Differences in Tic Severity Among Adolescent Girls and Boys with Tourette Syndrome During the Pandemic.

Limited data are available regarding the impact of the coronavirus disease 2019 (COVID-19) pandemic on adolescents with Tourette syndrome (TS). We sought to compare sex differences in tic severity experienced by adolescents before and during the COVID-19 pandemic. We extracted from the electronic health record and retrospectively reviewed Yale Global Tic Severity Scores (YGTSS) from adolescents (ages 13 through 17) with TS presenting to our clinic before (36 months) and during (24 months) the pandemic. A total of 373 unique adolescent patient encounters (prepandemic: 199; pandemic: 173) were identified. Compared with prepandemic, girls accounted for a significantly greater proportion of visits during the pandemic (p < 0.001). Prepandemic, tic severity did not differ between girls and boys. During the pandemic, compared with girls, boys had less clinically severe tics (p = 0.003). During the pandemic, older girls, but not boys, had less clinically severe tics (ρ =- 0.32, p = 0.003). These findings provide evidence that, regarding tic severity assessed with YGTSS, the experiences of adolescent girls and boys with TS have differed during the pandemic.

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.

Spectrum of Pediatric to Early Adulthood POLR3A-Associated Movement Disorders.

Background: POLR3A pathogenic variants are associated with hypomyelination, hypodontia, hypogonadism, and movement disorders. Cases: We describe the range of movement disorders seen in six patients (four female, two male) with POLR3A variants [three novel (c.2214del, c.3775G>A, c.3905G>T) and six previously reported (c.760C>T, c.1771-7C>G, c.1909+22G>A, c.2005C>T, c.2422C>T, c.3337-11T>C)]. Patient 1 presented with a neonatal progeroid syndrome and developed parkinsonism, dystonia, ataxia, and spasticity. Patient 2 presented with infant-onset rapidly progressive chorea, and dystonia. Three patients (patients 3, 5, 6) presented predominantly with ataxia in combination with spasticity and dystonia. Patient 4 developed segmental dystonia during adolescence and ataxia in early adulthood. Four patients had vertical gaze impairment. The most common brain MRI abnormality was T2-weighted/FLAIR hyperintensity of the superior cerebellar peduncles and midbrain. Conclusion: POLR3A-related disorders exhibit significant phenotypic pleomorphism. Vertical gaze dysfunction and T2-weighted/FLAIR hyperintensity of the superior cerebellar peduncles and midbrain may be useful signs suggestive of this condition.

From urges to tics in children with Tourette syndrome: associations with supplementary motor area GABA and right motor cortex physiology.

Tourette syndrome (TS) is a childhood-onset disorder in which tics are often preceded by premonitory sensory urges. More severe urges correlate with worse tics and can render behavioral therapies less effective. The supplementary motor area (SMA) is a prefrontal region believed to influence tic performance. To determine whether cortical physiological properties correlate with urges and tics, we evaluated, in 8-12-year-old right-handed TS children (n = 17), correlations of urge and tic severity scores and compared both to cortical excitability (CE) and short- and long-interval cortical inhibition (SICI and LICI) in both left and right M1. We also modeled these M1 transcranial magnetic stimulation measures with SMA gamma-amino butyric acid (GABA) levels in TS and typically developing control children (n = 16). Urge intensity correlated strongly with tic scores. More severe urges correlated with lower CE and less LICI in both right and left M1. Unexpectedly, in right M1, lower CE and less LICI correlated with less severe tics. We found that SMA GABA modulation of right, but not left, M1 CE and LICI differed in TS. We conclude that in young children with TS, lower right M1 CE and LICI, modulated by SMA GABA, may reflect compensatory mechanisms to diminish tics in response to premonitory urges.

EEG Correlates of Active Stopping and Preparation for Stopping in Chronic Tic Disorder.

Motor inhibition is an important cognitive process involved in tic suppression. As the right frontal lobe contains important inhibitory network nodes, we characterized right superior, middle, and inferior frontal gyral (RSFG, RMFG, RIFG) event-related oscillations during motor inhibition in youth with chronic tic disorders (CTD) versus controls. Fourteen children with CTD and 13 controls (10-17 years old) completed an anticipated-response stop signal task while dense-array electroencephalography was recorded. Between-group differences in spectral power changes (3-50 Hz) were explored after source localization and multiple comparisons correction. Two epochs within the stop signal task were studied: (1) preparatory phase early in the trial before motor execution/inhibition and (2) active inhibition phase after stop signal presentation. Correlation analyses between electrophysiologic data and clinical rating scales for tic, obsessive-compulsive symptoms, and inattention/hyperactivity were performed. There were no behavioral or electrophysiological differences during active stopping. During stop preparation, CTD participants showed greater event-related desynchronization (ERD) in the RSFG (γ-band), RMFG (ß, γ-bands), and RIFG (θ, α, ß, γ-bands). Higher RSFG γ-ERD correlated with lower tic severity (r = 0.66, p = 0.04). Our findings suggest RSFG γ-ERD may represent a mechanism that allows CTD patients to keep tics under control and achieve behavioral performance similar to peers.

Effect of dopaminergic medication on postural sway in advanced Parkinson's disease.

BACKGROUND: The effect of dopaminergic therapy on balance in Parkinson's disease (PD) remains unclear, including previous studies that excluded the effect of dyskinesias or other involuntary movements on postural sway. Additionally, medication's effects may differ between fallers and non-fallers. In this study, the authors quantify the effect of dopaminergic medication on postural balance (sway) in advanced PD, with and without dyskinesias, and consider the patient's history of falls. METHODS: In 24 patients with advanced idiopathic PD, postural balance was measured using a strain-gage force platform. Before and after taking dopaminergic medication, the patient's postural sway was measured at 30-s intervals to determine sway length (SL) and sway area (SA). Data analysis included the presence of dyskinesias during "ON" medication condition and history of previous falls. RESULTS: No significant changes occurred in SL or SA with dopaminergic treatment for fallers without dyskinesias or non-fallers with dyskinesias. However, after dopaminergic treatment, SL and SA were 37.8 and 45% lower, respectively, in non-fallers without dyskinesias (indicating better balance) and were 87.4 and 162.8% higher, respectively, in fallers with dyskinesias (indicating poorer balance). In the ON-medication condition, SL and SA were larger in patients with dyskinesias when compared with patients without dyskinesias; SL was larger in fallers than non-fallers in both groups with or without dyskinesias. CONCLUSION: Dopaminergic medication effects on postural sway could be a predictive factor for fall risk in PD patients with and without dyskinesias: specifically, decreased sway could indicate minimal fall risk whereas no change or increased postural sway could indicate a high risk.