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This article provides recommendations on the minimum standards for recording routine (“standard”) and sleep electroencephalography (EEG). The joint working group of the International Federation of Clinical Neurophysiology (IFCN) and the International League Against Epilepsy (ILAE) developed the standards according to the methodology suggested for epilepsy-related clinical practice guidelines by the Epilepsy Guidelines Working Group. We reviewed the published evidence using the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) statement. The quality of evidence for sleep induction methods was assessed by the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) method. A tool for Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) was used to assess the risk of bias in technical and methodological studies. Where high-quality published evidence was lacking, we used modified Delphi technique to reach expert consensus. The GRADE system was used to formulate the recommendations. The quality of evidence was low or moderate. We formulated 16 consensus-based recommendations for minimum standards for recording routine and sleep EEG. The recommendations comprise the following aspects: indications, technical standards, recording duration, sleep induction, and provocative methods.

Contains fulltext : 243955.pdf (Publisher’s version ) (Open Access) PURPOSE: Pathogenic variants in SETD1B have been associated with a syndromic neurodevelopmental disorder including intellectual disability, language delay, and seizures. To date, clinical features have been described for 11 patients with (likely) pathogenic SETD1B sequence variants. This study aims to further delineate the spectrum of the SETD1B-related syndrome based on characterizing an expanded patient cohort. METHODS: We perform an in-depth clinical characterization of a cohort of 36 unpublished individuals with SETD1B sequence variants, describing their molecular and phenotypic spectrum. Selected variants were functionally tested using in vitro and genome-wide methylation assays. RESULTS: Our data present evidence for a loss-of-function mechanism of SETD1B variants, resulting in a core clinical phenotype of global developmental delay, language delay including regression, intellectual disability, autism and other behavioral issues, and variable epilepsy phenotypes. Developmental delay appeared to precede seizure onset, suggesting SETD1B dysfunction impacts physiological neurodevelopment even in the absence of epileptic activity. Males are significantly overrepresented and more severely affected, and we speculate that sex-linked traits could affect susceptibility to penetrance and the clinical spectrum of SETD1B variants. CONCLUSION: Insights from this extensive cohort will facilitate the counseling regarding the molecular and phenotypic landscape of newly diagnosed patients with the SETD1B-related syndrome.

This study evaluated the accuracy of neonatal amplitude-integrated electroencephalography (aEEG) brain monitoring for predicting development of postneonatal epilepsy after perinatal hypoxic ischemic encephalopathy (HIE).We studied a population-based cohort of 85 consecutive neonates with moderate-to-severe HIE that had aEEG started12 hours postnatally. We marked electrographic seizures and graded each hour of the aEEG background as inactive, burst-suppression, or continuous without or with sleep cycling. These aEEG parameters were compared to outcome at 4-years age (deceased, epilepsy, cerebral palsy without epilepsy, favorable), which was available for 80 children.At group level, total seizure burden (p = 0.003), maximum hourly seizure burden (p = 0.007), and aEEG background recovery (p 0.001) were all significantly associated with outcome. At individual level six children developed epilepsy, and the most accurate predictors for later epilepsy were inactive aEEG at 24 hours (accuracy 97%, positive predictive value 100%, two false negatives) and inactive aEEG at the onset of seizures (accuracy 97%, sensitivity of 100%, one false positive).At individual level aEEG background recovery was a better predictor for later epilepsy than neonatal seizures, although both were associated with epilepsy at group level.Poor aEEG background recovery predicts development of epilepsy after perinatal HIE at individual level.

Publisher Copyright: © 2022 Korean Cleft Palate-Craniofacial Association. Background: Deformational plagiocephaly is usually managed conservatively, as it tends to improve over time and with the use of conser-vative measures. However, before the year 2017 we operated on patients with severe plagiocephaly and neurological symptoms at the Helsinki Cleft Palate and Craniofacial Center. Methods: Of the 20 infants with severe deformational plagiocephaly and neurological symptoms referred to us between 2014 and 2016, 10 underwent cranioplasty open reshaping of the posterior cranial vault. The parents of the last 10 patients were given information on the natural history of the condition and the patients were followed up with an outpatient protocol. The aim of this study was to gain information on the brain electrophysiology and recovery of patients after total cranial vault reconstruction by measuring the electroencephalogram (EEG) somatosensory evoked potentials (SEP; median nerve). Results: Of the 10 participants in the operation arm, six had abnormal SEP at least on the affected cerebral hemisphere and all SEPs were recorded as normal when controlled postoperatively. In the follow-up arm, eight out of 10 participants had abnormal SEP at the age of ap-proximately 24 months, and all had normalized SEPs at control visits. Conclusion: Our data suggest that cranioplasty open reshaping of the posterior cranial vault did not affect abnormal SEP-EEG recordings. We have abandoned the operations in deformational plagiocephaly patients due to findings suggesting that expanding cranioplasty is not beneficial for brain function in this patient group.

