Concurrent transverse myelitis and acute inflammatory demyelinating polyneuropathy.

A woman in her 40s presented with thoracic banding dysaesthesia and lower motor neuron weakness. Spinal imaging revealed a short segment of transverse myelitis and neurophysiology was suggestive of concurrent acute inflammatory demyelinating polyneuropathy. The patient improved with consecutive intravenous immunoglobulin and methylprednisolone treatment. Acute inflammatory demyelinating polyneuropathy is a progressive immune-mediated peripheral neuropathy which responds to intravenous immunoglobulin or plasmapheresis, whereas transverse myelitis is a central inflammatory syndrome usually treated with corticosteroid. We highlight differentiating features of the clinical presentation and the utility of investigations such as neurophysiology and MRI along with a review of treatment and the role for corticosteroid therapy.

Fragile X-associated tremor/ataxia syndrome treated with multitarget deep brain stimulation.

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a progressive hereditary neurodegenerative disorder which causes intention tremor and cerebellar ataxia. It typically affects the ageing population. Deep brain stimulation (DBS) is widely accepted in the treatment of common movement disorders and has been trialled in treating rare and complex neurodegenerative disorders. We report a case of a man in his 40s with a long history of tremor affecting his hands. MRI brain revealed high T2 signal in the middle cerebellar peduncles. Genetic testing revealed FMR1 premutation confirming the diagnosis of FXTAS. Subsequently, he was treated with multitarget DBS of the ventralis intermediate nucleus and ventralis oralis posterior nuclei bilaterally, with excellent neurological function at 9 years follow-up. This case suggests multitarget DBS for FXTAS with neurophysiology-guided DBS programming can provide excellent long-term tremor suppression in selected patients.

Technical Tips: The Essential NeuroAnalyst: Creation of a Successful Program.

The NeuroAnalyst role is relatively new with the NA-CLTM credential first becoming available in 2021. Many institutions express interest in utilizing this new role in neurodiagnostic departments, but there is a relative dearth of information about the benefits and challenges of developing a NeuroAnalyst role to support clinical neurophysiologists. The aim of this article is to share the positive experience of one institution in developing a team of NeuroAnalysts. The addition of the role can decrease EEG report turnaround time and balance the workload of clinical neurophysiologists, which improves patient care and allows physicians to increase productivity in other ways.

Chronic Visual Abnormality in an Elderly Patient With Temporal Lobe Epilepsy.

A 79-year-old woman visited our department for chronic visual field abnormalities with a floating sensation for two months. Neurological and ophthalmologic examinations yielded normal results, except for brain MRI indicating left hippocampal atrophy. Cognitive function tests were normal. EEG revealed frequent spikes and slow waves in the left frontotemporal region, corroborated by reduced accumulation in 123I-iomazenil single photon emission computed tomography. A diagnosis of temporal lobe epilepsy was established, and treatment with lacosamide resulted in a remarkable improvement in symptoms and EEG findings. Mild focal seizures from the temporal region might cause mild impaired awareness, resulting in the patient's report as a sensation of uncertainty between the self and the outside world, mimicking ophthalmologic abnormalities. The repeated nature of the seizures contributed to the absence of the term "transient" in symptom description. Diagnosing epilepsy in the elderly proves challenging due to nonspecific complaints.

A randomized controlled educational pilot trial of interictal epileptiform discharge identification for neurology residents.

