Functional Tic-like Behaviors: From the COVID-19 Pandemic to the Post-Pandemic Era.

During the COVID-19 pandemic, there have been multiple reports about an unforeseen surge in adolescents and young adults exhibiting sudden onset functional tic-like behaviors. This phenomenon has been mainly associated with the female gender and occasionally after exposure to social media content featuring similar patterns of functional tic-like behaviors. A significant portion of these individuals have been directed to specialist clinics for movement disorders with initial misdiagnoses of late-onset refractory Tourette syndrome. Distinguishing between rapid onset functional tic-like behaviors and neurodevelopmental tics as part of Tourette syndrome can be challenging; however, the differential diagnosis is facilitated by focusing on specific clinical and demographic factors, which we have explored in a systematic literature review. Compared to neurodevelopmental tics, functional tic-like behaviors typically present with a more abrupt and intense manifestation of symptoms, onset at a later age, higher prevalence among females, inability to suppress tics, coexisting anxiety and depression, and sometimes a history of exposure to social media content portraying tic-like behaviors of a similar nature. This novel manifestation of a functional neurological disorder may thus be viewed as an emerging neuropsychiatric condition potentially triggered/exacerbated by the psychosocial repercussions of the COVID-19 crisis.

Gilles de la Tourette syndrome as a rare co-morbidity of Klinefelter syndrome.

BACKGROUND: Klinefelter syndrome (47, XXY) is the most common sex chromosome aneuploidy. In addition to male hypergonadotropic hypogonadism, a wide range of neurodevelopmental disorders, anxiety and affective symptoms have been reported in a substantial proportion of cases. CASE DESCRIPTION: We document the rare case of a 43-year-old man diagnosed with Klinefelter syndrome and co-morbid Gilles de la Tourette syndrome. He presented with multiple motor and vocal tics since adolescence, as well as anxiety and affective symptoms as his main tic-exacerbating factors. Tic severity was rated as marked (Yale Global Tic Severity Scale score of 78/100), and recommendations for the treatment of both tics and psychiatric co-morbidities were formulated. DISCUSSION: Neurodevelopmental tics in the context of Klinefelter syndrome have been previously documented in three cases only. Gilles de la Tourette syndrome is 3-4 times more common in males than females and its etiological factors include multiple genetic components (genetic heterogeneity). Our case report widens the spectrum of neurodevelopmental disorders observed in the context of Klinefelter syndrome and contributes to genetic research on the role of the X chromosome in the pathophysiology of tic disorders.

Functional tics: Expanding the phenotypes of functional movement disorders?

BACKGROUND AND PURPOSE: Until the outbreak reported during the COVID-19 pandemic, functional tics were considered to be a relatively rare clinical phenotype, as opposed to other functional movement disorders such as functional tremor and dystonia. To better characterize this phenotype, we compared the demographic and clinical characteristics of patients who developed functional tics during the pandemic and those of patients with other functional movement disorders. METHODS: Data from 110 patients were collected at the same neuropsychiatry centre: 66 consecutive patients who developed functional tics without other functional motor symptoms or neurodevelopmental tics and 44 patients with a mix of functional dystonia, tremor, gait, and myoclonus. RESULTS: Both groups were characterized by female sex preponderance (70%-80%) and (sub)acute onset of functional symptoms (~80%). However, patients with functional tics had a significantly earlier age at onset of functional symptoms (21 vs. 39 years). Exposure to relevant social media content was reported by almost half of the patients with functional tics, but by none of the patients with other functional movement disorders. Comorbidity profiles were similar, with relatively high rates of anxiety/affective symptoms and other functional neurological symptoms (nonepileptic attacks). CONCLUSIONS: Patients who developed functional tics during the pandemic represent a phenotypic variant of the wider group of patients with functional movement disorders, associated with younger age at onset and influenced by pandemic-related factors, including increased exposure to specific social media content. Diagnostic protocols and treatment interventions should be tailored to address the specific features of this newly defined phenotype.

The contribution of sleep and co-occurring neurodevelopmental conditions to quality of life in children with epilepsy.

