Nerve Disorders Research Articles

The clinical profile of new-onset optic neuritis in arabs, a tertiary center experience in Kuwait

BackgroundOptic neuritis is an inflammatory disorder of the optic nerve and is often the initial manifestation of systemic demyelinating diseases such as multiple sclerosis (MS), neuromyelitis optic spectrum disorder (NMOSD), and myelin-oligodendrocyte glycoprotein (MOG) antibody-mediated disease. There are ethnic variations in the etiology of optic neuritis across the world. While multiple sclerosis is common in the West, NMOSD and MOG are more common causes in Asian patients. There is a paucity of reports on the clinical profile of optic neuritis in the Middle East. ObjectivesTo study the demographic and clinical features of patients with new onset optic neuritis in a main tertiary care center. MethodsA retrospective study of cases with new-onset optic neuritis at a tertiary care center between 2012 and 2022. The clinical and demographic characteristics were obtained from medical records and were summarized using descriptive statistics. Univariate analysis and multivariate analysis to assess the short-term visual outcome. ResultsSeventy-one patients with new-onset optic neuritis (70 unilateral and one bilateral) were included in the study. The mean age was 33.3 years, they were predominantly females (73 %), and most of the cases were MS (53 %) followed by idiopathic optic neuritis (42.3 %). Final visual acuity of at least 20/40 was seen in at least 91.5 %. ConclusionWhile the clinical profile of patients in this study closely resembles the Optic Neuritis Treatment Trial with a high incidence of MS and a good visual outcome in most patients and a good response to intravenous steroids, there is a significant proportion of idiopathic optic neuritis cases that may need to be better characterized with longer follow up and repeated serum biomarker testing.

Pharmacological Doses of Thiamine Benefit Patients with the Charcot-Marie-Tooth Neuropathy by Changing Thiamine Diphosphate Levels and Affecting Regulation of Thiamine-Dependent Enzymes.

Charcot-Marie-Tooth (CMT) neuropathy is a polygenic disorder of peripheral nerves with no effective cure. Thiamine (vitaminB1) is a neurotropic compound that improves neuropathies. Our pilot study characterizes therapeutic potential of daily oral administration of thiamine (100mg) in CMT neuropathy and its molecular mechanisms. The patient hand grip strength was determined before and after thiamine administration along with the blood levels of the thiamine coenzyme form (thiamine diphosphate, ThDP), activities of endogenous holo-transketolase (without ThDP in the assay medium) and total transketolase (with ThDP in the assay medium), and transketolase activation by ThDP [1-(holo-transketolase/total transketolase),%], corresponding to the fraction of ThDP-free apo-transketolase. Single cases of administration of sulbutiamine (200mg) or benfotiamine (150mg) reveal their effects on the assayed parameters within those of thiamine. Administration of thiamine or its pharmacological forms increased the hand grip strength in the CMT patients. Comparison of the thiamin status in patients with different forms of CMT disease to that of control subjects without diagnosed pathologies revealed no significant differences in the average levels of ThDP, holo-transketolase, or relative content of holo and apo forms of transketolase. However, the regulation of transketolase by thiamine/ThDP differed in the control and CMT groups: in the assay, ThDP activated transketolase from the control individuals, but not from CMT patients. Thiamine administration paradoxically decreased endogenous holo-transketolase in CMT patients; this effect was not observed in the control group. Correlation analysis revealed sex-specific differences in the relationship between the parameters of thiamine status in both the control subjects and patients with the CMT disease. Thus, our findings link physiological benefits of thiamine administration in CMT patients to changes in their thiamine status, in particular, the blood levels of ThDP and transketolase regulation.

Diagnosis and treatment of optic neuritis as a manifestation of persistent herpesvirus infection: a clinical case

A clinical case of bilateral descending optic neuritis in a 63-year-old patient is presented. The patient, whose age is beyond the normal limit for this pathology, was previously treated for neuritis of the right eye, which had no effect and led to a complete atrophy of the optic nerve (ON). A timely correct diagnostics of the left eye, which revealed structural disorders of the optic nerve accompanied by inflammatory viral changes of brain tissue, was a key factor in choosing an adequate treatment, which helped retain visual functions. The clinical observation illustrates the polyetiological nature of inflammation of the ON, the viral agent playing a leading role. Importantly, ophthalmic manifestations of the herpesvirus infection may remain the only sign of this pathology for a long time, so the ophthalmologist must always be on the alert regarding the viral genesis of the disease.

