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Objectives: This study aimed to investigate the onset time to habitual psychogenic non-epileptic seizures (PNES) in adults referred to Guy's and St Thomas' Neurophysiology Department for home video telemetry (HVT) with a clinical question of PNES. The primary objective was to determine the optimal time window for HVT recording for patients with suspected PNES to try to improve the allocation of clinical resources. The secondary objective was to explore any potential association between time to habitual PN ES onset and demographic indexes and other clinical, neuro-radiological and semiological findings. Methods: We performed a retrospective analysis of our XLTEK database between 2019 and 2020. A multifactorial analysis of PNES semiologic subtypes, patient demographics, psychiatric comorbidities and neuroimaging was conducted to explore their impact on time to PNES within an HVT study. People who had at least one typical PNES during their recording were included. The exclusion criteria included people who had the test performed without video recording. The total number of participants was 37. The data were extracted from our local XLTEK database. Statistical analyses using Mann–Whitney U and Fischer exact tests were carried out. Results: The mean time to first habitual PNES onset was seven hours, with a mean recording duration of 46 h. The most commonly occurring event type was blank spells (12, 32%), with the least common presentation being déjà vu (1, 3%). There was a significant association between time to PNES onset and male sex (p = 0.04). There was a significant association between time to PNES onset and abnormal MRI findings (p = 0.02). Particular PNES semiologic subtypes were not significantly linked with PNES onset time. Conclusions: Our study highlights that on average, patients with PNES will rapidly have their first habitual event within an HVT study (mean time to event onset of seven hours), consistent with the current literature. This raises the question of whether HVT study duration could be reduced to release study resources and aid departmental efficiencies. We also observe the novel finding that men presented significantly earlier with their habitual PNES event than women, and that abnormal imaging findings were also significantly associated with an earlier time to event onset, although the reason for this association is yet to be determined. [ABSTRACT FROM AUTHOR]

Background/Objectives: Increasing evidence shows an involvement of brain plasticity mechanisms in both motor and central manifestations of neuromuscular disorders (NMDs). These mechanisms could be specifically addressed with neuromodulation or rehabilitation protocols. The aim of this scoping review is to summarise the evidence on plasticity mechanisms' involvement in NMDs to encourage future research. Methods: A scoping review was conducted searching the PubMed and Scopus electronic databases. We selected papers addressing brain plasticity and central nervous system (CNS) studies through non-invasive brain stimulation techniques in myopathies, muscular dystrophies, myositis and spinal muscular atrophy. Results: A total of 49 papers were selected for full-text examination. Regardless of the variety of pathogenetic and clinical characteristics of NMDs, studies show widespread changes in intracortical inhibition mechanisms, as well as disruptions in glutamatergic and GABAergic transmission, resulting in altered brain plasticity. Therapeutic interventions with neurostimulation techniques, despite being conducted only anecdotally or on small samples, show promising results; Conclusions: despite challenges posed by the rarity and heterogeneity of NMDs, recent evidence suggests that synaptic plasticity may play a role in the pathogenesis of various muscular diseases, affecting not only central symptoms but also strength and fatigue. Key questions remain unanswered about the role of plasticity and its potential as a therapeutic target. As disease-modifying therapies advance, understanding CNS involvement in NMDs could lead to more tailored treatments. [ABSTRACT FROM AUTHOR]

The reactivity of an electroencephalogram (EEG) to external stimuli is impaired in comatose patients showing burst-suppression (BS) patterns following hypoxic–ischemic brain injury (HIBI). We explored the reactivity of BS induced by isoflurane in rat models of HIBI and controls using intermittent photic stimulation (IPS) delivered to one eye. The relative time spent in suppression referred to as the suppression ratio (SR) was measured on the contralateral fronto-occipital cortical EEG channel. The BS reactivity (BSR) was defined as the decrease in the SR during IPS from the baseline before stimulation (SRPRE). We found that BSR increased with SRPRE. To standardize by anesthetic depth, we derived the BSR index (BSRi) as BSR divided by SRPRE. We found that the BSRi was decreased at 3 days after transient global cerebral ischemia in rats, which is a model of brain injury after cardiac arrest. The BSRi was also reduced 2 months after experimental perinatal asphyxia in rats, a model of birth asphyxia, which is a frequent neonatal complication in humans. Furthermore, Oxytocin attenuated BSRi impairment, consistent with a neuroprotective effect in this model. Our data suggest that the BSRi is a promising translational marker in HIBI which should be considered in future neuroprotection studies. [ABSTRACT FROM AUTHOR]