BACKGROUND: Health-related quality of life (HRQOL) in children with epilepsy (CWE) is multifactorial and can be affected not only by epilepsy-specific variables but also co-occurring conditions such as sleep disturbances, autism, and attention deficit hyperactivity disorder (ADHD). While highly prevalent in CWE, these conditions are underdiagnosed despite having a significant impact on HRQOL. Sleep problems have a complex relationship with epilepsy and neurodevelopmental characteristics. However, little is known about how these issues interact and contribute to HRQOL. OBJECTIVES: The current study aims to explore the relationship between sleep and neurodevelopmental characteristics on HRQOL in CWE. METHODS: 36 CWE aged 4-16 years old were recruited from two hospitals and asked to wear an actiwatch for a period of 14 days and caregivers completed a series of questionnaires assessing co-occurrences and epilepsy-specific variables. RESULTS: A high proportion of CWE (78.13%) presented significant sleep problems. Informant-reported sleep problems were significantly predictive of HRQOL above seizure severity and the number of antiseizure medications. Interestingly, informant-reported sleep problems were no longer significantly predictive of HRQOL when neurodevelopmental characteristics were considered, indicating a possible mediating effect. Similarly, actigraphy-defined sleep (variability in sleep onset latency) displayed a similar effect but only for ADHD characteristics, whereas autistic characteristics and variability in sleep onset latency continued to exert an individual effect on HRQOL. CONCLUSION: These data from our study shed light on the complicated relationship between sleep, neurodevelopmental characteristics and epilepsy. Findings suggest that the impact of sleep on HRQOL in CWE is possibly mediated by neurodevelopmental characteristics. Furthermore, the impact this triangular relationship exerts on HRQOL is dependent on the type of tool used to measure sleep. These findings highlight the importance of a multidisciplinary approach to epilepsy management.

Neurodevelopmental versus functional tics: A controlled study.

BACKGROUND: An unprecedented increase in newly developed functional tics, mainly in young females, has been reported during the COVID-19 pandemic. We set out to complement existing case series with the largest controlled study to date on the clinical phenomenology of functional tics versus neurodevelopmental tics. METHODS: Data from 166 patients were collected at a specialist clinic for tic disorders during a three-year period overlapping with the COVID-19 pandemic (2020-2023). We compared the clinical features of patients who developed functional tics during the COVID-19 pandemic (N = 83) to patients with Tourette syndrome matched for age and gender (N = 83). RESULTS: Female adolescents and young adults accounted for 86% of the clinical sample of patients with functional tics, who were less likely to report a family history of tic disorders than their matched controls with Tourette syndrome. Co-morbidity profiles were significantly different: anxiety and other functional neurological disorders were more strongly associated with functional tics, whereas attention-deficit and hyperactivity disorder and tic-related obsessive-compulsive behaviors co-occurred more frequently with neurodevelopmental tics. Overall, absence of tic-related obsessive-compulsive behaviors (t = 8.096; p < 0.001) and absence of a family history of tics (t = 5.111; p < 0.001) were the strongest predictors of the diagnosis of functional tics. Compared to neurodevelopmental tics, functional tics were more likely to present acutely/subacutely at a later age (21 versus 7 years), without a clear rostro-caudal progression. Coprophenomena, self-injurious behaviors, and complex clinical manifestations such as blocking tics, throwing tics, and tic attacks, were all over-represented in the functional group. CONCLUSIONS: Our findings provide robust confirmation of both patient-related variables and tic characteristics contributing to the differential diagnosis between functional tics developed during the pandemic and neurodevelopmental tics reported by patients with Tourette syndrome.

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 Western origins of mindfulness therapy in ancient Rome.

Stoic philosophy has multiple parallels with cognitive behavioural therapy interventions. In their ancient texts, the Roman Stoics present a set of theoretical principles and behavioural strategies that are directly relevant to the clinical care of patients with a wide range of neuropsychiatric conditions. Mindfulness is a key component of the 'third wave' of modern psychotherapy that closely resembles the ancient Stoic practice of attention or 'concentration on the present moment'. Stoic mindfulness draws attention to one of the main principles driving both Stoicism and modern psychotherapy: the assumption that cognitive activity (reasoning) mediates emotions and behaviours. This principle can be traced back to Epictetus' Enchiridion, where he recognises that 'men are disturbed not by things, but by the views which they take of things'. It has been shown that cognitive behavioural therapies and mindfulness-based interventions directed at patients with neuropsychiatric disorders were originally developed as Stoic-inspired treatment interventions. Both Albert Ellis and Aaron Beck (the founders of rational emotive behaviour therapy and cognitive behavioural therapy, respectively) explicitly acknowledged the role of Stoicism as the philosophical precursor of their treatment approaches. The effective implementation of evidence-based guidelines would benefit from an increased awareness of the influence of the Stoic tradition of philosophical therapy on the treatment approaches currently in use in neuropsychiatry.