Application of Ophthalmic Electrophysiology in Inflammatory Disorders of Retina and Optic Nerve.

This review covers the utility of electrophysiological studies relevant to inflammatory diseases of the retina in conditions such as acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, Adamantiades-Behçet disease, autoimmune retinopathy and neuro-retinopathy, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and Vogt-Koyanagi-Harada disease. Electrophysiological studies can help with the diagnosis, prognostication, evaluation of treatment effects, and follow-up for these conditions.

Vagus nerve modulates acute-on-chronic liver failure progression via CXCL9

Abstract Background: Hepatic inflammatory cell accumulation and the subsequent systematic inflammation drive acute-on-chronic liver failure (ACLF) development. Previous studies showed that the vagus nerve exerts anti-inflammatory activity in many inflammatory diseases. Here, we aimed to identify the key molecule mediating the inflammatory process in ACLF and reveal the neuroimmune communication arising from the vagus nerve and immunological disorders of ACLF. Methods: Proteomic analysis was performed and validated in ACLF model mice or patients, and intervention animal experiments were conducted using neutralizing antibodies. PNU-282987 (acetylcholine receptor agonist) and vagotomy were applied for perturbing vagus nerve activity. Single-cell RNA sequencing (scRNA-seq), flow cytometry, immunohistochemical and immunofluorescence staining, and CRISPR/Cas9 technology were used for in vivo or in vitro mechanistic studies. Results: The unbiased proteomics identified C-X-C motif chemokine ligand 9 (CXCL9) as the greatest differential protein in the livers of mice with ACLF and its relation to the systematic inflammation and mortality were confirmed in patients with ACLF. Interventions on CXCL9 and its receptor C-X-C chemokine receptor 3 (CXCR3) improved liver injury and decreased mortality of ACLF mice, which were related to the suppressing of hepatic immune cells’ accumulation and activation. Vagus nerve stimulation attenuated while vagotomy aggravated the expression of CXCL9 and the severity of ACLF. Blocking CXCL9 and CXCR3 ameliorated liver inflammation and increased ACLF-associated mortality in ACLF mice with vagotomy. scRNA-seq revealed that hepatic macrophages served as the major source of CXCL9 in ACLF and were validated by immunofluorescence staining and flow cytometry analysis. Notably, the expression of CXCL9 in macrophages was modulated by vagus nerve-mediated cholinergic signaling. Conclusions: Our novel findings highlighted that the neuroimmune communication of the vagus nerve–macrophage–CXCL9 axis contributed to ACLF development. These results provided evidence for neuromodulation as a promising approach for preventing and treating ACLF.

Identifikasi Bakteri Escherichia Coli Pada Sampel Daging Bakso Sebagai Pangan Jajanan Anak Sekolah (PJAS)

Pangan Jajanan Anak Sekolah (PJAS) is one of the foods consumed by people in Indonesia, especially by children and adolescents. This food is very vulnerable to contamination by microorganisms that can damage and can be toxic to the human body when consumed. Microbes can contaminate food through water, dust, air, soil, processing equipment (during the production or preparation process) as well as secretions from the intestines of humans and animals. The method used is literature review by collecting, evaluating, and synthesizing existing studies in the scientific literature. In this case using a search engine such as google scholar. The years obtained from articles from 2018 to 2024 are relevant to the topic of this research. The topic used is the identification of Escherichia coli bacteria in meatball meat samples as school snack food (PJAS), this method is used to gain an in-depth understanding of Escherichia coli bacteria in meatball meat samples as school snacks. According to the World Health Organization (WHO), an estimated 600 million people globally (nearly 1 in 10 people) fall ill including diarrhea, kidney and liver failure, brain and nerve disorders, reactive arthritis and cancer from food-borne diseases, with 420,000 deaths reported in 2010. Food-borne illnesses arise from the consumption of contaminated food, which can be.

