Cognitive deficits and rehabilitation mechanisms in mild traumatic brain injury patients revealed by EEG connectivity markers

ObjectiveTo explore the multiple specific biomarkers and cognitive compensatory mechanisms of mild traumatic brain injury (mTBI) patients at recovery stage.MethodsThe experiment was performed in two sections. In Section I, using event-related potential, event-related oscillation and spatial phase-synchronization, we explored neural dynamics in 24 volunteered healthy controls (HC) and 38 patients at least 6 months post-mTBI (19 with epidural hematoma, EDH; 19 with subdural hematoma, SDH) during a Go/NoGo task. In Section II, according to the neuropsychological scales, patients were divided into sub-groups to assess these electroencephalography (EEG) indicators in identifying different rehabilitation outcomes of mTBI.ResultsIn Section I, mean amplitudes of NoGo-P3 and P3d were decreased in mTBI patients relative to HC, and NoGo-theta power in the non-injured hemisphere was decreased in SDH patients only. In Section II, patients with chronic neuropsychological defects exhibited more serious impairments of intra-hemispheric connectivity, whereas inter-hemispheric centro-parietal and frontal connectivity were enhanced in response to lesions.ConclusionsEEG distinguished mTBI patients from healthy controls, and estimated different rehabilitation outcomes of mTBI. The centro-parietal and frontal connectivity are the main compensatory mechanism for the recovery of mTBI patients.SignificanceEEG measurements and network connectivity can track recovery process and mechanism of mTBI.     

Expanding the clinical and EEG spectrum of CNKSR2-related encephalopathy with status epilepticus during slow sleep (ESES)

ObjectiveTo investigate the clinical and EEG features of Encephalopathy with Status Epilepticus during slow Sleep (ESES) related to CNKSR2 pathogenic variants.MethodsDetailed clinical history, repeated wakefulness/overnight sleep EEGs, brain MRI were collected in five patients, including one female, with CNKSR2-related ESES.ResultsNeurodevelopment in infancy was normal in two patients, delayed in three. Epilepsy onset (age range: 2–6 years) was associated with appearance or aggravation of cognitive impairment, language regression and/or behavioral disorders. Worsening of epilepsy and of cognitive/behavioral disturbances paralleled by enhancement of non-rapid eye movement (NREM) sleep-related, frontally predominant, EEG epileptic discharges [spike-wave-index (SWI): range 60–96%] was consistent with ESES. In three patients, episodes of absence status epilepticus or aggravation of atypical absences occurred, in this latter case associated with striking increment of awake SWI. Speech/oro-motor dyspraxia was diagnosed in four patients. In two patients, long-term follow-up showed epilepsy remission and persistence of mild/moderate cognitive disorders and behavioral disturbances into adulthood.ConclusionsNovel findings of our study are occurrence also in females, normal neurodevelopment before epilepsy onset, epilepsy aggravation associated with enhanced awake SWI, mild/moderate evolution in adulthood and language disorder due to speech/oro-motor dyspraxia.SignificanceOur findings expand the phenotypic spectrum of CNKSR2-related ESES.     

Patterns and correlates of sleep duration in the Southern cohort community study

ObjectiveTo investigate whether race (African American (AA) and white) is associated with sleep duration among adults from low socioeconomic (SES) strata and whether SES status, lifestyle behaviors, or health conditions are associated with sleep duration within race-sex groups.MethodsThis cross-sectional study includes 78,549 participants from the Southern Community Cohort Study (SCCS). Averaged daily sleep duration was assessed by weighted averages of self-reported sleep duration on weekdays and weekends. Adjusted odds ratios (ORs) of very short (<5 h/day), short (5–6 h/day), and long sleep (≥9 h/day) associated with pre-selected risk factors in each race-sex group were determined by multinomial logistic models.ResultsThe prevalence of very short and short sleep was similar among AAs (6.2% and 29.1%) and whites (6.5% and 29.1%). Long sleep was considerably more prevalent among AAs (19.3%) than whites (13.0%). Very short sleep was associated with lower education and family income, with stronger associations among whites. Higher physical activity levels significantly decreased odds for both very short (OR = 0.80) and long sleep (OR = 0.78). Smoking, alcohol use, and dietary intake were not associated with sleep duration. Regardless of race or sex, very short, short, and long sleep were significantly associated with self-reported health conditions, especially depression (ORs were 2.06, 1.33, and 1.38, respectively).ConclusionsSleep duration patterns differed between AAs and whites from the underrepresented SCCS population with low SES. Sleep duration was associated with several socioeconomic, health behaviors, and health conditions depending on race and sex.     