Feasibility of non-invasive neuro-monitoring during extracorporeal membrane oxygenation in children.

INTRODUCTION: Detection of neurological complications during extracorporeal membrane oxygenation (ECMO) may be enhanced with non-invasive neuro-monitoring. We investigated the feasibility of non-invasive neuro-monitoring in a paediatric intensive care (PIC) setting. METHODS: In a single centre, prospective cohort study we assessed feasibility of recruitment, and neuro-monitoring via somatosensory evoked potentials (SSEP), electroencephalography (EEG) and near infrared spectroscopy (NIRS) during venoarterial (VA) ECMO in paediatric patients (0-15 years). Measures were obtained within 24h of cannulation, during an intermediate period, and finally at decannulation or echo stress testing. SSEP/EEG/NIRS measures were correlated with neuro-radiology findings, and clinical outcome assessed via the Pediatric cerebral performance category (PCPC) scale 30 days post ECMO cannulation. RESULTS: We recruited 14/20 (70%) eligible patients (median age: 9 months; IQR:4-54, 57% male) over an 18-month period, resulting in a total of 42 possible SSEP/EEG/NIRS measurements. Of these, 32/42 (76%) were completed. Missed recordings were due to lack of access/consent within 24 h of cannulation (5/42, 12%) or PIC death/discharge (5/42, 12%). In each patient, the majority of SSEP (8/14, 57%), EEG (8/14, 57%) and NIRS (11/14, 79%) test results were within normal limits. All patients with abnormal neuroradiology (4/10, 40%), and 6/7 (86%) with poor outcome (PCPC ≥4) developed indirect SSEP, EEG or NIRS measures of neurological complications prior to decannulation. No study-related adverse events or neuro-monitoring data interpreting issues were experienced. CONCLUSION: Non-invasive neuro-monitoring (SSEP/EEG/NIRS) during ECMO is feasible and may provide early indication of neurological complications in this high-risk population.

A Hypothalamic Mechanism Regulates the Duration of a Migraine Attack: Insights from Microstructural and Temporal Complexity of Cortical Functional Networks Analysis.

The role of the hypothalamus and the limbic system at the onset of a migraine attack has recently received significant interest. We analyzed diffusion tensor imaging (DTI) parameters of the entire hypothalamus and its subregions in 15 patients during a spontaneous migraine attack and in 20 control subjects. We also estimated the non-linear measure resting-state functional MRI BOLD signal's complexity using Higuchi fractal dimension (FD) and correlated DTI/fMRI findings with patients' clinical characteristics. In comparison with healthy controls, patients had significantly altered diffusivity metrics within the hypothalamus, mainly in posterior ROIs, and higher FD values in the salience network (SN). We observed a positive correlation of the hypothalamic axial diffusivity with migraine severity and FD of SN. DTI metrics of bilateral anterior hypothalamus positively correlated with the mean attack duration. Our results show plastic structural changes in the hypothalamus related to the attacks severity and the functional connectivity of the SN involved in the multidimensional neurocognitive processing of pain. Plastic changes to the hypothalamus may play a role in modulating the duration of the attack.

Functional hemispherotomy for epilepsy in the very young.