Highlights • MEG may aid in the localization of the epileptogenic zone in the parietal lobe. • SAMepi – a novel kurtosis beamformer – results in localizations similar to those of the ECD analysis. • A unifocal result in both the ECD and the SAMepi analysis is associated with a good clinical outcome., Objective To evaluate a novel analysis method (SAMepi) in the localization of interictal epileptiform magnetoencephalographic (MEG) activity in parietal lobe epilepsy (PLE) patients in comparison with equivalent current dipole (ECD) analysis. Methods We analyzed the preoperative interictal MEG of 17 operated PLE patients utilizing visual analysis and: (1) ECD with a spherical conductor model; (2) ECD with a boundary element method (BEM) conductor model; and (3) SAMepi – a kurtosis beamformer method. Localization results were compared between the three methods, to the location of the resection and to the clinical outcome. Results Fourteen patients had an epileptiform finding in the visual analysis; SAMepi detected spikes in 11 of them. A unifocal finding in both the ECD and in the SAMepi analysis was associated with a better chance of seizure-freedom (p = 0.02). There was no significant difference in the distances from the unifocal MEG localizations to the nearest border of the resection between the different analysis methods. Conclusions Localizations of unifocal interictal spikes detected by SAMepi did not significantly differ from the conventional ECD localizations. Significance SAMepi – a novel semiautomatic analysis method – is useful in localizing interictal epileptiform MEG activity in the presurgical evaluation of parietal lobe epilepsy patients.

Objective: The unspecific symptoms of neonatal stroke still challenge its bedside diagnosis. We studied the accuracy of routine electroencephalography (EEG) and simultaneously recorded somatosensory evoked potentials (EEG-SEP) for diagnosis and outcome prediction of neonatal stroke. Methods: We evaluated EEG and EEG-SEPs from a hospital cohort of 174 near-term neonates with suspected seizures or encephalopathy, 32 of whom were diagnosed with acute ischemic or hemorrhagic stroke in MRI. EEG was scored for background activity and seizures. SEPs were classified as present or absent. Developmental outcome of stroke survivors was evaluated from medical records at 8- to 18-months age. Results: The combination of continuous EEG and uni- or bilaterally absent SEP (n = 10) was exclusively seen in neonates with a middle cerebral artery (MCA) stroke (specificity 100%). Moreover, 80% of the neonates with this finding developed with cerebral palsy. Bilaterally present SEPs did not exclude stroke, but predicted favorable neuromotor outcome in stroke survivors (positive predictive value 95%). Conclusions: Absent SEP combined with continuous EEG background in near-term neonates indicates an MCA stroke and a high risk for cerebral palsy. Significance: EEG-SEP offers a bedside method for diagnostic screening and a reliable prediction of neuromotor outcome in neonates suspected of having a stroke. (C) 2019 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Objective: We assessed in extremely preterm born (EPB) children whether secondary somatosensory cor-tex (SII) responses recorded with magnetoencephalography (MEG) at term-equivalent age (TEA) correlate with neurodevelopmental outcome at age 6 years. Secondly, we assessed whether SII responses differ between 6-year-old EPB and term-born (TB) children. Methods: 39 EPB children underwent MEG with tactile stimulation at TEA. At age 6 years, 32 EPB and 26 TB children underwent MEG including a sensorimotor task requiring attention and motor inhibition. SII responses to tactile stimulation were modeled with equivalent current dipoles. Neurological outcome, motor competence, and general cognitive ability were prospectively evaluated at age 6 years. Results: Unilaterally absent SII response at TEA was associated with abnormal motor competence in 6-year-old EPB children (p = 0.03). At age 6 years, SII responses were bilaterally detectable in most EPB (88%) and TB (92%) children (group comparison, p = 0.69). Motor inhibition was associated with decreased SII peak latencies in TB children, but EPB children lacked this effect (p = 0.02). Conclusions: Unilateral absence of an SII response at TEA predicted poorer motor outcome in EPB children. Significance: Neurophysiological methods may provide new means for outcome prognostication in EPB children. (c) 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

Background Extremely low gestational age newborns (ELGANs) are at risk of neurodevelopmental impairments that may originate in early NICU care. We hypothesized that early oxygen saturations (SpO2), arterial pO2 levels, and supplemental oxygen (FiO2) would associate with later neuroanatomic changes. Methods SpO2, arterial blood gases, and FiO2 from 73 ELGANs (GA 26.4 ± 1.2; BW 867 ± 179 g) during the first 3 postnatal days were correlated with later white matter injury (WM, MRI, n = 69), secondary cortical somatosensory processing in magnetoencephalography (MEG-SII, n = 39), Hempel neurological examination (n = 66), and developmental quotients of Griffiths Mental Developmental Scales (GMDS, n = 58). Results The ELGANs with later WM abnormalities exhibited lower SpO2 and pO2 levels, and higher FiO2 need during the first 3 days than those with normal WM. They also had higher pCO2 values. The infants with abnormal MEG-SII showed opposite findings, i.e., displayed higher SpO2 and pO2 levels and lower FiO2 need, than those with better outcomes. Severe WM changes and abnormal MEG-SII were correlated with adverse neurodevelopment. Conclusions Low oxygen levels and high FiO2 need during the NICU care associate with WM abnormalities, whereas higher oxygen levels correlate with abnormal MEG-SII. The results may indicate certain brain structures being more vulnerable to hypoxia and others to hyperoxia, thus emphasizing the role of strict saturation targets. Impact This study indicates that both abnormally low and high oxygen levels during early NICU care are harmful for later neurodevelopmental outcomes in preterm neonates. Specific brain structures seem to be vulnerable to low and others to high oxygen levels. The findings may have clinical implications as oxygen is one of the most common therapies given in NICUs. The results emphasize the role of strict saturation targets during the early postnatal period in preterm infants.