Over the past three decades, substantial advancements have occurred in non-invasive brain stimulation (NIBS). These developments encompass various non-invasive techniques aimed at modulating brain function. Among the most widely utilized methods today are transcranial magnetic stimulation (TMS) and transcranial electrical stimulation (TES), which include direct- or alternating-current transcranial stimulation (tDCS/tACS). In addition to these established techniques, newer modalities have emerged, broadening the scope of non-invasive neuromodulation approaches available for research and clinical applications in movement disorders, particularly for Parkinson's disease (PD) and, to a lesser extent, atypical Parkinsonism (AP). All NIBS techniques offer the opportunity to explore a wide range of neurophysiological mechanisms and exert influence over distinct brain regions implicated in the pathophysiology of Parkinsonism. This paper's first aim is to provide a brief overview of the historical background and underlying physiological principles of primary NIBS techniques, focusing on their translational relevance. It aims to shed light on the potential identification of biomarkers for diagnostic and therapeutic purposes, by summarising available experimental data on individuals with Parkinsonism. To date, despite promising findings indicating the potential utility of NIBS techniques in Parkinsonism, their integration into clinical routine for diagnostic or therapeutic protocols remains a subject of ongoing investigation and scientific debate. In this context, this paper addresses current unsolved issues and methodological challenges concerning the use of NIBS, focusing on the importance of future research endeavours for maximizing the efficacy and relevance of NIBS strategies for individuals with Parkinsonism. [ABSTRACT FROM AUTHOR]

Main Topic: Artificial Intelligence and Digitalization: Applications to Neurosurgery and Neuroradiology. On behalf of the SSNS and SSNR, we are pleased to present the Abstracts of the Joint Annual Meeting, which is held at the Congress Kursaal Interlaken, Switzerland, 20–21 June 2024. In total, 62 abstracts were selected, of which 19 abstracts are oral presentations and 43 abstracts are for ePoster. We congratulate all the presenters on their research work and contribution. [ABSTRACT FROM AUTHOR]

On behalf of the SNS, we are pleased to present the Abstracts of the Annual Meeting which is held at the Congress Center in Basel, Switzerland, from 6–7 June 2024. In total, 83 abstracts were selected, whereof we include 8 abstracts for the Plenary Sessions, 6 abstracts for the SAYN GemSession, 30 abstracts for Poster flash presentations, and 39 abstracts as ePosters. We congratulate all the presenters on their research work and contributions. [ABSTRACT FROM AUTHOR]

Background: Clinical neurophysiology (CNP) involves the use of neurophysiological techniques to make an accurate clinical diagnosis, to quantify the severity, and to measure the treatment response. Despite several studies showing CNP to be a useful diagnostic tool in Movement Disorders (MD), its more widespread utilization in clinical practice has been limited. Objectives: To better understand the current availability, global perceptions, and challenges for implementation of diagnostic CNP in the clinical practice of MD. Methods: The International Parkinson and Movement Disorders Society (IPMDS) formed a Task Force on CNP. The Task Force distributed an online survey via email to all the members of the IPMDS between August 5 and 30, 2021. Descriptive statistics were used for analysis of the survey results. Some results are presented by IPMDS geographical sections namely PanAmerican (PAS), European (ES), African (AFR), Asian and Oceanian (AOS). Results: Four hundred and ninety‐one IPMDS members (52% males), from 196 countries, responded. The majority of responders from the AFR (65%) and PAS (63%) sections had no formal training in diagnostic CNP (40% for AOS and 37% for ES). The most commonly used techniques are electroencephalography (EEG) (72%) followed by surface EMG (71%). The majority of responders think that CNP is somewhat valuable or very valuable in the assessment of MD. All the sections identified "lack of training" as one of the biggest challenges for diagnostic CNP studies in MD. Conclusions: CNP is perceived to be a useful diagnostic tool in MD. Several challenges were identified that prevent widespread utilization of CNP in MD. [ABSTRACT FROM AUTHOR]

The clinical neurophysiological tests allow us to determine the type, extent, and nature of brachial plexus damage. They are crucial in decision making regarding surgical procedures or conservative treatment. This report aimed to present an algorithm for rehabilitation procedures in patients with brachial plexus injury of various origins based on the results of neurophysiology findings for the selection of procedures supporting the process of nerve and muscle regeneration. The research group consisted of patients whose medical documentation was analysed concerning the reason, level, and localization of damage to the brachial plexus structures, surgical and or rehabilitative treatment, as well as the MRI results. Among the group of fourteen patients, the clinical studies showed the greatest incidence of brachial plexus injuries of the mixed (both pre- and postganglionic), all trunks, and cervical root injuries, respectively. Results of the motor evoked potentials (MEP) and electroneurography (ENG) recordings induced at levels of spinal roots and Erb's point showed a decrease of more than 40% in amplitudes on the symptomatic side in comparison to the asymptomatic side. This diffeence was recorded for the axillary and radial innervation and the C5, C6, and C7 root domains, ranging from 57% to 66%; the lowest decrease was recorded following electrical stimulation at Erb's point for the ulnar nerve (34%). The latency prolongation on the symptomatic side in CMAP and MEP tests ranged from 0.2 to 1.7 ms, with the most following magnetic stimulation of the C5 cervical root for the axillary innervation. Most of the results indicated the axonotmesis and neuropraxia type of injury in motor fibers (40%) confirmed by EMG results. The sensory conduction studies (SNCS) in distal nerve branches did not confirm the severe advancement of the brachial plexus injury (63%). The proposed algorithm of the physiotherapeutic procedures should be mainly targeted for recovery of motor dysfunction as the consequence of brachial plexus injury. Rehabilitation should incorporate the treatment supporting nerve regeneration, muscle strengthening, and maintaining functional ranges of motion of the injured extremities. The rehabilitation treatment for patients with brachial plexus injuries is an individualised process, and the selection of procedures and the effectiveness of the treatment undertaken should be confronted with results of neurophysiological tests verifying the motor neural transmission from the level of the cervical motor centre to the effector, peripheral nerve function, and muscle's motor unit activity. [ABSTRACT FROM AUTHOR]

