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Introduction/Aims: Somatosensory evoked potentials (SSEPs) are described as a supportive tool to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, there is a lack of studies determining the effectiveness of SSEPs in monitoring the clinical course of individuals with this condition. The aims of this study are to evaluate the utility of SSEPs in monitoring patients with CIDP and to assess their association with clinical outcomes following immunomodulatory therapy. Methods: This was a single‐center retrospective observational study that included patients who met European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP between 2018 and 2023. SSEPs were performed at diagnosis and during follow‐up after the start of immunomodulatory treatment. Fisher's exact test was employed to assess the association between clinical improvement and SSEP improvement. Results: Eighteen patients were included in the study. Ten patients had a typical CIDP pattern and 11 were male. In 17, SSEPs were abnormal prior to the start of immunomodulatory treatment. In patients who showed clinical improvement with immunomodulatory therapy, we observed that 15/17 had partial or complete improvement in SSEPs. Patients who showed no clinical improvement with first‐line treatment exhibited worsening SSEPs. There was a significant association between clinical and SSEPs improvement (p = 0.009). Discussion: We observed a positive association between improvement in SSEPs and clinical improvement in patients with CIDP. Our data suggest that SSEPs may be useful for monitoring the clinical course of patients with CIDP, but additional, larger studies are needed. [ABSTRACT FROM AUTHOR]

An abstract of the article "Echocardiographic cardiac remodelling in patients with atrial fibrillation and cardiac resynchronization therapy," by N. Castro Bueno and colleagues is presented.

Introduction/objective: Biallelic expansion of the pentanucleotide AAGGG in the RFC1- gene is associated with cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS). This study aimed to comprehensively characterise this condition by conducting an in-depth neurophysiological examination of afflicted patients., Methods: A retrospective analysis was conducted in 31 RFC1-positive patients. Assessment included clinical examination and electrodiagnostic tests: nerve conduction studies, F waves, needle electromyography, H, blink and masseter reflexes; Autonomic Nervous System (ANS): Heart Rate Variability, Sympathetic Skin Response and Electrochemical Skin Conductance, and Quantitative Sensory Test (QST). Medullar tracts were assessed using Somatosensory Evoked Potentials (SEPs) and Transcranial Magnetic Stimulation., Results: All patients exhibited moderate to severe sensory axonal neuropathy/neuronopathy, whereas motor nerve conduction parameters and the pyramidal pathway showed no abnormalities. Tibial nerve SEPs were absent or severely attenuated in all patients, and were moderately affected by the median nerve. H-reflexes were preserved in 85.7% and blink reflex in 60% of tested patients. ANS tests yielded predominantly normal results, although 74% demonstrated impaired QST., Conclusions: Our findings highlight the characteristic features of sensory neuropathy/neuronopathy and severe somatosensory deficits, with predominantly preserved H-reflexes and motor sparing. Thermoalgesic fibres are frequently involved, whereas the ANS is relatively preserved., Significance: Neurophysiological examinations can objectively characterize the spectrum of RFC1-related disease., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Syringomyelia can be associated with multiple etiologies. The treatment of the underlying causes is first-line therapy; however, a direct approach to the syrinx is accepted as rescue treatment. Any direct intervention on the syrinx requires a myelotomy, posing a significant risk of iatrogenic spinal cord (SC) injury. Intraoperative neurophysiological monitoring (IONM) is crucial to detect and prevent surgically induced damage in neural SC pathways. We retrospectively reviewed the perioperative and intraoperative neurophysiological data and perioperative neurological examinations in ten cases of syringomyelia surgery. All the monitored modalities remained stable throughout the surgery in six cases, correlating with no new postoperative neurological deficits. In two patients, significant transitory attenuation, or loss of motor evoked potentials (MEPs), were observed and recovered after a corrective surgical maneuver, with no new postoperative deficits. In two cases, a significant MEP decrement was noted, which lasted until the end of the surgery and was associated with postoperative weakness. A transitory train of neurotonic electromyography (EMG) discharges was reported in one case. The surgical plan was adjusted, and the patient showed no postoperative deficits. The dorsal nerve roots were stimulated and identified in the seven cases where the myelotomy was performed via the dorsal root entry zone. Dorsal column mapping guided the myelotomy entry zone in four of the cases. In conclusion, multimodal IONM is feasible and reliable and may help prevent iatrogenic SC injury during syringomyelia surgery.

Genetic screens are used to identify genes involved in specific biological processes. An EMS mutagenesis screen in Drosophila melanogaster identified growth control phenotypes in the developing eye. One mutant line from this screen, H.3.2 , was phenotypically characterized using the FLP/FRT system and genetically mapped by complementation analysis and genomic sequencing by undergraduate students participating in the multi-institution Fly-CURE consortium. H.3.2 was found to have a nonsense mutation in short stop ( shot ), anortholog of the mammalian spectraplakin dystonin ( DST ). shot and DST are involved in cytoskeletal organization and play roles during cell growth and proliferation., (Copyright: © 2021 by the authors.)