EEG functional connectivity contributes to outcome prediction of postanoxic coma

ObjectiveTo investigate the additional value of EEG functional connectivity features, in addition to non-coupling EEG features, for outcome prediction of comatose patients after cardiac arrest.MethodsProspective, multicenter cohort study. Coherence, phase locking value, and mutual information were calculated in 19-channel EEGs at 12 h, 24 h and 48 h after cardiac arrest. Three sets of machine learning classification models were trained and validated with functional connectivity, EEG non-coupling features, and a combination of these. Neurological outcome was assessed at six months and categorized as “good” (Cerebral Performance Category [CPC] 1–2) or “poor” (CPC 3–5).ResultsWe included 594 patients (46% good outcome). A sensitivity of 51% (95% CI: 34–56%) at 100% specificity in predicting poor outcome was achieved by the best functional connectivity-based classifier at 12 h after cardiac arrest, while the best non-coupling-based model reached a sensitivity of 32% (0–54%) at 100% specificity using data at 12 h and 48 h. Combination of both sets of features achieved a sensitivity of 73% (50–77%) at 100% specificity.ConclusionFunctional connectivity measures improve EEG based prediction models for poor outcome of postanoxic coma.SignificanceFunctional connectivity features derived from early EEG hold potential to improve outcome prediction of coma after cardiac arrest.     

Effect of partial and total sleep deprivation on serum testosterone in healthy males: a systematic review and meta-analysis

BackgroundCurrently, there is no consensus on the effect of sleep deprivation on male serum testosterone. This systematic review and meta-analysis aimed to determine the association between partial/total sleep deprivation and male serum testosterone level.MethodsThe literature related to sleep deprivation and male serum testosterone in the PubMed, Embase, and Cochrane Library databases were searched from their inception to July 15, 2021. Data were pooled using the Stata 15 software. The results were presented as standard mean differences (SMDs) with their 95% confidence intervals (CIs).ResultsEighteen studies involving 252 men were included in the systematic review and meta-analysis. The findings revealed that short-term partial sleep deprivation had no significant effect on male serum testosterone (SMD = −0.22; 95% CI: −0.5, 0.06; P = 0.13), while total sleep deprivation reduced the male testosterone levels (SMD = −0.64; 95% CI: −0.87, −0.42; P < 0.001). According to the intervention duration of total sleep deprivation, subgroup analysis was conducted by a fixed-effects model. The results revealed that the serum testosterone was significantly decreased after 24 h total sleep deprivation (SMD = − 0.67; 95% CI = − 0.93, −0.42, P < 0.001), as well as 40–48 h total sleep deprivation (SMD = − 0.74; 95% CI = − 1.22, −0.26, P = 0.002).ConclusionsThis meta-analysis revealed that total sleep deprivation (more than or equal to 24 h) reduces the male testosterone levels, while short-term partial sleep deprivation has no significant effect on male serum testosterone. Sleep duration plays a pivotal role in maintaining male serum testosterone levels.     