We proposed that the brain's electrical activity is composed of a sequence of alternating states with repeating topographic spectral distributions on scalp electroencephalogram (EEG), referred to as oscillatory macrostates. The macrostate showing the largest decrease in the probability of occurrence, measured as a percentage (reactivity), during sensory stimulation was labelled as the default EEG macrostate (DEM). This study aimed to assess the influence of awareness on DEM reactivity (DER). We included 11 middle cerebral artery ischaemic stroke patients with impaired awareness having a median Glasgow Coma Scale (GCS) of 6/15 and a group of 11 matched healthy controls. EEG recordings were carried out during auditory 1 min stimulation epochs repeating either the subject's own name (SON) or the SON in reverse (rSON). The DEM was identified across three SON epochs alternating with three rSON epochs. Compared with the patients, the DEM of controls contained more posterior theta activity reflecting source dipoles that could be mapped in the posterior cingulate cortex. The DER was measured from the 1 min quiet baseline preceding each stimulation epoch. The difference in mean DER between the SON and rSON epochs was measured by the salient EEG reactivity (SER) theoretically ranging from −100% to 100%. The SER was 12.4 ± 2.7% (Mean ± standard error of the mean) in controls and only 1.3 ± 1.9% in the patient group (P < 0.01). The patient SER decreased with the Glasgow Coma Scale. Our data suggest that awareness increases DER to SON as measured by SER. [ABSTRACT FROM AUTHOR]

Background: Dementia and mild cognitive impairment are characterised by symptoms of cognitive decline, which are typically assessed using neuropsychological assessments (NPAs), such as the Mini-Mental State Examination (MMSE) and Frontal Assessment Battery (FAB). Magnetoencephalography (MEG) is a novel clinical assessment technique that measures brain activities (summarised as oscillatory parameters), which are associated with symptoms of cognitive impairment. However, the relevance of MEG and regional cerebral blood flow (rCBF) data obtained using single-photon emission computed tomography (SPECT) has not been examined using clinical datasets. Therefore, this study aimed to investigate the relationships among MEG oscillatory parameters, clinically validated biomarkers computed from rCBF, and NPAs using outpatient data retrieved from hospital records. Methods: Clinical data from 64 individuals with mixed pathological backgrounds were retrieved and analysed. MEG oscillatory parameters, including relative power (RP) from delta to high gamma bands, mean frequency, individual alpha frequency, and Shannon's spectral entropy, were computed for each cortical region. For SPECT data, three pathological parameters--'severity', 'extent', and 'ratio'--were computed using an easy z-score imaging system (eZIS). As for NPAs, the MMSE and FAB scores were retrieved. Results: MEG oscillatory parameters were correlated with eZIS parameters. The eZIS parameters associated with Alzheimer's disease pathology were reflected in theta power augmentation and slower shift of the alpha peak. Moreover, MEG oscillatory parameters were found to reflect NPAs. Global slowing and loss of diversity in neural oscillatory components correlated with MMSE and FAB scores, whereas the associations between eZIS parameters and NPAs were sparse. Conclusion: MEG oscillatory parameters correlated with both SPECT (i.e. eZIS) parameters and NPAs, supporting the clinical validity of MEG oscillatory parameters as pathological and symptomatic indicators. The findings indicate that various components of MEG oscillatory characteristics can provide valuable pathological and symptomatic information, making MEG data a rich resource for clinical examinations of patients with cognitive impairments. SPECT (i.e. eZIS) parameters showed no correlations with NPAs. The results contributed to a better understanding of the characteristics of electrophysiological and pathological examinations for patients with cognitive impairments, which will help to facilitate their co-use in clinical application, thereby improving patient care. [ABSTRACT FROM AUTHOR]

Purpose: Glioma is associated with pathologically high (peri)tumoral brain activity, which relates to faster progression. Functional connectivity is disturbed locally and throughout the entire brain, associating with symptomatology. We, therefore, investigated how local activity and network measures relate to better understand how the intricate relationship between the tumor and the rest of the brain may impact disease and symptom progression. Methods: We obtained magnetoencephalography in 84 de novo glioma patients and 61 matched healthy controls. The offset of the power spectrum, a proxy of neuronal activity, was calculated for 210 cortical regions. We calculated patients' regional deviations in delta, theta and lower alpha network connectivity as compared to controls, using two network measures: clustering coefficient (local connectivity) and eigenvector centrality (integrative connectivity). We then tested group differences in activity and connectivity between (peri)tumoral, contralateral homologue regions, and the rest of the brain. We also correlated regional offset to connectivity. Results: As expected, patients' (peri)tumoral activity was pathologically high, and patients showed higher clustering and lower centrality than controls. At the group-level, regionally high activity related to high clustering in controls and patients alike. However, within-patient analyses revealed negative associations between regional deviations in brain activity and clustering, such that pathologically high activity coincided with low network clustering, while regions with 'normal' activity levels showed high network clustering. Conclusion: Our results indicate that pathological activity and connectivity co-localize in a complex manner in glioma. This insight is relevant to our understanding of disease progression and cognitive symptomatology. [ABSTRACT FROM AUTHOR]