Myelinated peripheral axons are more vulnerable to mechanical trauma in a model of enlarged axonal diameters.

The velocity of axonal impulse propagation is facilitated by myelination and axonal diameters. Both parameters are frequently impaired in peripheral nerve disorders, but it is not known if the diameters of myelinated axons affect the liability to injury or the efficiency of functional recovery. Mice lacking the adaxonal myelin protein chemokine-like factor-like MARVEL-transmembrane domain-containing family member-6 (CMTM6) specifically from Schwann cells (SCs) display appropriate myelination but increased diameters of peripheral axons. Here we subjected Cmtm6-cKo mice as a model of enlarged axonal diameters to a mild sciatic nerve compression injury that causes temporarily reduced axonal diameters but otherwise comparatively moderate pathology of the axon/myelin-unit. Notably, both of these pathological features were worsened in Cmtm6-cKo compared to genotype-control mice early post-injury. The increase of axonal diameters caused by CMTM6-deficiency thus does not override their injury-dependent decrease. Accordingly, we did not detect signs of improved regeneration or functional recovery after nerve compression in Cmtm6-cKo mice; depleting CMTM6 in SCs is thus not a promising strategy toward enhanced recovery after nerve injury. Conversely, the exacerbated axonal damage in Cmtm6-cKo nerves early post-injury coincided with both enhanced immune response including foamy macrophages and SCs and transiently reduced grip strength. Our observations support the concept that larger peripheral axons are particularly susceptible toward mechanical trauma.

Radiofrequency thermocoagulation as a treatment for hemifacial spasm: long-term follow-up and management of recurrences

BackgroundRadiofrequency thermocoagulation (RFT) is a treatment used to relieve symptoms of cranial nerve disorders. The current study is the first to describe the results of hemifacial spasm (HFS) patients with a history of repeated RFT in the second-largest consecutive single-center patient series with long-term follow-up.MethodThis retrospective study was conducted in the largest hospital district in Finland (Helsinki and Uusimaa). Consecutive HFS patients who had an RFT to treat HFS in the Hospital District of Helsinki and Uusimaa between 2009-2020 were included.ResultsEighteen patients with 53 RFTs were identified from the medical records. 11 (61 %) patients had repeated RFTs, and the mean number of RFTs per patient was 3.33 (3.29 SD). The mean follow-up was 5.54 years (7.5 SD). 12 (67 %) patients had had microvascular decompression (MVD) before RFT.Patients were satisfied with the results after 87 % of RFTs. Relief of the twitching of the face lasted 11.27 months (11.94 SD). All patients had postoperatively transient facial paresis. Postoperative paresis lasted a mean of 6.47 months (6.80 SD). The depth of paresis was postoperatively typically moderate (36.54 %, House Brackmann III). 23.08 % had mild paresis (House-Brackmann II), 23.08 % had moderately severe dysfunction (House-Brackmann IV), 9.62 % had severe dysfunction, and 7.69 % had total paralysis of the facial muscles (House-Brackmann VI). Duration of relief in the face twitching (p 0.002) and temperature at the final coagulation point (p 0.004) were statistically significant predictors of satisfaction with the RFT results.ConclusionsRFT can be used to treat recurrences of HFS repeatedly. It provides symptom relief for around 11 months, lasting four times longer than with botulinum toxin injections. Patients are satisfied, although an RFT produces transient, sometimes even severe, facial paresis.

Ultrasound guided platelet rich plasma injections for post-traumatic greater occipital neuralgia following concussion: a pilot randomized controlled trial.