OBJECTIVE: To assess the effectiveness of an educational program leveraging technology-enhanced learning and retrieval practice to teach trainees how to correctly identify interictal epileptiform discharges (IEDs). METHODS: This was a bi-institutional prospective randomized controlled educational trial involving junior neurology residents. The intervention consisted of three video tutorials focused on the six IFCN criteria for IED identification and rating 500 candidate IEDs with instant feedback either on a web browser (intervention 1) or an iOS app (intervention 2). The control group underwent no educational intervention ("inactive control"). All residents completed a survey and a test at the onset and offset of the study. Performance metrics were calculated for each participant. RESULTS: Twenty-one residents completed the study: control (n = 8); intervention 1 (n = 6); intervention 2 (n = 7). All but two had no prior EEG experience. Intervention 1 residents improved from baseline (mean) in multiple metrics including AUC (.74; .85; p < .05), sensitivity (.53; .75; p < .05), and level of confidence (LOC) in identifying IEDs/committing patients to therapy (1.33; 2.33; p < .05). Intervention 2 residents improved in multiple metrics including AUC (.81; .86; p < .05) and LOC in identifying IEDs (2.00; 3.14; p < .05) and spike-wave discharges (2.00; 3.14; p < .05). Controls had no significant improvements in any measure. SIGNIFICANCE: This program led to significant subjective and objective improvements in IED identification. Rating candidate IEDs with instant feedback on a web browser (intervention 1) generated greater objective improvement in comparison to rating candidate IEDs on an iOS app (intervention 2). This program can complement trainee education concerning IED identification.

Walter Eichler and his role in the development of electroneurography.

Walter Eichler (1904-1942) performed the first in situ nerve conduction studies in humans. Eichler's work has been largely overlooked and there have been no biographical accounts written of him. His 1937 paper, Über die Ableitung der Aktionspotentiale vom menschlichen Nerven in situ (On the recording of the action potentials from human nerves in situ) was translated and reviewed. Archival material was obtained on his career that was housed predominantly at the University of Freiburg im Breisgau. He had memberships in Nazi organizations but did not appear to be politically active. During his brief career, he constructed novel equipment and established seminal principles for performing nerve conductions on humans. The authors repeated his experiment in the ulnar nerve, which duplicated Eichler's findings. His recordings were quite remarkable given advances in technology. In summary, the Eichler paper is the first study in the development of in situ clinical electroneurography in humans. Many of his procedural observations are still fundamental in the current practice of electroneurography. As best can be determined, his study in humans did not appear ethically compromised. Although Eichler's personal background remains open to question, his paper is a seminal study in the history and development of clinical electroneurography.Abbreviations: AP: Action potential; C: Capacitor; CNP: Compound nerve potential; DC: Direct current; E1: Preferred term for active electrode; E2: Preferred term for reference electrode; NSDÄB: Nationalsozialistische Deutsche NSD-Ärtzebund (National Socialist German Doctors' League; NSDAP: Nationalsozialistische Deutsche Arbeiterpartei (National Socialist German Workers' Party/ Nazi Party); SS: Schutzstaffel (Protective Echelon or Squad of the Nazi party).

Management of post blood patch severe rebound intracranial hypertension by the usage of an external ventricular drain.

Spontaneous intracranial hypotension (SIH) is a condition characterised by postural headaches due to low cerebrospinal fluid (CSF) pressure, often stemming from CSF leakage. Diagnosis poses a significant challenge, and the therapeutic approach encompasses both conservative measures and operative interventions, such as the epidural blood patch (EBP). However, EBP carries the potential risk of inducing rebound intracranial hypertension (RIH), subsequently leading to high-pressure headaches. We present a case wherein RIH following EBP was effectively managed through the implementation of an external ventricular drain (EVD) aimed at reducing CSF pressure. The patient improved significantly, underscoring the potential utility, if not necessity, of EVD in carefully selected cases, highlighting the imperative for further research to enhance the management of SIH and optimise EBP-related complications.

Oscillatory characteristics of resting-state magnetoencephalography reflect pathological and symptomatic conditions of cognitive impairment.

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.

Clinical neurophysiology of functional motor disorders: IFCN Handbook Chapter.