OBJECTIVE: Epilepsy is one of the most common neurological disorders in children. Among very young children, one-third are resistant to medical treatment, and lack of effective treatment may result in adverse outcomes. Although functional hemispherotomy is an established treatment for epilepsy, its outcome in the very young child has not been widely reported. In this study the authors investigated seizure and developmental results after hemispherotomy in children younger than 3 years. METHODS: The authors reviewed a prospective database of all children younger than 3 years with medically intractable epilepsy who underwent functional hemispherotomy at the authors' institution during the period between 2012 and 2020. Demographic data, epilepsy history, underlying etiology, operative and transfusion details, and seizure and developmental outcomes were analyzed. RESULTS: Twelve patients were included in this study. The mean age (± SD) at seizure onset was 3 ± 2.6 months and at surgery was 1.3 ± 0.77 years, with a mean follow-up of 4 years. Diagnoses included hemimegalencephaly (n = 5), hemidysplasia (n = 2), hypoxic/hemorrhagic (n = 2), traumatic (n = 1), Sturge-Weber syndrome (n = 1), and mild hemispheric structural abnormality with EEG/PET correlates (n = 1). Eleven patients achieved an Engel class I outcome, and 1 patient achieved Engel class IV at last follow-up. No deaths, infections, cerebrovascular events, or unexpected long-term neurological deficits were recorded. All children progressed neurodevelopmentally following surgery, but their developmental levels remained behind their chronological age, with an overall mean composite Vineland Adaptive Behavior Scale score of 58 (normal: 86-114, low: < 70). One patient required insertion of a subdural peritoneal shunt, 1 patient required dural repair for a CSF fluid leak, and 1 patient required aspiration of a pseudomeningocele. In 2 patients, both of whom weighed less than 5.7 kg, the first operation was incomplete due to blood loss. CONCLUSIONS: Hemispherotomy in children younger than 3 years offers excellent seizure control and an acceptable risk-to-benefit ratio in well-selected patients. Families of children weighing less than 6 kg should be counseled regarding the possibility of staged surgery. Postoperatively, children continue to make appropriate, despite delayed, developmental progress.

Perioperative electroencephalography in cardiac surgery with hypothermic circulatory arrest: a narrative review.

OBJECTIVES: Cardiac surgery with hypothermic circulatory arrest (HCA) is associated with neurological morbidity of variable severity and electroencephalography (EEG) is a sensitive proxy measure of brain injury. We conducted a narrative review of the literature to evaluate the role of perioperative EEG monitoring in cardiac surgery involving HCA. METHODS: Medline, Embase, Central and LILACS databases were searched to identify studies utilizing perioperative EEG during surgery with HCA in all age groups, published since 1985 in any language. We aimed to compare EEG use with no use but due to the lack of comparative studies, we performed a narrative review of its utility. Two or more reviewers independently screened studies for eligibility and extracted data. RESULTS: Fourty single-centre studies with a total of 3287 patients undergoing surgery were identified. Most were observational cohort studies (34, 85%) with only 1 directly comparing EEG use with no use. EEG continuity (18, 45%), seizures (15, 38%) and electrocerebral inactivity prior to circulatory arrest (15, 38%) were used to detect, monitor, prevent and prognose neurological injury. Neurological dysfunction was reported in almost all studies and occurred in 0-21% of patients. However, the heterogeneity of reported clinical and EEG outcome measures prevented meta-analysis. CONCLUSIONS: EEG is used to detect cortical ischaemia and seizures and predict neurological abnormalities and may guide intraoperative cerebral protection. However, there is a lack of comparative data demonstrating the benefit of perioperative EEG monitoring. Use of a standardized methodology for performing EEG and reporting outcome metrics would facilitate the conduct of high-quality clinical trials.

The AMPA receptor antagonist perampanel suppresses epileptic activity in human focal cortical dysplasia.

Focal cortical dysplasia (FCD) is one of the most common malformations causing refractory epilepsy. Dysregulation of glutamatergic systems plays a critical role in the hyperexcitability of dysplastic neurons in FCD lesions. The pharmacoresistant nature of epilepsy associated with FCD may be due to a lack of well-tolerated and precise antiepileptic drugs that can target glutamate receptors. Here, for the first time in human FCD brain slices, we show that the established, noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, perampanel has potent antiepileptic action. Moreover, we demonstrate that this effect is due to a reduction in burst firing behavior in human FCD microcircuits. These data support a potential role for the treatment of refractory epilepsy associated with FCD in human patients.

Structural-covariance networks identify topology-based cortical-thickness changes in children with persistent executive function impairments after traumatic brain injury.