On behalf of the SNS and SSNS, we are pleased to present the abstracts of the Joint Annual Meeting to be held in Zurich, Switzerland, on 23–24 November, 2023. In total, 119 abstracts have been selected: 4 abstracts for the Plenary Sessions, 12 abstracts for Free Communications, 18 abstracts for Poster flash presentations, 6 abstracts for the SAYN GemSession, and 79 abstracts as ePosters. We congratulate all the presenters on their research work and contributions. [ABSTRACT FROM AUTHOR]

The present study aimed to assess the function of muscular and nervous systems in high-maneuvering jet fighters with the chosen method of clinical neurophysiology, which methodologically has not yet been presented in detail. Fifteen pilots with the experience of more than 1700 flying hours at 7G overloading on average and fifteen healthy subjects for the comparison of health status declared to participate in this study. The dermatomal perception from C4 to S1 was examined using von Frey's filaments tactile method. Surface electromyography (sEMG) recordings examined the activity of proximal and distal muscles in the upper and lower extremities, the transmission of motor nerve impulses peripherally was diagnosed by electroneurography (ENG), the efferent transmission from C5–C7 and L4–L5 spinal centers to muscles was entirely verified with recordings of motor-evoked potentials induced oververtebrally with the magnetic field (MEP). The pilots estimated more lumbosacral than cervical pain at about 2 on the 10-point visual analog scale (VAS). Sensory perception studies did not reveal abnormal symptoms in the C2–S1 dermatomes innervation. Clinical neurophysiology studies indicated, in general, the lack of pathology during sEMG tests in comparison to healthy subjects or even better muscle motor unit contractile properties in pilots, both in the upper and lower extremities. In pilots, the parameters of ENG and MEP examinations show a statistically significant sensitivity for detecting the slight changes and their consequences in the transmission of neural impulses within L4–L5 ventral root fibers. The research results enable specifying the algorithm of future preventing rehabilitative treatment in high-maneuvering jet fighters with an average flight experience of 2000 h and working conditions at 7G on average. This study, for the first time, describes the application of a set of diagnostic neurophysiological methods with the particular importance of MEPs in the clinical evaluation of the jet fighters' health status. [ABSTRACT FROM AUTHOR]

Introduction: Even if electromyography (EMG) is routinely used to confirm the diagnosis of amyotrophic lateral sclerosis (ALS), few studies have analysed the correlation between electrophysiological parameters and clinical characteristics of ALS. We assessed if the quantification of active denervation (AD) and chronic denervation (CD) provides clinicians with information about phenotype, disease progression and survival in ALS patients. Methods: We studied a cohort of 689 ALS patients recording the following parameters: age and site of onset, survival, MRC scale for muscle strength evaluation, burden of upper and lower motor signs as measured with specific scales (PUMNS and LMNS, respectively), ALSFRS-R, progression rate (ΔFS), MITOS and King's Staging systems (KSS). We performed EMG on 11 muscles, and calculated semiquantitative AD and CD scores for each limb, as well as for the bulbar and spinal regions. Results: We found a positive correlation between AD and CD scores with LMNS (respectively p = 4.4 × 10–37 and p = 2.8 × 10–45) and a negative correlation with MRC (respectively p = 4.5 × 10–35 and p = 3.0 × 10–35). Furthermore, patients with higher spinal AD and CD scores had significantly lower ALSFRS-R scores, and higher KSS and MITOS stages. Conversely, only AD was associated to higher ΔFS (p = 1.0 × 10–6) and shorter survival (p = 1.1 × 10–5). Conclusion: Our results confirmed that EMG examination represents not only a diagnostic instrument, but also a prognostic tool. In this context, AD seems to be a reliable predictor of disease's progression and survival while CD better describes functional disability. [ABSTRACT FROM AUTHOR]

Objective: to study the clinical and neurophysiological features in the Chornobyl clean-up workers with a verified chronic cerebrovascular disease/cerebral small vessels disease (SVD) exposed to low doses of ionizing radiation (IR), employees of the Chornobyl Nuclear Power Plant (SSE ChNPP), who were exposed to the stress factor of a full-scale war as a result of being held captive by the Russian military at their workplaces, and individuals of the non-irradiated comparison group. Design, object and methods. A cross-sectional clinical study with parallel external control groups. We studied and carried out an expert statistical analysis of the clinical and neurophysiological characteristics of 62 male subjects, from which three examination groups were formed: 1) a randomized sample from the Clinical Epidemiological Register (CER) of the State Institution «National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine» (NRCRM) of 22 Chornobyl clean-up workers ('Chornobyl liquidators') in 1986-1990, examined during 2020-2021, aged 50-68 years at the time of examination (M ± SD: (58.1 ± 5.2) years) with a documented external radiation dose of 0.03-2.30 Sv; 2) 24 SSE ChNPP employees exposed to the stress factor impact of a full-scale war as a result of being held captive by the Russian military at their workplaces. The average age of the examined was (54.5 ± 5.8) years (range 46-71 years). 3) Comparison group -- 16 non-exposed men with verified chronic cerebrovascular disease/cerebral small vessels disease (SVD). The average age of the examined was (57.8 ± 5.6) years (range 50-70 years). Results. Neurophysiological studies confirm the presence of the pronounced dysfunction of the cortico-limbic system of the left dominant hemisphere of the brain with special involvement of the hippocampus in the Chornobyl clean-up workers. In the SSE ChNPP group, for the first time, the disorders of cerebral neurodynamics were detected in the form of functional hypofrontality and hyperfunction of the cortico-limbic system with lateralization to the right hemisphere. Conclusions. Today there exists a long-term thorough methodological and evidence base for a possible neurophysiological diagnosis and differential diagnosis of the combined cerebral effects of IR and psycho-emotional stress associated with the conditions of military conflicts. Neurophysiological technologies can be used in the objective professional and qualification selection of employees in a number of professions that require quick and responsible decision-making. Employees of SSE ChNPP need further medical and psychological support due to an increased risk of developing mental health disorders. [ABSTRACT FROM AUTHOR]