Functional Motor Disorders are common and disabling. Clinical diagnosis has moved from one of exclusion of other causes for symptoms to one where positive clinical features on history and examination are used to make a "rule in" diagnosis wherever possible. Clinical neurophysiological assessments have developed increasing importance in assisting with this positive diagnosis, not being used simply to demonstrate normal sensory-motor pathways, but instead to demonstrate specific abnormalities that help to positively diagnose these disorders. Here we provide a practical review of these techniques, their application, interpretation and pitfalls. We also highlight particular areas where such tests are currently lacking in sensitivity and specificity, for example in people with functional dystonia and functional tic-like movements.

The relationship between pathological brain activity and functional network connectivity in glioma patients.

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.

Idiopathic membranous nephropathy and synchronous mononeuritis multiplex secondary to idiopathic small vessel vasculitis.

Membranous nephropathy has been associated with demyelinating polyneuropathies and antiglomerular membrane disease; however, an association with vasculitic neuropathy has not been described. This case describes a patient with biopsy-proven idiopathic membranous nephropathy and synchronous mononeuritis multiplex secondary to idiopathic small vessel vasculitis, who presented with lower limb microvascular ischaemia, peripheral neuropathy and active urinary sediment. Her extensive non-invasive screening for immunological disease and radiological investigations for occult malignancy were unremarkable. The patient received intravenous methylprednisolone and intravenous rituximab induction therapy resulting in complete remission of both the idiopathic membranous nephropathy and small vessel vasculitis at 7 months post treatment.

Global Perceptions and Utilization of Clinical Neurophysiology in Movement Disorders.

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.

Guillain-Barré syndrome following primary cytomegalovirus infection in a patient with liver transplantation.

We present the case of a man in his 60s with a 5-month medical history of deceased donor liver transplantation, who developed Guillain-Barré syndrome (GBS) secondary to a primary cytomegalovirus (CMV) infection. This was confirmed by molecular tests and serology antibodies that ruled out other frequent aetiologies. Therapy with intravenous immunoglobulin and valganciclovir was started and the patient gradually improved over the weeks. GBS is the most common aetiology of paralysis worldwide, and it is an autoimmune-mediated neuropathy that is frequently caused by a preceding infection. Few cases of GBS have been reported in the context of liver transplant recipients, and those related to CMV infection are extremely rare. This case highlights the importance of considering GBS as a possible differential diagnosis in patients with solid organ transplantation, and it contributes to the knowledge of other infrequent aetiologies of this condition.

Towards an electroencephalographic measure of awareness based on the reactivity of oscillatory macrostates to hearing a subject's own name.

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.

The necessity of electrodiagnostic studies and ultrasound in ulnar nerve entrapment according to surgeons in the Netherlands.

BACKGROUND: Ulnar nerve entrapment at the elbow (UNE) is the second most prevalent entrapment neuropathy after carpal tunnel syndrome. The objective of this study was to evaluate the expert opinion of different surgical disciplines regarding the need for electrodiagnostic or ultrasound confirmation of UNE and, if so, which test was preferred for confirmation. METHODS: A questionnaire was sent to all neurosurgeons and plastic or hand surgeons in the Netherlands to evaluate the current practice in planning surgical treatment of UNE. RESULTS: The response rate was 36.4 % (134 out of 368). 94 % of surgeons reported that > 95 % of their patients had EDX or ultrasound studies before surgery. 80.6 % of all surgeons who responded reported that they seldom operated on UNE without electrodiagnostic confirmation. Hand surgeons (25.9 %) were more willing to operate on clinically diagnosed UNE without EDX than neurosurgeons (9.4 %) CONCLUSIONS: Dutch surgeons prefer diagnostic confirmation of UNE either by ultrasound or EDX, with a preference for EDX and the vast majority of operated patients do have either EDX or ultrasound or both before surgery. Compared to neurosurgeons, hand surgeons are more willing to operate on patients with clinically defined UNE but normal electrodiagnostic studies.

Identification and reduction of retained primitive reflexes by sensory stimulation in autism spectrum disorder: effects on qEEG networks and cognitive functions.