Pathogenic variants in SETD1B have been associated with a syndromic neurodevelopmental disorder including intellectual disability, language delay and seizures. To date, clinical features have been described for eleven patients with (likely) pathogenic SETD1B sequence variants. We perform an in-depth clinical characterization of a cohort of 36 unpublished individuals with SETD1B sequence variants, describing their molecular and phenotypic spectrum. Selected variants were functionally tested using in vitro and genome-wide methylation assays. Our data present evidence for a loss-of-function mechanism of SETD1B variants, resulting in a core clinical phenotype of global developmental delay, language delay including regression, intellectual disability, autism and other behavioral issues, and variable epilepsy phenotypes. Developmental delay appeared to precede seizure onset, suggesting SETD1B dysfunction impacts physiological neurodevelopment even in the absence of epileptic activity. Interestingly, males are significantly overrepresented and more severely affected, and we speculate that sex-linked traits could affect susceptibility to penetrance and the clinical spectrum of SETD1B variants. Finally, despite the possibility of non-redundant contributions of SETD1B and its paralogue SETD1A to epigenetic control, the clinical phenotypes of the related disorders share many similarities, indicating that elucidating shared and divergent downstream targets of both genes will help to understand the mechanism leading to the neurobehavioral phenotypes. Insights from this extensive cohort will facilitate the counseling regarding the molecular and phenotypic landscape of newly diagnosed patients with the SETD1B-related syndrome.

Background Somatosensory evoked potentials (SEPs) offer an additional bedside tool for outcome prediction after perinatal asphyxia. Aims To assess the reliability of SEPs recorded with bifrontoparietal amplitude-integrated electroencephalography (aEEG) brain monitoring setup for outcome prediction in asphyxiated newborns undergoing therapeutic hypothermia. Study design Retrospective observational single-center study. Subjects 27 consecutive asphyxiated full- or near-term newborns (25 under hypothermia) that underwent median nerve aEEG-SEPs as part of their clinical evaluation at the neonatal intensive care unit of Helsinki University Hospital. Outcome measures aEEG-SEP classification (present, absent or unreliable) was compared to classification of SEPs recorded with a full EEG montage (EEG-SEP), and outcome determined from medical records at approximately 12-months-age. Unfavorable outcome included death, cerebral palsy, or severe epilepsy. Results The aEEG-SEP and EEG-SEP classifications were concordant in 21 of the 22 newborns with both recordings available. All five newborns with bilaterally absent aEEG-SEPs had absent EEG-SEPs and the four with outcome information available had an unfavorable outcome (one was lost to follow-up). Of the newborns with aEEG-SEPs present, all with follow-up exams available had bilaterally present EEG-SEPs and a favorable outcome (one was lost to follow-up). One newborn with unilaterally absent aEEG-SEP at 25 h of age had bilaterally present EEG-SEPs on the next day, and a favorable outcome. Conclusions aEEG-SEPs recorded during therapeutic hypothermia on the first postnatal days are reliable for assessing brain injury severity. Adding SEP into routine aEEG brain monitoring offers an additional tool for very early outcome prediction after birth asphyxia.

Correction: Volume88, Page158-158 DOI10.1016/j.seizure.2021.04.001 PublishedMAY 2021 Purpose: To evaluate the accuracy of hypoxic ischemic encephalopathy (HIE) grade, and neonatal neurophysiological and neuroimaging measures for predicting development of infantile spasms syndrome (IS) or other postneonatal, infantile onset epilepsy after perinatal HIE. Methods: We examined a population-based cohort of 92 consequent infants with moderate-to-severe HIE. The HIE grade and neonatal neuroimaging (MRI) and neurophysiology (EEG and somatosensory evoked potentials, SEPs) findings were compared to the development of IS or other epilepsy within the first year of life. Results: Out of 74 surviving infants with follow-up information, five developed IS and one developed a focal onset epilepsy. They all had recovered from severe HIE. All survivors with inactive neonatal EEG (recorded within the first few postnatal days, n = 4) or the most severe type of brain injury in MRI (n = 3) developed epilepsy (positive predictive value, PPV 100 %). Bilaterally absent SEPs had 100 % sensitivity and 75 % PPV for epilepsy. A combination of absent SEPs and a poor MRI finding (combined deep and cortical gray matter injury) resulted in higher PPV (86 %) without lowering sensitivity (100 %). Follow-up EEGs showed recurrent epileptiform activity already between 1- and 2-months age in those that developed epilepsy, distinguishing them from those surviving without epilepsy. Conclusions: Poor neonatal neuroimaging and neurophysiological findings provide accurate prediction for development of infantile onset epilepsy after HIE. Of the neonates with severe HIE, the ones with severe neonatal MRI and neurophysiological abnormalities need frequent follow-up, including repeated EEGs, for early detection of IS.