Background: Patients with Parkinson's disease (PD) and rest tremor may also have tremor during posture holding, with tremor being transiently suppressed during the transition between resting and posture holding. Other PD patients show no tremor suppression between resting and posture holding. The mechanisms responsible for tremor suppression in PD are unknown. Understanding the mechanisms of tremor suppression would expand our knowledge of tremor pathophysiology in PD. Objective: To investigate whether tremor suppression reflects the activity of the primary motor cortex (M1) and assess whether tremor features are different in patients with and without tremor suppression. Methods: We compared corticomuscular coherence (CMC) at tremor frequency and transcranial magnetic stimulation tremor resetting between 10 PD patients with tremor suppression and 10 patients without suppression. We also compared tremor spectral features between the two groups. Results: Patients with tremor suppression had higher CMC at tremor frequency during both rest tremor and postural tremor, and a higher postural tremor resetting index and stability when compared with patients without tremor suppression. Rest tremor frequency was similar between the two groups, but postural tremor frequency was lower in patients with tremor suppression as compared to patients without. Conclusion: M1 plays a major role in tremor suppression in PD, and the mechanisms of postural tremor may differ between patients with and without tremor suppression. [ABSTRACT FROM AUTHOR]

Background and purpose: We evaluated the clinical and neurophysiological efficacy of rituximab (RTX) in a neurophysiologically homogeneous group of patients with monoclonal gammopathy and immunoglobulin M (IgM) anti‐myelin‐associated glycoprotein antibody (anti‐MAG) demyelinating polyneuropathy. Methods: Twenty three anti‐MAG‐positive polyneuropathic patients were prospectively evaluated before and for 2 years after treatment with RTX 375 mg/m2. The Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale (INCAT‐ds), modified INCAT sensory score (mISS), Medical Research Council sum score, Patients' Global Impression of Change scale were used, IgM levels were assessed and extensive electrophysiological examinations were performed before (T0) and 1 year (T1) and 2 years (T2) after RTX treatment. Results: At T1 and T2 there was a significant reduction from T0 both in mISS and in INCAT‐ds, with a p value < 0.001 in the inferential Friedman's test overall analysis. Ulnar nerve Terminal Latency Index and distal motor latency significantly changed from T0 to T1 and in the overall analysis (p = 0.001 and p = 0.002), and ulnar nerve sensory nerve action potential (SNAP) amplitude was significantly increased at T2 from T1, with a p value < 0.001 in the overall analysis. Analysis of the receiver‐operating characteristic curves showed that a 41.8% increase in SNAP amplitude in the ulnar nerve at T2 from T0 was a fair predictor of a mISS reduction of ≥2 points (area under the curve 0.85; p = 0.005; sensitivity: 90.9%, specificity: 83.3%). Conclusions: This study suggests that RTX is effective in patients with clinically active demyelinating anti‐MAG neuropathy over 2 years of follow‐up, and that some neurophysiological variables might be useful for monitoring this efficacy. [ABSTRACT FROM AUTHOR]

Clinical neurophysiology constructs a wealth of dynamic information pertaining to the integrity and function of both central and peripheral nervous systems. As with many technological fields, there has been an explosion of data in neurophysiology over recent years, and this requires considerable analysis by experts. Computational algorithms and especially advances in machine learning (ML) have the ability to assist with this task and potentially reveal hidden insights. In this update article, we will provide a brief overview where such technology is being applied in clinical neurophysiology and possible future directions. [ABSTRACT FROM AUTHOR]

The current concepts on the clinical neurophysiology examinations for the differential diagnosis of rheumatic diseases are presented. The review aims to provide experience and practical guidelines, especially regarding electromyography. More needle than surface electromyography examinations at muscle rest or during its maximal contraction may reveal the characteristic effects of the myogenic injury caused by particular rheumatic diseases. The diagnosis of myopathic disorders, often found in rheumatic diseases is difficult because of the frequent vasculitis coexistence in the patients evoking subsequent changes in nerve fibres leading to degenerative neurogenic changes that may overlap the diagnostic picture of the primary myogenic changes caused by rheumatic diseases. In these cases, the neurophysiological studies of efferent and afferent neural transmission often reveal peripheral neuropathies just at the subclinical level. [ABSTRACT FROM AUTHOR]

