6,163 results match your criteria: "Department of Clinical Neurophysiology.[Affiliation]"

Objectives: As epilepsy management medical devices emerge as potential technological solutions for prediction and prevention of sudden death in epilepsy (SUDEP), there is a gap in understanding the features and priorities that should be included in the design of these devices. This study aims to bridge the gap between current technology and emerging needs by leveraging insights from persons with epilepsy (PWE) and caregivers (CG) on current epilepsy management devices and understanding how SUDEP awareness influences preferences and design considerations for potential future solutions.

Methods: Two cross-sectional surveys were designed to survey PWE and CG on medical device design features, SUDEP awareness, and participation in medical device research.

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Optical Genome Mapping Identifies a Second Xq27.1 Rearrangement Associated With Charcot-Marie-Tooth Neuropathy CMTX3.

Mol Genet Genomic Med

September 2024

Laboratory of Cancer Genetics and Tumor Biology, Translational Medicine Research Unit, Medical Research Center Oulu and Biocenter Oulu, University of Oulu, Oulu, Finland.

Article Synopsis
  • - The study focuses on X-linked recessive type 3 Charcot-Marie-Tooth (CMTX3), a rare condition with a common genetic insertion found in affected patients.
  • - Optical genome mapping (OGM) in a male patient with symptoms similar to Dejerine-Sottas disease revealed a new genetic insertion linked to atypical CMTX3, which was inherited from his mother.
  • - The research suggests that further analysis of genomic rearrangements in a specific chromosome region should be included in diagnostic tests for childhood-onset CMT, as these genetic changes may disrupt important genes and contribute to related neurological symptoms.
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Study Objectives: To assess whether the frequency content of electroencephalography (EEG) and electrooculography (EOG) during nocturnal polysomnography (PSG) can predict all-cause mortality.

Methods: Power spectra from PSGs of 8,716 participants, included from the MrOS Sleep Study and the Sleep Heart Health Study (SHHS), were analyzed in deep learning-based survival models. The best-performing model was further examined using SHapley Additive Explanation (SHAP) for data-driven sleep-stage specific definitions of power bands, which were evaluated in predicting mortality using Cox Proportional Hazards models.

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EEG and semiology in the elderly: A systematic review.

Seizure

September 2024

Department of Clinical Neurophysiology, Danish Epilepsy Centre*, Visbys Allé 5, 4293 Dianalund, Denmark; Department of Clinical Neurophysiology, Aarhus University Hospital*, and Department of Clinical Medicine, Aarhus University, Palle Juul-Jensens Boulevard 165, 8200 Aarhus N, Denmark. Electronic address:

Article Synopsis
  • Epileptic seizures are increasingly common in the elderly but often go unreported and undiagnosed, leading to delays in treatment; this review aims to raise awareness and improve diagnosis for this population.
  • The study systematically reviews 102 original research studies focusing on EEG findings and seizure characteristics in older adults, categorizing results into four distinct groups.
  • Key findings indicate that about half of elderly individuals show EEG abnormalities, with focal onset seizures being the most prevalent, and highlight the complex relationship between epilepsy and dementia in older age.
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Small fiber neuropathy is a common complication in patients with sarcoidosis and its prevalence is estimated at 40-86%. The underlying mechanism influences the presentation of small fiber neuropathy. For example, patients with metabolic diseases are often associated with a classic length-dependent small fiber neuropathy pattern, while patients with inflammatory diseases are more often present with a non-length-dependent small fiber neuropathy.

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Introduction: Painful idiopathic distal sensory polyneuropathy (IDSP) and fibromyalgia syndrome (FMS) are cryptogenic chronic pain syndromes. The contribution of small fibre pathology (SFP) in FMS remains controversial. This study aims to quantify small nerve pathology in participants with IDSP and FMS and identify relationships of SFP with sensory phenotypes.

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Aim: Opioids might be harmful to the developing brain and dosing accuracy is important. We aimed at investigating fentanyl effects on cortical activity in infants using computational re-analysis of bedside recorded EEG signals.

Methods: Fifteen infants born at median 26.