Funding Information: This work was supported by Helsinki University Hospital, Neuroscience Center (HiLIFE; Univ. Helsinki), Aivosäätiö, Finnish Pediatric Foundation/ Lastentautiensäätiö (SV), Sigrid Juselius Foundation, and Academy of Finland (SV, 313242, 288220, 3104450). Funding Information: This work was supported by Helsinki University Hospital, Neuroscience Center (HiLIFE; Univ. Helsinki), Aivos??ti?, Finnish Pediatric Foundation/ Lastentautiens??ti? (SV), Sigrid Juselius Foundation, and Academy of Finland (SV, 313242, 288220, 3104450). Publisher Copyright: © 2021 International Federation of Clinical Neurophysiology Objective: To examine the usability of long-range temporal correlations (LRTCs) in non-invasive localization of the epileptogenic zone (EZ) in refractory parietal lobe epilepsy (RPLE) patients. Methods: We analyzed 10 RPLE patients who had presurgical MEG and underwent epilepsy surgery. We quantified LRTCs with detrended fluctuation analysis (DFA) at four frequency bands for 200 cortical regions estimated using individual source models. We correlated individually the DFA maps to the distance from the resection area and from cortical locations of interictal epileptiform discharges (IEDs). Additionally, three clinical experts inspected the DFA maps to visually assess the most likely EZ locations. Results: The DFA maps correlated with the distance to resection area in patients with type II focal cortical dysplasia (FCD) (p

Highlights • Neonatal SEPs can be reliably detected using the 4-electrode aEEG monitoring setup. • SEP is discernible in most fullterm newborns with 300 averages. • Recording SEPs jointly with aEEG facilitates SEP assessment after birth asphyxia., Objective To evaluate the reliability of recording cortical somatosensory evoked potentials (SEPs) in asphyxiated newborns using the 4-electrode setup applied in routine long-term amplitude-integrated EEG (aEEG) brain monitoring and to assess the number of averages needed for reliably detecting the cortical responses. Methods We evaluated median nerve SEPs in 50 asphyxiated full-term newborns. The SEP interpretation (present or absent) from the original recordings with 21-electrodes and approximately 600 trials served as the reference. This was compared to SEP classification (absent, present, or unreliable) based on a reduced (300 or 150) number of averages, and to classification based on only four electrodes (F3, P3, F4, P4). Results Compared to the original classification, cortical SEPs were uniformly interpreted as present or absent in all 50 newborns with the 4-electrode setup and 600 averages. Reducing number of averages to 300 still resulted in correct SEP interpretation in 49/50 newborns with 21-electrode setup, and 46/50 newborns with 4-electrode setup. Conclusions Evaluation of early cortical neonatal SEPs is reliable from the 4-electrode setup commonly used in aEEG monitoring. SEP is discernible in most newborns with 300 averages. Significance Adding SEP into routine aEEG monitoring offers an additional tool for early neonatal neurophysiological evaluation.

Background:. Active shoulder external rotation in adduction can be restored by selective neurotization of the infraspinatus muscle with the spinal accessory nerve in select patients with brachial plexus birth injury. Does the improved shoulder external rotation stand the test of time? Methods:. Fourteen consecutive brachial plexus birth injury patients with active shoulder external rotation in adduction of ≤ 0 degrees and active shoulder elevation ≥ 90 degrees underwent selective neurotization of the infraspinatus muscle at mean 2 years of age between 2012 and 2016. All 14 patients had congruent shoulders joints with passive external rotation in adduction of 30 degrees. Pre-and postoperative electromyography was done to seven patients. Shoulder function and the subjective outcome was assessed after a mean follow-up of 3.8 years. Results:. Shoulder external rotation in adduction improved by a mean 57 degrees in the 12 children who did not develop shoulder internal rotation contracture. Shoulder external rotation in abduction and shoulder abduction increased in all 14 patients. Reinnervation of the supraspinatus muscle was evident in all seven children who underwent postoperative EMG. Thirteen patients’ parents were satisfied with the outcome. Conclusions:. Functionally significant shoulder external rotation can be restored and maintained by reinnervation of the infraspinatus muscle in brachial plexus birth injury patients with congruent shoulder joints, if internal rotation contracture does not develop.