Introduction: Clinical neurophysiology deals with nervous system functions assessed with electrophysiological and ultrasound-based imaging techniques. Even though the need for highly specialized neurophysiologists has increased, residency training rarely takes today's requirements into account. This study aimed to snapshot the neurophysiological training provided by Italian specialization schools in neurology.Methods: A single-page web-based survey comprising 13 multiple-choice categorical and interval scale questions was sent via e-mail to neurology specialization school directors. The survey addressed the programs' structural neurophysiology organization, time dedicated to each clinical neurophysiology subspecialty, and descriptors assessing the discipline's importance (e.g., residents who attempted residential courses, gained certifications, or awards gained).Results: The most studied neurophysiological techniques were electroencephalography (EEG) and electromyography (EMG). Most specialization schools devoted less than 3 months each to multimodal evoked potentials (EPs), ultrasound sonography (US), and intra-operative monitoring. Of the 35 specialization schools surveyed, 77.1% reported that four students, or fewer, participated in the Italian Society of Clinical Neurophysiology Examination in Neurophysiology. Of the 35 specialization centers surveyed, 11.4% declared that the final evaluation required students to discuss a neurophysiological test.Discussion: Our survey underlined the poorly standardized technical requirements in postgraduate neurology specialization schools, wide variability among training programs, and limited training on multi-modal evoked potentials, intraoperative monitoring, and sonography. These findings underline the need to reappraise and improve educational and training standards for clinical neurophysiology during postgraduate specialization schools in neurology with an international perspective. [ABSTRACT FROM AUTHOR]

Comparative studies on the second exteroceptive suppression period (ES2) of the masseter or temporalis muscle in migraineurs and controls have provided conflicting results. As the interneurons responsible for ES2 are probably close to the trigeminal nucleus caudalis and receive afferents also from the anti-nociceptive system, the study of ES2 could provide information on neural circuits involved in migraine pathophysiology. The aim of this observational, pilot study was to assess whether erenumab treatment may affect the exteroceptive suppression reflex of the temporalis muscle activity in migraineurs. The exteroceptive suppression reflex of the temporalis muscle activity was previously studied in a small case series of three chronic female migraineurs and after 4 months of beneficial erenumab treatment, administered according to current clinical indications. There was a statistically significant decrease in ES2 latency (p-value 0.039) and duration (p-value 0.030) after treatment. The change observed in the temporalis ES2 during erenumab treatment indicates that ES2 may play some kind of role as a neurophysiological marker and that this monoclonal antibody can modulate the brainstem circuits involved in migraine pathophysiology, at least indirectly. Further studies are required to confirm this intriguing hypothesis. [ABSTRACT FROM AUTHOR]

A concomitant presentation of relapsing remitting multiple sclerosis (RRMS) and amyotrophic lateral sclerosis (ALS) is quite rare. However, a review of the literature showed an increased co-occurrence of both diseases, including in genetically determined cases. We report the case of a 49-year-old woman with a history of RRMS who developed a progressive subacute loss of strength in her left arm. The patient's father died from ALS, and her paternal uncle had Parkinson's disease. Brain and cervical MRIs were performed, and new demyelinating lesions were excluded. Electromyography (EMG) of the upper limbs showed fibrillations and fasciculations in distal muscles of both arms. In the following months, the patient presented a progressive loss of strength in the proximal and distal muscles of the right arm and hyperreflexia in the lower limbs. EMG and central motor conduction were consistent with ALS. A genetic test was carried out, revealing a mutation in the FUS gene (exon 15; c. 1562 G>A). To our knowledge, the co-occurrence of MS and ALS in patients with FUS mutation is extremely rare. We hypothesize a common pathway for both diseases based on the possibility of a shared oligodendroglial dysfunction due to FUS mutation. [ABSTRACT FROM AUTHOR]

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Introduction/Aims: Neurogenic thoracic outlet syndrome (NTOS) is a heterogeneous and often disputed entity. An electrodiagnostic pattern of T1 > C8 axon involvement is considered characteristic for the diagnosis of NTOS. However, since the advent of high‐resolution nerve ultrasound (US) imaging, we have encountered several patients with a proven entrapment of the lower brachial plexus who showed a different, variable electrodiagnostic pattern. Methods: In this retrospective case series, 14 patients with an NTOS diagnosis with a verified source of compression of the lower brachial plexus and abnormal findings on their electrodiagnostic testing were included. Their medical records were reviewed to obtain clinical, imaging, and electrodiagnostic data. Results: Seven patients showed results consistent with the "classic" T1 axon > C8 pattern of involvement. Less typical findings included equally severe involvement of T1 and C8 axons, more severe C8 involvement, pure motor abnormalities, neurogenic changes on needle electromyography in the flexor carpi radialis and biceps brachii muscles, and one patient with an abnormal sensory nerve action potential (SNAP) amplitude for the median sensory response recorded from the third digit. Patients with atypical findings on electrodiagnostic testing underwent nerve imaging more often compared to patients with classic findings (seven of seven patients vs. five of seven respectively), especially nerve ultrasound. Discussion When there is a clinical suspicion of NTOS, an electrodiagnostic finding other than the classic T1 > C8 pattern of involvement does not rule out the diagnosis. High resolution nerve imaging is valuable to diagnose additional patients with this treatable condition. See Editorial on pages 4‐6 in this issue. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Neurophysiological patterns in patients with foodborne botulism are rarely described after the acute phase. We report data from a large Italian outbreak of botulism, with patients evaluated at different timepoints after poisoning. Methods: Eighteen male patients (mean age 47 ± 8.4 y) underwent 22 clinical and neurophysiological evaluations (4 patients were re‐evaluated). The resting compound muscle action potential (CMAP) amplitude, postexercise CMAP amplitude, CMAP change after high‐frequency (50 Hz) repetitive nerve stimulation (HFRNS), and motor unit action potentials (MUAPs) were assessed in the acute (4–8 days after poisoning; 5 patients), early post‐acute (32–39 days after poisoning; 5 patients), and late post‐acute (66–80 days after poisoning; 12 patients) phases. Results: In the acute, early post‐acute, and late post‐acute phases, respectively, reduced CMAP amplitudes were found in 100%, 20%, and 17% of patients; abnormal postexercise CMAP facilitation was observed in 100%, 40%, and 0% of patients; and pathological incremental responses to HFRNS were found in 80%, 50%, and 8% of patients. Baseline CMAP amplitudes, postexercise CMAP facilitation, and CMAP increases in response to HFRNS differed significantly between the acute and post‐acute phases. Small MUAPs were found in 100% of patients in the acute and early post‐acute phases and in 50% of patients in the late post‐acute phase. Discussion: The neurophysiological findings of foodborne botulism vary considerably according to the evaluation time point. In the post‐acute phase, different neurophysiological techniques must be applied to support a diagnosis of botulism. [ABSTRACT FROM AUTHOR]