Automated electrical source imaging with scalp EEG to define the insular irritative zone: Comparison with simultaneous intracranial EEG

Objective To evaluate the accuracy of automated interictal low-density electrical source imaging (LD-ESI) to define the insular irritative zone (IZ) by comparing the simultaneous interictal ESI localization with the SEEG interictal activity.Methods Long-term simultaneous scalp electroencephalography (EEG) and stereo-EEG (SEEG) with at least one depth electrode exploring the operculo-insular region(s) were analyzed. Automated interictal ESI was performed on the scalp EEG using standardized low-resolution brain electromagnetic tomography (sLORETA) and individual head models. A two-step analysis was performed: i) sublobar concordance between cluster-based ESI localization and SEEG-based IZ; ii) time-locked ESI-/SEEG analysis. Diagnostic accuracy values were calculated using SEEG as reference standard. Subgroup analysis was carried out, based on the involvement of insular contacts in the seizure onset and patterns of insular interictal activity.Results Thirty patients were included in the study. ESI showed an overall accuracy of 53% (C.I. 29–76%). Sensitivity and specificity were calculated as 53% (C.I. 29–76%), 55% (C.I. 23–83%) respectively. Higher accuracy was found in patients with frequent and dominant interictal insular spikes.Conclusions LD-ESI defines with good accuracy the insular implication in the IZ, which is not possible with classical interictal scalp EEG interpretation.SignificanceAutomated LD-ESI may be a valuable additional tool to characterize the epileptogenic zone in epilepsies with suspected insular involvement.     

Impaired awareness in mesial temporal lobe epilepsy: Network analysis of foramen ovale and scalp EEG

ObjectiveWe use co-registration of foramen-ovale and scalp-EEG to investigate network alterations in temporal-lobe epilepsy during focal seizures without (aura) or with impairment of awareness (SIA).MethodsOne aura and one SIA were selected from six patients. Temporal dynamic among 4 epochs, as well as the differences between aura and SIA, were analyzed through partial directed coherence and graph theory-based indices of centrality.ResultsRegarding the auras temporal evolution, fronto-parietal (FP) regions showed decreased connectivity with respect to the interictal period, in both epileptogenic (EH) and non-epileptogenic hemisphere (nEH). During SIAs, temporal dynamic showed more changes than auras: centrality of mesial temporal (mT) regions changes during all conditions, and nEH FP centrality showed the same dynamic trend of the aura (decreased centrality), until the last epoch, close to the impaired awareness, when showed increased centrality. Comparing SIA with aura, in proximity of impaired awareness, increased centrality was found in all the regions, except in nEH mT.ConclusionsOur findings suggested that the impairment of awareness is related to network alterations occurring first in neocortical regions and when awareness is still retained.SignificanceThe analysis of ‘hub’ alteration can represent a suitable biomarker for scalp EEG-based prediction of awareness impairment.     

Family nurture intervention alters relationships between preterm infant EEG delta brush characteristics and term age EEG power

ObjectiveFamily Nurture Intervention (FNI) facilitates mother/infant emotional connection, improves neurodevelopmental outcomes and increases electroencephalogram (EEG) power at term age. Here we explored whether delta brushes (DB), early EEG bursts that shape brain development, are altered by FNI and mediate later effects of FNI on EEG.MethodsWe assessed DB characteristics in EEG data from a randomized controlled trial comparing infants with standard care (SC, n = 31) versus SC + FNI (n = 33) at ~35 and ~40 weeks GA.ResultsCompared to SC infants, FNI infant DB amplitude increased more from ~35 to ~40 weeks, and FNI infants had longer duration DBs. DB parameters (rate, amplitude, brush frequency) at ~35 weeks were correlated with power at ~40 weeks, but only in SC infants. FNI effects on DB parameters do not mediate FNI effects on EEG power or coherence at term.ConclusionsDBs are related to subsequent brain activity and FNI alters DB parameters. However, FNI’s effects on electrocortical activity at term age are not dependent on its earlier effects on DBs.SignificanceWhile early DBs can have important effects on later brain activity in preterm infants, facilitating emotional connection with FNI may allow brain maturation to be less dependent on early bursts.     