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Article Synopsis
  • The study aimed to evaluate children with epilepsy and motor speech regression by examining their speech disorders, seizure characteristics, and overall outcomes.
  • Eighteen children were assessed, revealing speech regression typically starting around age 5, often coinciding with seizures; common speech disorders included dysarthria and phonological errors, along with specific electrographic abnormalities in the brain.
  • While the speech intelligibility initially affected many children, those with Landau-Kleffner syndrome showed significant improvement over time, contrasting with a decline in expressive language abilities for children with Rasmussen syndrome; overall, motor speech disorders were complex and severity fluctuated with seizure activity.
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Article Synopsis
  • The study explores myoclonus dystonia caused by a variant in the SGCE gene, focusing on the microarchitectural brain abnormalities linked to this rare condition.
  • Researchers compared the brain structures of 18 MYC/DYT-SGCE patients with 24 healthy volunteers using advanced imaging techniques to assess neurite organization.
  • Results indicate that patients exhibited changes in cerebellar structure, with specific alterations correlating to the severity of dystonia, while no links were found between myoclonus severity and the microarchitectural measurements.
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Background: Cognitive and mental health problems are highly prevalent in adolescence. While higher levels of physical fitness may mitigate these problems, there is a lack of long-term follow-up studies on the associations of physical fitness from childhood with cognition and mental health in adolescence.

Objective: We investigated the associations of physical fitness from childhood to adolescence over an 8-year follow-up with cognition and mental health in adolescence.

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Patient-reported daily functioning after SARS-CoV-2 vaccinations in autoimmune neuromuscular diseases.

Eur J Neurol

December 2024

Department of Neurology and Neurophysiology, Amsterdam Neuroscience, Amsterdam UMC, location AMC, University of Amsterdam, Amsterdam, the Netherlands.

Background And Purpose: There are concerns for safety regarding SARS-CoV-2 vaccines for patients with autoimmune neuromuscular disease. We compared daily functioning using disease-specific patient-reported outcome measures (PROMs) before and after SARS-CoV-2 vaccinations.

Methods: In this substudy of a prospective observational cohort study (Target-to-B!), patients with myasthenia gravis (MG), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and idiopathic inflammatory myopathy (IIM) vaccinated against SARS-CoV-2 were included.

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Identifying early and non-invasive biomarkers to detect individuals in the earliest stages of the Alzheimer's disease continuum is crucial. As a result, electrophysiology and plasma biomarkers are emerging as great candidates in this pursuit due to their low invasiveness. This is the first magnetoencephalography study to assess the relationship between minimum spanning tree parameters, an alternative to overcome the comparability and thresholding problem issues characteristic of conventional brain network analyses, and plasma phosphorylated tau231 levels in unimpaired individuals, with different risk levels of Alzheimer's disease.

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The role of SEEG in the presurgical decision-making process in MRI-normal mesial temporal lobe epilepsy.

Rev Neurol (Paris)

December 2024

Department of Functional Neurology and Epileptology, Hospices Civils de Lyon and Lyon 1 University, Lyon, France; Lyon Neuroscience Research Center, Inserm U1028, CNRS UMR5292, Lyon 1 University, Lyon, France.

Article Synopsis
  • In patients with mesial temporal lobe epilepsy (mTLE) who have normal MRI results, there is debate about whether to perform anterior temporal lobectomy while sparing the hippocampus due to potential memory loss risks.
  • A study examined 17 patients with normal MRIs and EEG data that indicated unilateral seizure activity, categorizing them into two groups based on the timing of hippocampal involvement during seizures.
  • The results showed no significant difference in post-operative seizure and neuropsychological outcomes regardless of whether the hippocampus was removed during surgery, suggesting that SEEG should be part of the surgical planning for these patients.
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Facial nerve (FN) injury is a significant risk during complex cranial reconstruction surgeries, especially in revision cases where normal anatomy is distorted. The authors introduce a technique to mitigate FN injury, including preoperative FN mapping, intraoperative FN mapping, and continuous FN monitoring. Preoperative mapping uses a handheld ball-tip stimulator to elicit compound muscle action potentials (CMAP) in the frontalis muscle.

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Article Synopsis
  • Neonatal seizures are difficult to diagnose mainly because they are often detected only through electrographic means, with multichannel video continuous electroencephalography (cEEG) being the best method; however, access to this method can be limited outside regular hours.
  • This study evaluated the effectiveness of the Persyst neonatal seizure detection algorithm (SDA) by analyzing cEEG recordings from neonates in intensive care at Great Ormond Street Hospital, comparing the SDA results to expert neurophysiology reports.
  • Results showed that the Persyst neonatal SDA has high diagnostic accuracy with an area under the ROC curve of 0.94, indicating that it can reliably identify neonatal seizures using specific probability thresholds, achieving 80% sensitivity and 98
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Article Synopsis
  • Pathogenic variants in the CLDN19 gene lead to Familial Hypomagnesemia with Hypercalciuria and Nephrocalcinosis (FHHNC), which also includes eye-related issues.
  • A 25-year-old woman with FHHNC showed significant renal involvement and various eye problems, including high myopia and atrophic retinal plaques, alongside genetic testing that revealed a specific mutation in the CLDN19 gene.
  • The study suggests that FHHNC is linked to diverse ocular changes, and the described retinal issues in this patient may indicate a more favorable visual outlook than previously documented, highlighting the need for more research in this area.
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Background: Carpal tunnel syndrome (CTS) is a very common clinical syndrome manifested by signs and symptoms of irritation of the median nerve at the carpal tunnel in the wrist. Direct and indirect costs of CTS are substantial, with estimated costs of two billion US dollars for CTS surgery in the USA alone. Local corticosteroid injection has been used as a non-surgical treatment for CTS for many years, but its effectiveness is still debated.