Neonatal seizures are a common emergency in the neonatal intensive care unit (NICU). There are many questions yet to be answered regarding the temporal/spatial characteristics of seizures from different pathologies, response to medication, effects on neurodevelopment and optimal detection. The dataset presented in this descriptor contains EEG recordings from human neonates, the visual interpretation of the EEG by the human experts, supporting clinical data and codes to assist access. Multi-channel EEG was recorded from 79 term neonates admitted to the NICU at the Helsinki University Hospital. The median recording duration was 74 min (IQR: 64 to 96 min). The presence of seizures in the EEGs was annotated independently by three experts. An average of 460 seizures were annotated per expert in the dataset; 39 neonates had seizures and 22 were seizure free, by consensus. The dataset can be used as a reference set of neonatal seizures, in studies of inter-observer agreement and for the development of automated methods of seizure detection and other EEG analyses. Machine-accessible metadata file describing the reported data (ISA-Tab format)

Purpose In this study, we aimed to improve our knowledge of insular epilepsy by studying anatomoelectroclinical correlations in pure insular-onset epilepsy and characterizing differences between anterior and posterior insular-onset seizures. Methods Patients in whom seizure-onset zone was confined to the insula and peri-insular sulcus were selected from 301 consecutive presurgical stereo-electroencephalography (EEG) recordings performed between years 2010 and 2017 in two epilepsy centers. Ictal-onset zone in stereo-EEG was delineated visually and quantitatively using epileptogenic index method. Seizure characteristics were reanalyzed, and anatomoelectroclinical correlations were assessed. Characteristics of posterior and anterior insular-onset seizures were compared. Results Eleven insular cases were identified, five of them with an anterior insular seizure onset and six with a posterior one. Nonpainful somatosensory symptoms and autonomic symptoms were the most common symptoms (73% of patients) followed by speech-related symptoms (55%) and ipsilateral eye blinking (45%). Six patients had seizures restricted to somatosensory or viscerosensory symptoms. In all patients, seizures progressed to motor symptoms. Somatosensory symptoms did not differentiate anterior from posterior insular seizures. However, hyperkinetic signs, speech modifications, and viscerosensory symptoms were related to an anterior insular seizure-onset zone. Pain, asymmetric tonic, focal clonic, and tonic symptoms were more frequent in patients with a posterior insular seizure onset. Conclusions Seizure semiology is heterogeneous in pure insular-onset epilepsy. Differences between the anterior and posterior insular seizures reflect the functional organization of the insula. Particularly, the different types of motor symptoms may help to distinguish anterior from posterior insular seizure onset.

Idiopathic toe walking is a relatively common developmental condition often leading to secondary problems such as pain and muscle contractures in the lower extremities. The cause of idiopathic toe walking is unknown, which hinders the development of treatment strategies. To test whether children with idiopathic toe walking have functional alterations in their spinal motor circuits, we studied the properties of the soleus H-reflex and its modulation with vibration in 26 idiopathic toe walkers and 16 typically developing children. At the group level, the H-reflex properties did not differ, but at the individual level, in 7 of 25 idiopathic toe walkers, some of the H-reflex parameters fell out of normal limits of typically developing children. However, the H-reflex was suppressed by vibration to the Achilles tendon similarly in both the idiopathic toe walkers and typically developing children. In conclusion, idiopathic toe walking in some children can be associated with functional alterations in their spinal motor circuits.

Opioid exposure during pregnancy is a potential risk factor for the developing central nervous system of the fetus. We studied evoked responses in buprenorphine-exposed newborns who displayed neonatal abstinence syndrome (NAS) to elucidate the possible alterations in functioning of the somatosensory and auditory systems.We compared somatosensory (SEFs) and auditory evoked magnetic fields (AEFs), recorded with magnetoencephalography (MEG), of 11 prenatally buprenorphine-exposed newborns with those of 12 healthy newborns. Peak latencies, source strength and location of SEFs or AEFs were recorded.AEFs were present in all buprenorphine-exposed newborns without significant differences from those of healthy newborns. In contrast, though no group level differences in SEFs existed, at individual level the response deviated from the typical neonatal morphology in four buprenorphine-exposed newborns.Although buprenorphine exposure during pregnancy does not seem to cause constant deficiencies in somatosensory or auditory processing, in some newborns the typical development of somatosensory networks may be - at least transiently - disrupted.