Sleep spindles in children with restless sleep disorder,restless legs syndrome and normal controls

ObjectiveTo analyze and identify differences in sleep spindles in children with restless sleep disorder (RSD), restless legs syndrome (RLS) and normal controls.MethodsPSG (polysomnography) from children with RSD, RLS and normal controls were analyzed. Sleep spindle activity was detected on one frontal and one central electrode, for each epoch of N2 and N3 sleep. Sleep spindle density, duration and intensity (density × duration) were then obtained and used for analysis.ResultsThirty-eight children with RSD, twenty-three children with RLS and twenty-nine controls were included. The duration of frontal spindles in sleep stage N2 was longer in children with RSD than in controls. Frontal spindle density and intensity tended to be increased in RSD children. No significant differences were found for central spindles.ConclusionChildren with RSD had longer frontal spindles. This finding may contribute to explain the occurrence of excessive movement activity during sleep and the presence of daytime symptoms.SignificanceRecent research has demonstrated that children with RSD have increased NREM instability and sympathetic activation during sleep. Analyzing sleep spindles in children with RSD in comparison with children with RLS and controls adds to our understanding of the pathophysiology or RSD and its effects on daytime impairment.     

A standardized nomenclature for spectrogram EEG patterns: Inter-rater agreement and correspondence with common intensive care unit EEG patterns

ObjectiveTo determine the inter-rater agreement (IRA) of a standardized nomenclature for EEG spectrogram patterns, and to estimate the probability distribution of ictal-interictal continuum (IIC) patterns vs. other EEG patterns within each category in this nomenclature.MethodsWe defined seven spectrogram categories: “Solid Flames”, “Irregular Flames”, “Broadband-monotonous”, “Narrowband-monotonous”, “Stripes”, “Low power”, and “Artifact”. Ten electroencephalographers scored 115 spectrograms and the corresponding raw EEG samples. Gwet's agreement coefficient was used to calculate IRA.ResultsSolid Flames represented seizures or IIC patterns 69.4% of the time. Irregular Flames represented seizures or IIC patterns 38.7% of the time. Broadband-monotonous primarily corresponded with seizures or IIC (54.3%) and Narrowband-monotonous with focal or generalized slowing (43.8%). Stripes were associated with burst-suppression (37.2%) and generalized suppression (34.4%). Low Power category was associated with generalized suppression (94%). There was “near perfect” agreement for Solid Flames (κ = 94.36), Low power (κ = 92.61), and Artifact (κ = 93.72). There was “substantial agreement” for all other categories (κ = 74.65–79.49).ConclusionsThis EEG spectrogram nomenclature has high IRA among electroencephalographers.SignificanceThe nomenclature can be a useful tool for EEG screening. Future studies are needed to determine if using this nomenclature shortens time to IIC identification, and how best to use it in practice to reduce time to intervention.     

Dynamic functional connectivity of the EEG in relation to outcome of postanoxic coma

ObjectiveEarly EEG contains reliable information for outcome prediction of comatose patients after cardiac arrest. We introduce dynamic functional connectivity measures and estimate additional predictive values.MethodsWe performed a prospective multicenter cohort study on continuous EEG for outcome prediction of comatose patients after cardiac arrest. We calculated Link Rates (LR) and Link Durations (LD) in the α, δ, and θ band, based on similarity of instantaneous frequencies in five-minute EEG epochs, hourly, during 3 days after cardiac arrest. We studied associations of LR and LD with good (Cerebral Performance Category (CPC) 1–2) or poor outcome (CPC 3–5) with univariate analyses. With random forest classification, we established EEG-based predictive models. We used receiver operating characteristics to estimate additional values of dynamic connectivity measures for outcome prediction.ResultsOf 683 patients, 369 (54%) had poor outcome. Patients with poor outcome had significantly lower LR and longer LD, with largest differences 12 h after cardiac arrest (LR_θ 1.87 vs. 1.95 Hz and LD_α 91 vs. 82 ms). Adding these measures to a model with classical EEG features increased sensitivity for reliable prediction of poor outcome from 34% to 38% at 12 h after cardiac arrest.ConclusionPoor outcome is associated with lower dynamics of connectivity after cardiac arrest.SignificanceDynamic functional connectivity analysis may improve EEG based outcome prediction.     