Several authors have reported finding retained primitive reflexes (RPRs) in individuals with autism spectrum disorders (ASD). This case report describes the reduction of RPRs and changes in cognitive function after transcutaneous electrical nerve stimulation (TENS) of muscle. Three individuals were examined in a study at the Institute for Neurology and Neurosurgery in Havana, Cuba. Two child neurologists, not involved in the study, conducted clinical examinations on each participant and diagnosed each with ASD based on DSM-V criteria and the Autism Diagnostic Interview-Revised (an autism evaluation tool). Each child with ASD possessed a triad of impairments in three domains: social interaction, communication, and repetitive behaviour. Individuals were evaluated by quantitative electroencephalographic measures and tested by standardised cognitive function tests before and after 12 weeks of intervention. These interventions were associated with reduced ASD symptoms in the three domains, significant changes in qEEG network connectivity and significantly improved performance on standardised cognitive tests.

NEUROPHYSIOLOGICAL BASIS OF THE COMBINED EFFECTS OF ACUTE STRESS AND LOW DOSES OF IONIZING RADIATION ON HUMAN BRAIN. / НЕЙРОФІЗІОЛОГІЧНИЙ БАЗИС КОМБІНОВАНИХ ЕФЕКТІВ ГОСТРОГО СТРЕСУ ТА МАЛИХ ДОЗ ІОНІЗУЮЧОГО ВИПРОМІНЮВАННЯ НА ГОЛОВНИЙ МОЗОК ЛЮДИНИ.

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 Ukraine¼ (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.

Occult bowel cancer presenting as Morvan syndrome.

A man in his mid-60s presented with a 3-month history of progressive muscle twitching, agitation, cognitive impairment, insomnia, hyperhidrosis and lower limb pain. He had fasciculations, myokymia, myoclonus, exaggerated startle response and significant postural hypotension. Electrophysiological studies showed evidence of peripheral nerve hyperexcitability with neuromyotonia. Contactin-associated protein-like 2 antibodies (CASPR2) were strongly positive. A diagnosis of Morvan syndrome was made. CT of the chest, abdomen and pelvis was undertaken to identify any occult malignancy, and a large bowel carcinoma in situ was identified and resected. His central nervous system and autonomic symptoms significantly improved following surgery, but neuromyotonia persisted, and this was treated with intravenous immunoglobulins and steroids. Early detection of bowel cancer in this patient enabled curative treatment.

A proposal for harmonizing clinical neurophysiology training in the Europe, Middle East and Africa Chapter of the International Federation of Clinical Neurophysiology.

There are significant differences in duration and intensity of clinical neurophysiology specialty training within the countries of the Europe, Middle East and Africa Chapter of the International Federation of Clinical Neurophysiology. We address these differences by proposing recommendations which may facilitate harmonisation of training and education within the Chapter. They arose from two workshops whose recommendations were then circulated widely within national societies in the Chapter for feedback and for consensus. The recommendations are applicable to clinical neurophysiology as a medical monospecialty and/or as a subspecialty (usually of neurology). We make a number of recommendations on governance and regulation of training, on the requirements for competence and the numbers of various examinations and tests performed by trainees, some under supervision. We also recommend a modular approach considering primary and complementary modules. Primary modules are electroencephalography, electromyography, nerve conduction studies and evoked potentials, while complementary ones include sleep analysis, intraoperative monitoring, small fibre testing, peripheral nerve and muscle ultrasound, intracortical recordings, and analysis of movement disorders. It is recommended that national examinations should include a variety of techniques to assess knowledge and judgement, practical skills, teamwork, communication skills, as well as safety and quality. The aim of the suggested recommendations is to harmonize clinical neurophysiology training in the member societies throughout the Chapter. It is realised that this may mean that the numbers for competence are aspirational for some, though ways to mitigate this, for instance through supranational training centres, are also discussed.