Objective: To evaluate the added value of somatosensory (SEPs) and visual evoked potentials (VEPs) recorded simultaneously with routine EEG in early outcome prediction of newborns with hypoxicischemic encephalopathy under modern intensive care. Methods: We simultaneously recorded multichannel EEG, median nerve SEPs, and flash VEPs during the first few postnatal days in 50 term newborns with hypoxic-ischemic encephalopathy. EEG background was scored into five grades and the worst two grades were considered to indicate poor cerebral recovery. Evoked potentials were classified as absent or present. Clinical outcome was determined from the medical records at a median age of 21 months. Unfavorable outcome included cerebral palsy, severe mental retardation, severe epilepsy, or death. Results: The accuracy of outcome prediction was 98% with SEPs compared to 90% with EEG. EEG alone always predicted unfavorable outcome when it was inactive (n = 9), and favorable outcome when it was normal or only mildly abnormal (n = 17). However, newborns with moderate or severe EEG background abnormality could have either favorable or unfavorable outcome, which was correctly predicted by SEP in all but one newborn (accuracy in this subgroup 96%). Absent VEPs were always associated with an inactive EEG, and an unfavorable outcome. However, presence of VEPs did not guarantee a favorable outcome. Conclusions: SEPs accurately predict clinical outcomes in newborns with hypoxic-ischemic encephalopathy and improve the EEG-based prediction particularly in those newborns with severely or moderately abnormal EEG findings. Significance: SEPs should be added to routine EEG recordings for early bedside assessment of newborns with hypoxic-ischemic encephalopathy. (C) 2017 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Cerebral palsy (CP) is characterized by difficulty in control of movement and posture due to brain damage during early development. In addition, tactile discrimination deficits are prevalent in CP. To study the function of somatosensory and motor systems in CP, we compared the reactivity of sensorimotor cortical oscillations to median nerve stimulation in 12 hemiplegic CP children vs. 12 typically developing children using magnetoencephalography. We also determined the primary cortical somatosensory and motor representation areas of the affected hand in the CP children using somatosensory‐evoked magnetic fields and navigated transcranial magnetic stimulation, respectively. We hypothesized that the reactivity of the sensorimotor oscillations in alpha (10 Hz) and beta (20 Hz) bands would be altered in CP and that the beta‐band reactivity would depend on the individual pattern of motor representation. Accordingly, in children with CP, suppression and rebound of both oscillations after stimulation of the contralateral hand were smaller in the lesioned than intact hemisphere. Furthermore, in two of the three children with CP having ipsilateral motor representation, the beta‐ but not alpha‐band modulations were absent in both hemispheres after affected hand stimulation suggesting abnormal sensorimotor network interactions in these individuals. The results are consistent with widespread alterations in information processing in the sensorimotor system and complement current understanding of sensorimotor network development after early brain insults. Precise knowledge of the functional sensorimotor network organization may be useful in tailoring individual rehabilitation for people with CP. Hum Brain Mapp 35:4105–4117, 2014. © 2014 Wiley Periodicals, Inc.

Children born extremely preterm may have difficulties in response inhibition, but the neural basis of such problems is unknown. We recorded magnetoencephalography (MEG) during a somatosensory Go/NoGo task in six-year-old children born extremely preterm (EPT; n = 22) and in children born full term (FT; n = 21). The children received tactile stimuli randomly to their left little (target) and index (non-target) finger and were instructed to squeeze a soft toy with the opposite hand every time they felt a stimulus on the little finger. Behaviorally, the EPT children performed worse than the FT children, both in responding to the target finger stimulation and in refraining from responding to the non-target finger stimulation. In MEG, after the non-target finger stimulation (i.e., during the response inhibition), the sensorimotor alpha and beta oscillation levels in the contralateral-to-squeeze hemisphere were elevated in the FT children when compared with a condition with corresponding stimulation but no task (instead the children were listening to a story and not attending to the fingers). This NoGo task effect was absent in the EPT children. Further, in the sensorimotor cortex contralateral to the tactile stimulation, the post-stimulus suppression was less pronounced in the EPT than FT children. We suggest that the missing NoGo task effect and lower suppression of sensorimotor oscillations are markers of deficient functioning of the sensorimotor networks in the EPT children.

Objectives: To measure changes in the visual interpretation of the EEG by the human expert for neonatal seizure detection when reducing the number of recording electrodes. Methods: EEGs were recorded from 45 infants admitted to the neonatal intensive care unit (NICU). Three experts annotated seizures in EEG montages derived from 19, 8 and 4 electrodes. Differences between annotations were assessed by comparing intra-montage with inter-montage agreement (K). Results: Three experts annotated 4464 seizures across all infants and montages. The inter-expert agreement was not significantly altered by the number of electrodes in the montage (p = 0.685, n = 43). Reducing the number of EEG electrodes altered the seizure annotation for all experts. Agreement between the 19-electrode montage (K-19,K-19 = 0.832) was significantly higher than the agreement between 19 and 8-electrode montages (dK = 0.114; p

Aim Treatment with therapeutic hypothermia has challenged the use of amplitude-integrated electroencephalography in predicting outcomes after perinatal asphyxia. In this study, we assessed the feasibility and gain of somatosensory evoked potentials (SEP) during hypothermia. Methods This retrospective study comprised neonates from 35 + 6 to 42 + 2 gestational weeks and treated for asphyxia or hypoxic-ischaemic encephalopathy at Helsinki University Hospital between 14 February 2007 and 23 December 2009. This period was partly before the introduction of routine therapeutic hypothermia, which enabled us to include normothermic neonates who would these days receive hypothermia treatment. We analysed SEPs from 47 asphyxiated neonates and compared the results between 23 normothermic and 24 hypothermic neonates. Results Our data showed that hypothermia led to SEP latencies lengthening by a few milliseconds, but the essential gain for predicting outcomes by SEPs was preserved during hypothermia. Of the 24 hypothermic neonates, bilaterally absent SEPs were associated with poor outcome in 2/2 neonates, normal SEPs were associated with good outcomes in 13/15 neonates and 5/7 neonates with unilaterally absent or grossly delayed SEPs had a poor outcome. Conclusion Our findings indicated that SEPs were a reliable tool for evaluating the somatosensory system in asphyxiated neonates in both normothermic and hypothermic conditions.