Fixation-related potentials in naming speed: A combined EEG and eye-tracking study on children with dyslexia

ObjectiveWe combined electroencephalography (EEG) and eye-tracking recordings to examine the underlying factors elicited during the serial Rapid-Automatized Naming (RAN) task that may differentiate between children with dyslexia (DYS) and chronological age controls (CAC).MethodsThirty children with DYS and 30 CAC (M_age = 9.79 years; age range 7.6 through 12.1 years) performed a set of serial RAN tasks. We extracted fixation-related potentials (FRPs) under phonologically similar (rime-confound) or visually similar (resembling lowercase letters) and dissimilar (non-confounding and discrete uppercase letters, respectively) control tasks.ResultsResults revealed significant differences in FRP amplitudes between DYS and CAC groups under the phonologically similar and phonologically non-confounding conditions. No differences were observed in the case of the visual conditions. Moreover, regression analysis showed that the average amplitude of the extracted components significantly predicted RAN performance.ConclusionFRPs capture neural components during the serial RAN task informative of differences between DYS and CAC and establish a relationship between neurocognitive processes during serial RAN and dyslexia.SignificanceWe suggest our approach as a methodological model for the concurrent analysis of neurophysiological and eye-gaze data to decipher the role of RAN in reading.     

Motor dysfunction in mild cognitive impairment as tested by kinematic analysis and transcranial magnetic stimulation

ObjectivePrevious studies have demonstrated voluntary movement alterations as well as motor cortex excitability and plasticity changes in patients with mild cognitive impairment (MCI). To investigate the pathophysiology of movement abnormalities in MCI, we tested possible relationships between movement abnormalities and primary motor cortex alterations in patients.MethodsFourteen amnestic MCI (aMCI) patients and 16 healthy controls were studied. Cognitive assessment was performed using clinical scales. Finger tapping was recorded by a motion analysis system. Transcranial magnetic stimulation was used to test the input/output curve of motor evoked potentials, intracortical inhibition, and short-latency afferent inhibition. Primary motor cortex plasticity was probed by theta burst stimulation. We investigated correlations between movement abnormalities, clinical scores, and cortical neurophysiological parameters.ResultsMCI patients showed less rhythmic movement but no other movement abnormalities. Cortical excitability measures were normal in patients, whereas plasticity was reduced. Movement rhythm abnormalities correlated with frontal dysfunction scores.ConclusionOur study in MCI patients demonstrated abnormal voluntary movement and plasticity changes, with no correlation between the two. Altered rhythm correlated with frontal dysfunction.SignificanceOur results contribute to the understanding of pathophysiological mechanisms of motor impairment in MCI.     

Scalp EEG interictal high frequency oscillations as an objective biomarker of infantile spasms

ObjectiveTo investigate the diagnostic utility of high frequency oscillations (HFOs) via scalp electroencephalogram (EEG) in infantile spasms.MethodsWe retrospectively analyzed interictal slow-wave sleep EEGs sampled at 2,000 Hz recorded from 30 consecutive patients who were suspected of having infantile spasms. We measured the rate of HFOs (80–500 Hz) and the strength of the cross-frequency coupling between HFOs and slow-wave activity (SWA) at 3–4 Hz and 0.5–1 Hz as quantified with modulation indices (MIs).ResultsTwenty-three patients (77%) exhibited active spasms during the overnight EEG recording. Although the HFOs were detected in all children, increased HFO rate and MIs correlated with the presence of active spasms (p < 0.001 by HFO rate; p < 0.01 by MIs at 3–4 Hz; p = 0.02 by MIs at 0.5–1 Hz). The presence of active spasms was predicted by the logistic regression models incorporating HFO-related metrics (AUC: 0.80–0.98) better than that incorporating hypsarrhythmia (AUC: 0.61). The predictive performance of the best model remained favorable (87.5% accuracy) after a cross-validation procedure.ConclusionsIncreased rate of HFOs and coupling between HFOs and SWA are associated with active epileptic spasms.SignificanceScalp-recorded HFOs may serve as an objective EEG biomarker for active epileptic spasms.     