Paediatric traumatic brain injury (pTBI) results in inconsistent changes to regional morphometry of the brain across studies. Structural-covariance networks represent the degree to which the morphology (typically cortical-thickness) of cortical-regions co-varies with other regions, driven by both biological and developmental factors. Understanding how heterogeneous regional changes may influence wider cortical network organization may more appropriately capture prognostic information in terms of long term outcome following a pTBI. The current study aimed to investigate the relationships between cortical organisation as measured by structural-covariance, and long-term cognitive impairment following pTBI. T1-weighted magnetic resonance imaging (MRI) from n = 83 pTBI patients and 33 typically developing controls underwent 3D-tissue segmentation using Freesurfer to estimate cortical-thickness across 68 cortical ROIs. Structural-covariance between regions was estimated using Pearson's correlations between cortical-thickness measures across 68 regions-of-interest (ROIs), generating a group-level 68 × 68 adjacency matrix for patients and controls. We grouped a subset of patients who underwent executive function testing at 2-years post-injury using a neuropsychological impairment (NPI) rule, defining impaired- and non-impaired subgroups. Despite finding no significant reductions in regional cortical-thickness between the control and pTBI groups, we found specific reductions in graph-level strength of the structural covariance graph only between controls and the pTBI group with executive function (EF) impairment. Node-level differences in strength for this group were primarily found in frontal regions. We also investigated whether the top n nodes in terms of effect-size of cortical-thickness reductions were nodes that had significantly greater strength in the typically developing brain than n randomly selected regions. We found that acute cortical-thickness reductions post-pTBI are loaded onto regions typically high in structural covariance. This association was found in those patients with persistent EF impairment at 2-years post-injury, but not in those for whom these abilities were spared. This study posits that the topography of post-injury cortical-thickness reductions in regions that are central to the typical structural-covariance topology of the brain, can explain which patients have poor EF at follow-up.

Hypothalamic structural integrity and temporal complexity of cortical information processing at rest in migraine without aura patients between attacks.

The hypothalamus has been attributed an important role during the premonitory phase of a migraine attack. Less is known about the role played by the hypothalamus in the interictal period and its relationship with the putative neurocognitive networks previously identified in the pathophysiology of migraine. Our aim was to test whether the hypothalamic microstructure would be altered during the interictal period and whether this co-existed with aberrant connectivity at cortical level. We collected multimodal MRI data from 20 untreated patients with migraine without aura between attacks (MO) and 20 healthy controls (HC) and studied fractional anisotropy, mean (MD), radial (RD), and axial diffusivity of the hypothalamus ROI as a whole from diffusion tensor imaging (DTI). Moreover, we performed an exploratory analysis of the same DTI metrics separately for the anterior and posterior hypothalamic ROIs bilaterally. From resting-state functional MRI, we estimated the Higuchi's fractal dimension (FD), an index of temporal complexity sensible to describe non-periodic patterns characterizing BOLD signature. Finally, we correlated neuroimaging findings with migraine clinical features. In comparison to HC, MO had significantly higher MD, AD, and RD values within the hypothalamus. These findings were confirmed also in the exploratory analysis on the sub-regions of the hypothalamus bilaterally, with the addition of lower FA values on the posterior ROIs. Patients showed higher FD values within the salience network (SN) and the cerebellum, and lower FD values within the primary visual (PV) network compared to HC. We found a positive correlation between cerebellar and SN FD values and severity of migraine. Our findings of hypothalamic abnormalities between migraine attacks may form part of the neuroanatomical substrate that predisposes the onset of the prodromal phase and, therefore, the initiation of an attack. The peculiar fractal dimensionality we found in PV, SN, and cerebellum may be interpreted as an expression of abnormal efficiency demand of brain networks devoted to the integration of sensory, emotional, and cognitive information related to the severity of migraine.

MEG detection of high frequency oscillations and intracranial-EEG validation in pediatric epilepsy surgery.

OBJECTIVE: To assess the feasibility of automatically detecting high frequency oscillations (HFOs) in magnetoencephalography (MEG) recordings in a group of ten paediatric epilepsy surgery patients who had undergone intracranial electroencephalography (iEEG). METHODS: A beamforming source-analysis method was used to construct virtual sensors and an automatic algorithm was applied to detect HFOs (80-250 Hz). We evaluated the concordance of MEG findings with the sources of iEEG HFOs, the clinically defined seizure onset zone (SOZ), the location of resected brain structures, and with post-operative outcome. RESULTS: In 8/9 patients there was good concordance between the sources of MEG HFOs and iEEG HFOs and the SOZ. Significantly more HFOs were detected in iEEG relative to MEG t(71) = 2.85, p < .05. There was good concordance between sources of MEG HFOs and the resected area in patients with good and poor outcome, however HFOs were also detected outside of the resected area in patients with poor outcome. CONCLUSION: Our findings demonstrate the feasibility of automatically detecting HFOs non-invasively in MEG recordings in paediatric patients, and confirm compatibility of results with invasive recordings. SIGNIFICANCE: This approach provides support for the non-invasive detection of HFOs to aid surgical planning and potentially reduce the need for invasive monitoring, which is pertinent to paediatric patients.