highlights In 46 healthy newborns, activity from both the primary (SI) and secondary somatosensory cortices (SII) was detectable with magnetoencephalography to tactile stimulation of the contralateral index finger dur- ing quiet sleep (QS). No significant interhemispheric differences in the responses from SI or SII existed. Within the neonatal period (postmenstrual age (PMA) between 37 and 44 weeks) PMA, height, or gen- der did not significantly affect the latency or strength of the somatosensory evoked magnetic fields (SEFs). abstract Objective: To determine interhemispheric differences and effect of postmenstrual age (PMA), height, and gender on somatosensory evoked magnetic fields (SEFs) from the primary (SI) and secondary (SII) somatosensory cortices in healthy newborns. Methods: We recorded SEFs to stimulation of the contralateral index finger (right in 46 and left in 12) healthy fullterm newborns and analyzed the magnetic responses with equivalent current dipoles. Results: Activity from both the SI and SII was consistently detectable in the contralateral hemisphere of the newborns during quiet sleep. No significant interhemispheric differences existed in SI or SII response peak latencies, source strengths, or location (n = 8, quiet sleep). SI or SII response peak latency or source strength were not significantly affected by PMA, height, or gender. Conclusions: During the neonatal period (PMA 37-44 weeks), activity from the contralateral SI and SII can be reliably evaluated with MEG. The somatosensory responses are similar in the left and right hemi- spheres and no corrections for exact PMA, height, or gender are necessary for interpreting the results. However, the evaluation should be conducted in quiet sleep. Significance: The reproducibility of the magnetic SI and SII responses suggests clinical applicability of the presented MEG method.

Summary Magnetoencephalography (MEG) is a noninvasive method to study brain activity. In the previous decade the advantages of MEG — good temporal resolution combined with good spatial resolution allowing separation of activated brain areas — have been successfully used in gaining new information about the neonatal brain functioning. In this review, we discuss the findings from studies of spontaneous magnetoencephalogram and evoked responses to somatosensory, auditory, and visual stimulation. Our group has shown that stimulation of the upper limb in neonates evokes a response sequence reflecting activation of both primary (S I ) and secondary somatosensory (S II ) cortices. Like in mature brains, the earliest cortical response to median nerve stimulation reflects the arrival of afferent information to S I . However, source modeling of the subsequent activation from S I suggests immature cortical functioning in neonates. Another feature typical for neonates is that the S II response is prominent in quiet sleep, unlike in adults in whom it diminishes in sleep. Interestingly, in very prematurely-born infants, we found alterations of the somatosensory responses at both group and individual levels. MEG provides a novel way to look at brain activity in neonates and can be used to increase knowledge of the development of brain processing and its disturbances.

Aim In individuals with cerebral palsy (CP), cerebral insults during early development may induce profound reorganization of the motor representation. This study determined the extent of alterations in cortical somatosensory functions in adolescents with hemiplegic CP with subcortical brain lesions. Method We recorded somatosensory evoked magnetic fields in response to hand area stimulation from eight adolescents with hemiplegic CP (five females and three males; mean age 14y 6mo, SD 2y 3mo) and eight age- and sex-matched healthy comparison adolescents (mean age 15y 4mo, SD 2y 4mo). All participants in the CP group had purely subcortical brain lesions in magnetic resonance images. Results The somatosensory representation of the affected limb was contralateral (i.e. ipsilesional), but detailed inspection of the evoked responses showed alterations bilaterally. In the primary somatosensory cortex, the representation areas of digits II and V were in both hemispheres closer to each other in participants with CP than in comparison participants [ANOVA main effect group F1,14=5.58; p=0.03]. In addition, the morphology of median nerve evoked fields was altered in the participants with CP. Interpretation In hemiplegic CP, modification of the somatosensory cortical network extends beyond what would be expected based on the unilateral symptoms and the anatomical lesion. Further understanding of the functional alterations in the sensorimotor networks may aid in developing more precisely designed rehabilitation strategies.