Reduced EEG montage has a high accuracy in the post cardiac arrest setting

ObjectiveTo study if comatose cardiac arrest patients can be assessed with a reduced number of EEG electrodes.Methods110 routine EEGs from 67 consecutive patients, including both hypothermic and normothermic EEGs were retrospectively assessed by three blinded EEG-experts using two different electrode montages. A standard 19-electrode-montage was compared to the reduced version of the same EEGs, down-sampled to six electrodes (F3, T3, P3, F4, T4, P4). We used intra-rater and inter-observer statistics to assess the reliability of the reduced montage for background features and discharges.ResultsThe reduced montage had almost perfect performance for background continuity (κ 0.80–0.88), including identification of highly malignant backgrounds (burst-suppression/suppression) (κ 0.85–0.94) and benign backgrounds (continuous/nearly continuous) (κ 0.85–0.91). We found substantial performance for identifying rhythmic/periodic discharges (κ 0.79–0.86). The reduced montage had high accuracy for assessment of both highly malignant (sensitivity 91–95%, specificity 94–99%) and benign (sensitivity 89–98%, specificity 91–96%) backgrounds, and periodic/rhythmic patterns (sensitivity 79–100%, specificity 89–99%), compared to the full montage. The inter-observer variability was not increased by the reduced montage.ConclusionReduced EEG had high performance for classifying important background and discharge patterns in this post cardiac arrest cohort.SignificanceOur results support the use of reduced EEG-montage for monitoring comatose cardiac arrest patients.     

Source-level EEG and graph theory reveal widespread functional network alterations in focal epilepsy

ObjectiveThe hypersynchronous neuronal activity associated with epilepsy causes widespread functional network disruptions extending beyond the epileptogenic zone. This altered network topology is considered a mediator for non-seizure symptoms, such as cognitive impairment. The aim of this study was to investigate functional network alterations in focal epilepsy patients with good seizure control and high quality of life.MethodsWe compared twenty-two focal epilepsy patients and sixteen healthy controls on graph metrics derived from functional connectivity of source-level resting-state EEG. Graph metrics were calculated over a range of network densities in five frequency bands.ResultsWe observed a significantly increased small world index in patients relative to controls. On the local level, two left-hemisphere regions displayed a shift towards greater alpha band “hubness”. The findings were not mediated by age, sex or education, nor by age of epilepsy onset, duration or focus lateralisation.ConclusionsWidespread functional network alterations are evident in focal epilepsy, even in a cohort characterised by successful anti-seizure medication therapy and high quality of life. These findings might support the position that functional network analysis could hold clinical relevance for epilepsy.SignificanceFocal epilepsy is accompanied by global and local functional network aberrancies which might be implied in the sustenance of non-seizure symptoms.     

