patients were included. Their median age at surgery and follow-up period were 25 years (range, 18-47) and 12 months (range, 6-24), respectively. All patients were videotaped pre- and postoperatively, and tic frequencies were counted. We also analyzed the precision of lead placement and evaluated the normative connectome associated with the microlesion area. RESULTS: The microlesion effect was observed as an improvement in tic symptoms in all patients, and the long-term clinical outcomes were favorable. The median motor tic frequency was 20.2 tics/min (range, 9.7-60) at baseline and decreased to 3.2 tics/min (1.2-11.3) in patients on postoperative day 1 ( P = .043) and to 5.7 tics/min (range, 1.9-16.6) in patients on postoperative day 7 ( P = .028). Phonic tic tended to improve immediately after surgery although the changes were not significant. Image analyses revealed that the precise position of the electrode was directed toward the anteromedial centromedian nucleus. Normative connectome analysis demonstrated connections between improvement-related areas and wide areas of the prefrontal cortex. CONCLUSION: This study shows that the microlesion effect may seem as an immediate improvement after optimal DBS lead placement in patients with Tourette syndrome.

Pre-movement gating of somatosensory evoked potentials in Tourette syndrome.

OBJECTIVE: Tourette syndrome (TS) is a neurobehavioral disorder characterized by motor and vocal tics. Simple tics are purposeless involuntary movements that spontaneously resolve during middle adolescence. Complex tics appear to be semi-voluntary movements that may become intractable when associated with obsessive-compulsive disorder (OCD). Sensory tics or urges preceded by tics suggest sensorimotor processing impairment in TS. We aimed to clarify its pathophysiology by exploring the pre-movement gating (attenuation) of somatosensory evoked potentials (SEPs). METHODS: We examined 42 patients (aged 9-48 years), 4 of whom underwent follow-up assessment, along with 19 healthy controls. We defined patients with only simple tics as TS-S and patients with complex tics as TS-C. Pre-movement gating of SEPs was assessed using a previously described method. Frontal N30 (FrN30) amplitudes were compared between pre-movement and resting states. The gating ratio of pre-movement/resting amplitude of the FrN30 component was assessed: the larger the ratio, the less the gating. RESULTS: The gating ratio for TS-C patients was larger than that of TS-S patients and healthy controls, but a statistical difference between TS-S and TS-C appeared after 15 years and over (p < 0.001). There were no significant differences in the gating ratio between TS-S patients and healthy controls. The gating ratio was related to the severity of OCD (p < 0.05). CONCLUSION: Sensorimotor processing was preserved for simple tics but impaired in complex tics, specifically after middle adolescence. Our study supports an age-dependent dysfunction of both motor and non-motor cortico-striato-thalamo-cortical circuits in complex tics. SEP gating seems promising as a tool for assessing age-dependent sensorimotor disintegration in TS.