Introduction Differential diagnostics and outcome prediction in newborns presenting with seizures or encephalopathy remain difficult in the bedside. The objectives of the present study were (i) to determine the accuracy of simultaneously recorded EEG and somatosensory evoked potentials (SEPs) in differentiating between hypoxic-ischemic encephalopathy (HIE) and stroke as the underlying etiology of neonatal seizures or encephalopathy and (ii) to determine the accuracy of EEG and SEPs and their combination for predicting outcome in newborns with HIE or stroke. Methods The study comprised of 133 newborns (gestational age > 34 weeks) that underwent a clinically indicated EEG and simultaneous SEPs in the acute phase of their illness. 102 of the newborns had HIE and 31 had stroke. EEG background was scored as continuous (sleep stages identifiable and EEG continuous at least in active sleep or awake state) or discontinuous (sleep stages not identifiable and EEG not continuous at any time during the recording). The SEPs were classified as bilaterally absent, unilaterally absent, or bilaterally present. Outcome was evaluated from medical records at approximately 1–1.5 years age. Death, cerebral palsy, severe mental retardation, and epilepsy were considered as unfavorable outcomes. Normal or mildly abnormal development (e.g. mild motor or language delay) were considered as favorable outcomes. Results The combinations of EEG and SEP abnormalities were indicative of etiology (HIE vs stroke) and predictive of outcome. If the EEG was discontinuous, the etiology was always HIE (33 newborns) regardless of SEPs. Furthermore, if the EEG was continuous, but SEPs were uni- (9 newborns) or bilaterally (1 newborn) absent, the etiology was always stroke. Normal SEPs and continuous EEG occurred in 69 newborns with HIE and 21 newborns with stroke, and hence in these newborns the distinction between the etiologies could not be made with the present EEG-SEP criteria. This combination was, however, highly predictive of a favorable outcome regardless of the etiology: The outcome was favorable in 67/69 of those HIE and 20/21 of those with stroke. On the contrary, bi- or unilaterally absent SEPs were associated with poor outcome in all newborns with HIE (22/22) and all but two newborns with stroke (8/10). Furthermore, in newborns with HIE, even if the EEG was discontinuous but SEPs were bilaterally present, the outcome was likely to be favorable (8/11 newborns). Conclusion EEG-SEP is useful in bedside differential diagnostics between HIE and stroke in newborns presenting with seizures or general signs of encephalopathy. Furthermore, EEG-SEP is accurate in outcome prediction in both etiologies.

Reported here is the 30-year follow-up of a patient, diagnosed with juvenile neuronal ceroid lipofuscinosis, who was compound heterozygous for the common 1-kb deletion and the missense mutation p.Glu295Lys in the CLN3 gene. Visual failure was noticed at 6 years of age, but thereafter disease progression was atypical. Polyneuropathy and cerebellar signs were observed after age 20, and epilepsy and slight mental decline after age 35. From then on, there was rapid deterioration, and the patient died at age 39. This case highlights the importance of exact genotyping for disease course prediction and management.

Increased survival of extremely low birth weight infants has led to a need for new prognostic methods to predict possible future neurological impairment. We investigated the early development of the somatosensory system by recording the somatosensory evoked magnetic fields (SEFs) during natural sleep at fullterm age in 16 very prematurely born infants and 16 healthy newborns born at term. The purpose was to determine possible changes in the function of the somatosensory cortex in the prematurely born infants by comparing the latency, strength, location and morphology of the SEFs with those of healthy fullterm newborns. We recorded reliable SEFs in all patients and controls. The equivalent current dipole (ECD) strength of the first cortical response, M60, was significantly lower in the patients. Otherwise, the general morphology and latency of the SEFs were similar in the two groups of babies. The similar response latencies in the two groups indicate normally developed conduction in the somatosensory system of the prematurely born infants. The attenuated ECD strength may reflect weaker synchrony in firing or a smaller number of the cortical neurons activated by the somatosensory stimulation. At the individual level, in four of the preterm infants, a later M200 response was not present or could not be modeled: all of them had lesions of the underlying hemisphere depicted by ultrasound and magnetic resonance imaging.

We studied somatosensory evoked magnetic fields (SEFs) to electric and tactile stimulation in newborns. The electric stimulation was applied to the left median nerve at the wrist and the tactile stimulation to the tip of the left or right index finger. An interstimulus interval of 2 s was used. The evoked magnetic fields were measured over the right hemisphere with a 306-channel MEG-system (Elekta Neuromag Oy). The data were off-line analyzed for active and quiet sleep stages. The first deflection after the median nerve stimulation, M30, corresponding to the adult N20 m, peaked at about 30 ms. The field pattern of this early peak was dipolar, and the activity could be modeled using an equivalent current dipole (ECD). The coordinates of the ECD were in agreement with activity generated at the primary somatosensory cortex, the current dipole pointing anteriorly. The locations of the later deflections were compared with the origin of M30.

Objective : Our objective was to characterize the effects of sleep stages on tactile somatosensory evoked responses in full-term newborns. Methods : Somatosensory evoked potentials (SEPs) and magnetic fields (SEFs) to tactile stimulation of the tip of the index finger and/or thenar eminence were measured from 14 healthy newborns. The stimulus was a gentle tap produced by a moving membrane driven by an air-pressure pulse. Results : SEPs and SEFs to tactile stimulation of the skin were similar in waveform and latency to SEPs known to be produced by electrical stimulation of the fingertip of neonates. The two most distinguishable positive deflections of SEPs, P1 and P2, within 300 ms of the stimulation, and their magnetic counterparts were clearly smaller in active compared to quiet sleep. Conclusions : Our study demonstrates for the first time that it is possible to record SEFs in neonates, and that clear late cortical somatosensory responses are produced by tactile stimulation. In addition, the effect of sleep stage on these responses indicates differences in the processing of the incoming information, at least in the somatosensory modality, in active and quiet sleep. Significance : Tactile stimulation may be useful as a completely non-invasive technique for studying the physiology of the somatosensory system in neonates. Methodologically, since the effect of sleep stage is profound, one must carefully monitor the sleep stages in studies of event-related responses in newborns, or else this effect may confound the phenomena being studied.