Pediatric stereo-electroencephalography: effects of robot assistance and other variables on seizure outcome and complications.

OBJECTIVE: The safety of stereo-electroencephalography (SEEG) has been investigated; however, most studies have not differentiated pediatric and adult populations, which have different anatomy and physiology. The purpose of this study was to assess SEEG safety in the pediatric setting, focusing on surgical complications and the identification of patient and surgical risk factors, if any. The authors also aimed to determine whether robot assistance in SEEG was associated with a change in practice, surgical parameters, and clinical outcomes. METHODS: The authors retrospectively studied all SEEG cases performed in their department from December 2014 to March 2020. They analyzed both demographic and surgical variables and noted the types of surgery-related complications and their management. They also studied the clinical outcomes of a subset of the patients in relation to robot-assisted and non-robot-assisted SEEG. RESULTS: Sixty-three children had undergone 64 SEEG procedures. Girls were on average 3 years younger than the boys (mean age 11.1 vs 14.1 years, p < 0.01). The overall complication rate was 6.3%, and the complication rate for patients with left-sided electrodes was higher than that for patients with right-sided electrodes (11.1% vs 3.3%), although the difference between the two groups was not statistically significant. The duration of recording was positively correlated to the number of implanted electrodes (r = 0.296, p < 0.05). Robot assistance was associated with a higher number of implanted electrodes (mean 12.6 vs 7.6 electrodes, p < 0.0001). Robot-assisted implantations were more accurate, with a mean error of 1.51 mm at the target compared to 2.98 mm in nonrobot implantations (p < 0.001). Clinical outcomes were assessed in the first 32 patients treated (16 in the nonrobot group and 16 in the robot group), 23 of whom proceeded to further resective surgery. The children who had undergone robot-assisted SEEG had better eventual seizure control following subsequent epilepsy surgery. Of the children who had undergone resective epilepsy surgery, 42% (5/12) in the nonrobot group and 82% (9/11) in the robot group obtained an Engel class IA outcome at 1 year (χ2 = 3.885, p = 0.049). Based on Kaplan-Meier survival analysis, the robot group had a higher seizure-free rate than the nonrobot group at 30 months postoperation (7/11 vs 2/12, p = 0.063). Two complications, whose causes were attributed to the implantation and head-bandaging steps, required surgical intervention. All complications were either transient or reversible. CONCLUSIONS: This is the largest single-center, exclusively pediatric SEEG series that includes robot assistance so far. SEEG complications are uncommon and usually transient or treatable. Robot assistance enabled implantation of more electrodes and improved epilepsy surgery outcomes, as compared to those in the non-robot-assisted cases.

Reliability in the assessment of paediatric somatosensory evoked potentials post cardiac arrest.

OBJECTIVE: To measure inter- and intra-rater agreement in the interpretation of cortical somatosensory evoked potential (SSEP) components following paediatric cardiac arrest (CA) in multi-professional neurophysiology teams. METHODS: Thirteen professionals blinded to patient outcome interpreted 96 SSEPs in paediatric patients 24-/48-/72-hours following CA. Of these, 34 were duplicates used to assess intra-rater agreement. Consistent interpretations (absent/present/indeterminate) between scientists (who record/identify SSEP components) and neurophysiologists (who provide prognostic SSEP interpretation) were expressed as percentages. Rates of agreement were calculated using Fleiss' kappa coefficient (K). RESULTS: Unanimous agreement between professionals was present in 40% (95%CI: 28-54%) of the interpreted SSEPs, with a K value of 0.62 (95%CI: 0.55-0.70) based on average agreement. Agreement was similar between neurophysiologists (K = 0.67; 95%CI: 0.57-0.77) and scientists (K = 0.62; 95%CI: 0.54-0.70) but lower in patients < 2 years old (K = 0.23; 95%CI: 0.14-0.33) and in those with poor outcome (K = 0.21; 95%CI: 0.07-0.35). No SSEP was unanimously interpreted as absent and 92% (95%CI: 89-95%) of duplicate SSEPs were interpreted consistently. CONCLUSION: Despite substantial agreement when interpreting prognostic SSEPs, this was significantly lower in children with poor outcome and of younger age. SIGNIFICANCE: Clinicians using SSEPs in the intensive care unit should be aware of the inter-rater variability when interpreting SSEPs as absent.