Up to 48% of patients with medically unexplained symptoms seen in neurological practice suffer from sensory symptoms, which could be of functional nature or secondary to psychiatric disorders. These patients show high medical care utilization causing elevated healthcare costs. Despite the high prevalence, little is known about clinical characteristics and pathophysiological mechanisms. For functional disorders such as irritable bowel syndrome, a reduction of heart rate variability (HRV) has been shown, suggesting a dysfunction of the autonomic nervous system (ANS). The aim of this study was to investigate psychological data and functional changes of the ANS in patients with medically unexplained sensory symptoms (MUSS). In this exploratory pilot study, 16 patients (11 females, 31.6 ± 11.9 years) with MUSS, who were recruited at a single tertiary neurological center, underwent a structured clinical interview (SCID) to evaluate psychiatric comorbidities. Patients and age- and sex-matched healthy volunteers filled in questionnaires, and individual sensory thresholds (perception, pain) were detected by quantitative sensory testing (QST). HRV was assessed at baseline and under three different experimental conditions (tonic pain stimulus, placebo application, cold-face test). All tests were repeated after 6–8 weeks. SCID interviews revealed clinical or subclinical diagnoses of psychiatric comorbidities for 12 patients. Questionnaires assessing somatization, depression, anxiety, and perceived stress significantly discriminated between patients with MUSS and healthy controls. While there was no difference in QST, reduced ANS reactivity was found in patients during experimental conditions, particularly with regard to vagally mediated HRV. Our pilot study of neurological patients with MUSS reveals a high prevalence of psychiatric comorbidities and provides evidence for altered ANS function. Our data thus give insight in possible underlying mechanisms for these symptoms and may open the door for a better diagnostic and therapeutic approach for these patients in the future. [ABSTRACT FROM AUTHOR]

The functional anatomy of the anteromesial portion of the temporal lobe and its involvement in epilepsy can be explored by means of intracerebral electrical stimulations. Here, we aimed to expand the knowledge of its physiological and pathophysiological symptoms by conducting the first large‐sample systematic analysis of 1529 electrical stimulations of this anatomical region. We retrospectively analysed all clinical manifestations induced by intracerebral electrical stimulations in 173 patients with drug‐resistant focal epilepsy with at least one electrode implanted in this area. We found that high‐frequency stimulations were more likely to evoke electroclinical manifestations (p <.0001) and also provoked 'false positive' seizures. Multimodal symptoms were associated with EEG electrical modification (after discharge) (p <.0001). Visual symptoms were not associated with after discharge (p =.0002) and were mainly evoked by stimulation of the hippocampus (p =.009) and of the parahippocampal gyrus (p =.0212). 'False positive seizures' can be evoked by stimulation of the hippocampus, parahippocampal gyrus and amygdala, likely due to their intrinsic low epileptogenic threshold. Visual symptoms evoked in the hippocampus and parahippocampal gyrus, without EEG changes, are physiological symptoms and suggest involvement of these areas in the visual ventral stream. Our findings provide meaningful guidance in the interpretation of intracranial EEG studies of the temporal lobe. [ABSTRACT FROM AUTHOR]

Introduction: In everyday clinical neurophysiology practice, mononeuropathies are evaluated primarily by traditional electrodiagnostic testing. We sought to assess the additional benefit of neuromuscular ultrasound (US) in this scenario. Methods: All consecutive mononeuropathies undergoing combined US and electrodiagnostic evaluation over a 23‐mo period at a single neurophysiology practice were reviewed. Three independent examiners assessed how often US was: (a) "contributory" ‐ enabling a definite diagnosis not made by electrophysiology alone and/or impacting on the therapeutic decision, (b) "confirmatory" of the electrodiagnostic findings, but not adding further diagnostic or therapeutic information, or (c) "negative" ‐ missed the diagnosis. Results: There were 385 studies included. US was "contributory" in 36%, "confirmatory" in 61% and "negative" in 3%. Discussion: In this study of everyday neurophysiology practice, neuromuscular US contributed significant diagnostic or therapeutic information in over 1/3 of the investigations for common mononeuropathies. False negative US studies were uncommon in this setting. See Editorial on pages 437–438 in this issue. [ABSTRACT FROM AUTHOR]