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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.

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Stiff Person Syndrome With Positive Anti-glutamic Acid Decarboxylase (GAD) Autoantibodies.

Cureus

August 2024

Research Team in Neurology, Department of Neurology A and Neuropsychology, Faculty of Medicine and Pharmacy, Specialty Hospital, University Mohammed V, Rabat, MAR.

Stiff person syndrome (SPS) is a progressive autoimmune disorder characterized by muscle rigidity, frequent falls, and spasms, affecting primarily women. Recent advances have linked SPS to specific antibodies, such as anti-glutamic acid decarboxylase (GAD)-65, but effective treatments remain elusive. We report the case of a 53-year-old female who developed chronic lower back pain, tingling paresthesias, and progressive rigidity in the lower limbs.

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Epilepsy is one of the most common neurological disorders in children. Diagnosing epilepsy in children can be very challenging, especially as it often coexists with neurodevelopmental conditions like autism and ADHD. Functional brain networks obtained from neuroimaging and electrophysiological data in wakefulness and sleep have been shown to contain signatures of neurological disorders, and can potentially support the diagnosis and management of co-occurring neurodevelopmental conditions.

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A teenage girl with drug-resistant epilepsy and a hippocampal angiocentric neuroepithelial tumor (ANET) - illustrative case of 7T MRI in clinical practice.

Seizure

October 2024

Academic Center for Epileptology, Maastricht University Medical Center and Kempenhaeghe, Maastricht-Heeze, the Netherlands; Department of Neurosurgery, Maastricht University Medical Center, Maastricht, the Netherlands; Mental Health and Neuroscience Research Institute (MHeNS), Maastricht University, Maastricht, the Netherlands.

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The proportion of patients becoming seizure-free after epilepsy surgery has stagnated. Large multi-center stereo-electroencephalography (SEEG) datasets can allow comparing new patients to past similar cases and making clinical decisions with the knowledge of how cases were treated in the past. However, the complexity of these evaluations makes the manual search for similar patients impractical.

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NORSE/FIRES: how can we advance our understanding of this devastating condition?

Front Neurol

August 2024

Institute of Health and Neurodevelopment, School of Health and Life Sciences, Aston University, Birmingham, United Kingdom.

Introduction: New onset refractory status epilepticus (NORSE) is a rare and devastating condition characterised by the sudden onset of refractory status epilepticus (RSE) without an identifiable acute or active structural, toxic, or metabolic cause in an individual without a pre-existing diagnosis of epilepsy. Febrile infection-related epilepsy syndrome (FIRES) is considered a subcategory of NORSE and presents following a febrile illness prior to seizure onset. NORSE/FIRES is associated with high morbidity and mortality in children and adults.

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Long term functioning with poor neurologic outcome after cardiac arrest.

Resuscitation

October 2024

Department of Clinical Neurophysiology, Technical Medical Center, University of Twente, Enschede, the Netherlands; Department of Neurology, Rijnstate Hospital, Arnhem, the Netherlands.

Aim: Around six percent of comatose patients after cardiac arrest have a Cerebral Performance Categories score of three (CPC3) at six months after the arrest, classified as severe neurological disability. There is limited knowledge regarding the likelihood of further recovery in the cognitive, emotional, and quality of life domains. We aimed to estimate the probability of recovery towards independency.

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Potential therapeutic effect of Lamotrigine in disorders of consciousness after severe traumatic brain injury: A series of 4 cases.

Ann Phys Rehabil Med

November 2024

Université Claude Bernard Lyon 1, CNRS, INSERM, Centre de Recherche en Neurosciences de Lyon CRNL, INSERM UMRS 1028, CNRS UMR 5292, Trajectoires, 69000, Lyon, France; Service de rééducation post-réanimation, pôle de rééducation, Hospices Civils de Lyon, Lyon, 69000, France. Electronic address:

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