Sleep disruption in children and adolescents with epilepsy: A systematic review and meta-analysis.

This systematic review and meta-analysis aims to assess and quantify putative differences in sleep architecture, sleep efficiency, sleep timing and broadly-defined sleep difficulties between children with and without epilepsy. Databases were searched systematically, and studies identified in PubMed, EMBASE, PsychINFO and Medline. The meta-analysis included 19 studies comparing a total of 901 children with epilepsy to 1470 healthy children. Relative to healthy children, children with epilepsy experienced reduced sleep time, sleeping on average 34 mins less across self-report, actigraphy, 24-h video-EEG and polysomnography measures. They had more sleep difficulties specifically in the domains of night waking, parasomnias and sleep disordered breathing. The analysis also revealed a significantly increased percentage of N2 sleep and decreased sleep efficiency in children with epilepsy compared to healthy children. These results illustrate that children with epilepsy are vulnerable to more sleep difficulties compared to healthy children. This suggests that screening for sleep difficulties should be an integral part in a diagnosis of epilepsy to ensure that clinically relevant sleep difficulties are identified and treated. Such an approach may ultimately aid in the development of treatment strategies which can contribute to improvements in both developmental and diagnostic outcomes for children with epilepsy.

Peripheral nerve electrophysiology studies in relation to fatigue in patients with chronic inflammatory demyelinating polyneuropathy.

OBJECTIVE: To explore the relationship between fatigue, standard electrophysiological parameters and number and size of functioning motor units in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Experienced fatigue was assessed using the linearly-weighted, modified Rasch-built fatigue severity scale (R-FSS) and the multidimensional Checklist of Individual Strength (CIS). Averaged electrophysiology values were calculated from multiple nerves. Motor Unit Number Index (MUNIX) technique was utilised to assess motor unit function. Assessments were repeated in 15 patients receiving regular intravenous immunoglobulin therapy, with changes in parameters calculated. RESULTS: R-FSS and CIS scores did not correlate MUNIX or MUSIX sum scores from 3 different muscles. Inverse correlation was observed only between distal CMAP area and R-FSS but not CIS scores. However, changes in distal CMAP area and R-FSS scores on repeat assessment were not correlated. CONCLUSIONS: Experienced fatigue does not appear to correlate with loss of functioning motor units in patients with CIDP. Changes in experienced fatigue on repeat assessment did not correlate with changes in any of the electrophysiological parameters, suggesting fatigue experienced in CIDP is not strongly correlated with peripheral nerve dysfunction. SIGNIFICANCE: Nerve conduction studies and MUNIX values do not appear to be useful surrogate markers for fatigue in CIDP.

Light-emitting-diode and Grass PS 33 xenon lamp photic stimulators are equivalent in the assessment of photosensitivity: Clinical and research implications.

The assessment of the effect of photic stimulation is an integral component of an EEG exam and is especially important in patients referred for ascertained or suspected photosensitivity with or without a diagnosis of epilepsy. A positive test result relies on eliciting a specific abnormality defined as the "photoparoxysmal response". Reliability of this assessment is strongly influenced by technical and procedural variables, a critical one represented by the physical properties of the stimulators used. Established clinical norms are based on data acquired with the "gold-standard" Grass PS stimulators. These are no longer commercially available and have been replaced by stimulators using light emitting diode (LED) technology. To our knowledge no comparative study on their efficacy has been conducted. To address this gap, we recruited 39 patients aged 5-54 years, referred to two specialized centers with confirmed of suspected diagnosis of photosensitive epilepsy or generalized epilepsy with photosensitivity in a prospective randomized single-blind cross-over study to compare two commercially available LED-bases stimulation systems (FSA 10® and Lifeline® stimulators) against the Grass PS 33 xenon lamp device. Our findings indicate that the LED systems tested are equivalent to the Grass stimulator both in identifying the PPR in affected individuals.