Lacosamide (LCM) is a treatment option for status epilepticus (SE) described in several series. We therefore proposed to describe its use in status epilepticus patients in our hospital. All patients admitted to our hospital for SE from September 2010 to April 2012 were evaluated. We collected related variables including the type of SE, etiology, antiepileptic drugs (AEDs) used, loading dose of AEDs, cessation of SE after AEDs, ICU admission and mortality. In those patients receiving LCM, we reviewed the infusion rate and time to response. We compared patients receiving LCM with patients in whom it was not used. This was a retrospective and uncontrolled study. A total of 92 patients were included; 67.7 % of SE patients who received LCM responded to treatment. The vast majority of the patients presented non-convulsive and motor focal SE. When we selected patients to receive four or more AEDs, the LCM efficacy was 55.6 %, a very similar result compared to when it was not used. Subsequently, we analyzed the sample regarding the AED administered as the second or third line of treatment, and the responder rate was significantly higher when LCM was used (84.6 vs. 47.8 %, p 0.041). After an adjusted regression analysis, the use of LCM was independently associated with cessation of SE. The total percentage of undesirable effects was very low (12 %), and they were all mild. No relationship was found between a specific etiology and better response. LCM is a useful drug that represents an alternative in the treatment of non-convulsive or focal motor SE. Its efficacy might be more important when it is administered as a second or third option after benzodiazepines. A randomized trial is required to confirm these results.

Introduction: Encephalopathy due to valproic acid (VPA) is a rare complication leading to a disorder that affects the patient's mental status to a greater or lesser extent and which can be accompanied by a paradoxical worsening of the seizures. The diagnosis is obvious when it appears within the context of hyperammonemia or a liver pathology, but can be difficult to diagnose if it appears in isolation in patients who show no other signs of intoxication due to VPA., Case Report: We report the case of an adolescent who suffered idiopathic generalised epilepsy and presented sub-acute cognitive impairment and a worsening of his pattern of seizures some months after starting treatment with VPA. These manifestations were accompanied by a slowing of the baseline electroencephalogram (EEG) tracing; no biochemical signs of overdosage or toxicity that could be attributed to the drug or any other possible aetiology were observed. Withdrawing VPA resulted in a swift improvement in the patient's mental status and in the control of his seizures. Likewise, EEG recordings also returned to normal., Conclusions: Encephalopathy due to VPA should be considered in patients who present a deterioration of their neurological status, whether associated to an aggravation of their seizures or not, despite the absence of any analytical signs suggestive of VPA toxicity or overdosage. If liver functioning is not affected, withdrawal of the drug will determine the disappearance of the symptoms and will also allow confirmation of the diagnosis.

Chronic inflammation is a major contributing factor in the pathogenesis of many age-associated diseases. One central protein that regulates inflammation is NF-κB, the activity of which is modulated by post-translational modifications as well as by association with co-activator and co-repressor proteins. SIRT1, an NAD(+)-dependent protein deacetylase, has been shown to suppress NF-κB signaling through deacetylation of the p65 subunit of NF-κB resulting in the reduction of the inflammatory responses mediated by this transcription factor. The role of SIRT1 in the regulation of NF-κB provides the necessary validation for the development of pharmacological strategies for activating SIRT1 as an approach for the development of a new class of anti-inflammatory therapeutics. We report herein the development of a quantitative assay to assess compound effects on acetylated p65 protein in the cell. We demonstrate that small molecule activators of SIRT1 (STACs) enhance deacetylation of cellular p65 protein, which results in the suppression of TNFα-induced NF-κB transcriptional activation and reduction of LPS-stimulated TNFα secretion in a SIRT1-dependent manner. In an acute mouse model of LPS-induced inflammation, the STAC SRTCX1003 decreased the production of the proinflammatory cytokines TNFα and IL-12. Our studies indicate that increasing SIRT1-mediated NF-κB deacetylation using small molecule activating compounds is a novel approach to the development of a new class of therapeutic anti-inflammatory agents.

Evoked Potentials (EP) are a neurophysiological tool that makes it possible for us to make an extensive study of the cerebral cortex, the brainstem and the spinal cord. Different techniques can be applied while performing the EPs, such as Visual Evoked Potentials (VEPs), Somatosensory Evoked Potentials (SSEPs), Long Latency Somatosensory Evoked Potentials (LLSEPs), Brainstem Auditory Evoked Potentials (BAEPs), Middle Latency Auditory Evoked Potentials (MLAEPs), Long Latency Auditory Evoked Potentials (LLAEP) and Mismatch Negativity (MMN). The combination of the different techniques of Evoked Potentials (EP) allows us to make a neurofunctional evaluation of comatose patients in the Intensive Care Units (ICU). It is also a useful tool in the diagnosis of the origin of coma, to confirm brain death, and as an evolutive prognosis value of the different central nervous system diseases (SNC). There are also studies that propose using EPs as a monitoring tool of the SNC. We present an up-dated review on the principal aspects of EP neuromonitoring in Intensive Care Unit (ICU) patients.

A series of triamide derivatives bearing a benzothiazole core is shown to be potent microsomal triglyceride transfer protein (MTP) inhibitors. In order to minimize liver toxicity, these compounds have been optimized to have activity only in the enterocytes and have limited systemic bioavailability. Upon oral administration, selected analogs within this series have been further demonstrated to reduce food intake along with body weight and thereby improve glucose homeostasis and insulin sensitivity in a 28-day mice diet-induced obesity (DIO) model.