EEG patterns and their correlations with short- and long-term mortality in patients with hypoxic encephalopathy

ObjectiveTo analyze the association between electroencephalographic (EEG) patterns and overall, short- and long-term mortality in patients with hypoxic encephalopathy (HE).MethodsRetrospective, mono-center analysis of 199 patients using univariate log-rank tests (LR) and multivariate cox regression (MCR).ResultsShort-term mortality, defined as death within 30-days post-discharge was 54.8%. Long-term mortality rates were 69.8%, 71.9%, and 72.9%, at 12-, 24-, and 36-months post-HE, respectively. LR revealed a significant association between EEG suppression (SUP) and short-term mortality, and identified low voltage EEG (LV), burst suppression (BSP), periodic discharges (PD) and post-hypoxic status epilepticus (PSE) as well as missing (aBA) or non-reactive background activity (nrBA) as predictors for overall, short- and long-term mortality. MCR indicated SUP, LV, BSP, PD, aBA and nrBA as significantly associated with overall and short-term mortality to varying extents. LV and BSP were significant predictors for long-term mortality in short-term survivors. Rhythmic delta activity, stimulus induced rhythmic, periodic or ictal discharges and sharp waves were not significantly associated with a higher mortality.ConclusionThe presence of several specific EEG patterns can help to predict overall, short- and long-term mortality in HE patients.SignificanceThe present findings may help to improve the challenging prognosis estimation in HE patients.     

Social jetlag and sleep deprivation are associated with altered activity in the reward-related brain areas: an exploratory resting-state fMRI study

ObjectiveThe aim of this research was to assess the effect of social jetlag (SJL) and its interaction with partial sleep deprivation on resting-state brain activity using the fractional amplitude of low-frequency fluctuation (fALFF) during free-living conditions.MethodsA total of 28 normal weight healthy subjects were enrolled in four study groups (with SJL [with sleep deprivation and without sleep deprivation] and without SJL [with sleep deprivation and without sleep deprivation]), matched 1:1:1:1 for age, gender, and body mass index (BMI). Resting-state functional magnetic resonance imaging (fMRI) scans were collected with SIEMENS 3T scanner while subjects were in a fasting state.ResultsParticipants with SJL had significantly higher fALFF values in right lingual gyrus and right putamen and significantly lower fALFF values in left and right inferior parietal lobe in comparison with participants without SJL and without sleep deprivation. Subjects with sleep deprivation had significantly higher fALFF in the thalamus and left superior frontal gyrus. In those with both SJL and sleep deprivation, we observed higher fALFF values in right Brodmann Area (BA)18 and lower values in left and right parietal inferior lobe. Subjects with SJL alone had significantly lower fALFF values in left frontal mid gyrus (BA6) than those with sleep deprivation alone.ConclusionsSJL was associated with altered resting-state brain activity in regions that have been shown to be involved in hedonic feeding. The effect of SJL was independent of effects induced by short sleep duration. These alterations might represent the substrate for the increased risk of obesity observed in those with SJL.     

Quantitative EEG improves prediction of Sturge-Weber syndrome in infants with port-wine birthmark

ObjectivePort-wine birthmark (PWB) is a common occurrence in the newborn, and general pediatricians, dermatologists, and ophthalmologists are often called on to make an assessment of risk for Sturge-Weber syndrome (SWS) due to workforce shortages in pediatric neurologists and MRI’s low sensitivity for SWS brain involvement in infants. We therefore aimed to develop a quantitative EEG (qEEG) approach to safely screen young infants with PWB for SWS risk and optimal timing of diagnostic MRI.MethodsForty-eight infants (prior to first birthday) underwent EEG recording. Signal processing methods compared voltage between left and right sides using a previously defined pipeline and diagnostic threshold. In this test sample, we compared sensitivity/specificity of the qEEG metric against MRI performed after the first birthday. We also used likelihood ratio testing to determine whether qEEG adds incremental information beyond topographical extent of PWB, another risk marker of brain involvement.ResultsqEEG helped predict SWS risk in the first year of life (p = 0.031), with a sensitivity of 50% and a specificity of 81%. It added about 40% incremental information beyond PWB extent alone (p = 0.042).ConclusionqEEG adds information to risk prediction in infants with facial PWB.SignificanceqEEG can be used to help determine whether to obtain an MRI in the first year of life. The data collected can assist in developing a predictive model risk calculator that incorporates both PWB extent and qEEG results, which can be validated and then employed in the community.