Deep brain stimulation (DBS) of the subthalamic nucleus, pallidum, and thalamus is an established therapy for various movement disorders. Limbic targets have also been increasingly explored for their application to neuropsychiatric and cognitive disorders. The brainstem constitutes another DBS substrate, although the existing literature on the indications for and the effects of brainstem stimulation remains comparatively sparse. The objective of this review was to provide a comprehensive overview of the pertinent anatomy, indications, and reported stimulation-induced acute and long-term effects of existing white and grey matter brainstem DBS targets. We systematically searched the published literature, reviewing clinical trial articles pertaining to DBS brainstem targets. Overall, 164 studies describing brainstem DBS were identified. These studies encompassed 10 discrete structures: periaqueductal/periventricular grey (n = 63), pedunculopontine nucleus (n = 48), ventral tegmental area (n = 22), substantia nigra (n = 9), mesencephalic reticular formation (n = 7), medial forebrain bundle (n = 8), superior cerebellar peduncles (n = 3), red nucleus (n = 3), parabrachial complex (n = 2), and locus coeruleus (n = 1). Indications for brainstem DBS varied widely and included central neuropathic pain, axial symptoms of movement disorders, headache, depression, and vegetative state. The most promising results for brainstem DBS have come from targeting the pedunculopontine nucleus for relief of axial motor deficits, periaqueductal/periventricular grey for the management of central neuropathic pain, and ventral tegmental area for treatment of cluster headaches. Brainstem DBS has also acutely elicited numerous motor, limbic, and autonomic effects. Further work involving larger, controlled trials is necessary to better establish the therapeutic potential of DBS in this complex area. [ABSTRACT FROM AUTHOR]

Background: Stroke is a leading cause of disability. Nonetheless, the care pathway for stroke rehabilitation takes partially into account the needs of chronic patients. This is due in part to the lack of evidence about the mechanisms of recovery after stroke, together with the poor knowledge of related and influencing factors. Here we report on the study protocol "Rehabilitation and Biomarkers of Stroke Recovery," which consists of 7 work-packages and mainly aim to investigate the effects of long-term neurorehabilitation on stroke patients and to define a related profile of (clinical-biological, imaging, neurophysiological, and genetic-molecular) biomarkers of long-term recovery after stroke. The work-package 1 will represent the main part of this protocol and aims to compare the long-term effects of intensive self-rehabilitation vs. usual (rehabilitation) care for stroke. Methods: We planned to include a total of 134 adult subacute stroke patients (no more than 3 months since onset) suffering from multidomain disability as a consequence of first-ever unilateral ischemic stroke. Eligible participants will be randomly assigned to one of the following groups: intensive self-rehabilitation (based on the principles of "Guided Self-Rehabilitation Contract") vs. usual care (routine practice). Treatment will last 1 year, and patients will be evaluated every 3 months according to their clinical presentation. The following outcomes will be considered in the main work-package: Fugl-Meyer assessment, Cognitive Oxford Screen Barthel Index, structural and functional neuroimaging, cortical excitability, and motor and somatosensory evoked potentials. Discussion: This trial will deal with the effects of an intensive self-management rehabilitation protocol and a related set of biomarkers. It will also investigate the role of training intensity on long-term recovery after stroke. In addition, it will define a set of biomarkers related to post-stroke recovery and neurorehabilitation outcome in order to detect patients with greater potential and define long-term individualized rehabilitation programs. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT04323501. [ABSTRACT FROM AUTHOR]

Background and purpose: Some epilepsy syndromes (sleep‐related epilepsies, SREs) have a strong link with sleep. Comorbid sleep disorders are common in patients with SRE and can exert a negative impact on seizure control and quality of life. Our purpose was to define the standard procedures for the diagnostic pathway of patients with possible SRE (scenario 1) and the general management of patients with SRE and comorbidity with sleep disorders (scenario 2). Methods: The project was conducted under the auspices of the European Academy of Neurology, the European Sleep Research Society and the International League Against Epilepsy Europe. The framework entailed the following phases: conception of the clinical scenarios; literature review; statements regarding the standard procedures. For the literature search a stepwise approach starting from systematic reviews to primary studies was applied. Published studies were identified from the National Library of Medicine's MEDLINE database and Cochrane Library. Results: Scenario 1: Despite a low quality of evidence, recommendations on anamnestic evaluation and tools for capturing the event at home or in the laboratory are provided for specific SREs. Scenario 2: Early diagnosis and treatment of sleep disorders (especially respiratory disorders) in patients with SRE are likely to be beneficial for seizure control. Conclusions: Definitive procedures for evaluating patients with SRE are lacking. Advice is provided that could be of help for standardizing and improving the diagnostic approach of specific SREs. The importance of identifying and treating specific sleep disorders for the management and outcome of patients with SRE is underlined. [ABSTRACT FROM AUTHOR]