Treatment for post-traumatic greater occipital neuralgia (GON) includes serial injections of steroid/anesthetic. While these injections can alleviate pain, effects can be transient, frequently lasting only 1 month. As a potential alternative, platelet-rich plasma (PRP) injections are an emerging biological treatment with beneficial effects in peripheral nerve disorders. We investigated the feasibility, safety, and effectiveness of a single PRP injection for post-traumatic GON in comparison to saline or steroid/anesthetic injection. In this pilot randomized, double-blinded, placebo-controlled trial, 32 adults with post-traumatic GON were allocated 1:1:1 to receive a single ultrasound-guided injection of (1) autologous PRP (2) steroid/anesthetic or (3) normal saline. Our primary outcome was feasibility (recruitment, attendance, retention) and safety (adverse events). Exploratory measures included headache intensity and frequency (daily headache diaries) and additional questionnaires (headache impact, and quality of life) assessed at pre-injection, 1 week, 1 month, and 3 months post-injection. We screened 67 individuals, 55% were eligible and 95% of those participated. Over 80% of daily headache diaries were completed with 91% of participants completing the 3-month outcome questionnaires. No serious adverse events were reported. There were no significant differences between groups for headache intensity or frequency. Headache impact on function test-6 scores improved at 3 month in the PRP (β = -9.7, 95% CI [-15.6, -3.74], p = 0.002) and saline (β = -6.7 [-12.7, -0.57], p = 0.033) groups but not steroid/anesthetic group (p = 0.135). PRP is a feasible and safe method for treating post-traumatic GON with comparable results to saline and steroid/anaesthetic. Further trials with larger sample sizes are required.Clinical trial registration:https://clinicaltrials.gov/, identifier NCT04051203.

Towards seamless conformable and comfortable wearable electronics: Polyimide-supported dry electrodes integrated in knitted fabrics

Wearable electronics contribute directly to advancing numerous biomedical devices in contract with the human body. These electronic devices have been transformed from rigid and bulky into soft and flexible. Conformable electronics is actively pursued in the literature to comply with the curvilinear surface of the skin. To increase the adoption rate, seamless comfortable operation is necessary, which requires more focus on convenient materials such as textiles, compared to elastomer-based technologies. This study demonstrates mechanically reliable stretchable interconnects embedded within textile to achieve both conformability and comfort. Electromyography (EMG) signals provide invaluable information about body motion analysis, muscle nerve and fatigue disorders. Non-invasive surface EMG is the preferred method of measurement since it can be conducted with minimal risk to the subject. In this study, polyimide-supported dry electrodes are integrated in knitted fabrics using a special knit pattern to ensure achieving higher contact surface area with the human skin. The received EMG signals are benchmarked against standard wet electrodes in time and frequency domain, revealing comparable performance. The presented technology exhibits extremely high mechanical reliability, withstanding up to 10,000 cyclic elongations at 60 % strain. The integration strategy is sufficiently strong and durable, retaining its electrical properties after 20 standard washing cycles. The findings indicate that the presented technology holds significant promise towards real-world implementation of seamless, conformable, and comfortable wearable electronics.

Pembrolizumab (pembro) for locally advanced (LA) or recurrent/metastatic (R/M) cutaneous squamous cell carcinoma (cSCC): Long-term results of the phase 2 KEYNOTE-629 study.

9554 Background: Pembro monotherapy is approved in certain countries, including the US, for treatment of LA or R/M cSCC based on results from the open-label phase 2 KEYNOTE-629 trial (NCT03284424). Promising antitumor activity was demonstrated with pembro in both the LA and R/M cohorts. ORR (95% CI) was 50.0% (36.1-63.9; 16.7% CRs) in the LA cohort and 35.2% (26.2-45.2; 10.5% CRs) in the R/M cohort. We present data from KEYNOTE-629 with an additional follow-up of 38 mo for LA and R/M cohorts. Methods: Adults with histologically confirmed LA or R/M cSCC, measurable disease per RECIST v1.1 by blinded independent central review (BICR), and ECOG PS 0 or 1 received pembro 200 mg IV every 3 weeks for up to 35 cycles (~2 years). The primary end point was ORR per RECIST v1.1 by BICR. Secondary end points were DOR, DCR (CR + PR + SD ≥12 wks), and PFS per RECIST v1.1 by BICR; OS; and safety. End points were analyzed in pts who received ≥1 dose of pembro. Results: A total of 159 pts were treated with pembro (LA, n = 54; R/M, n = 105). As of September 13, 2023, 33 pts (20.8%) completed treatment and 126 pts (79.2%) discontinued treatment. Median (range) follow-up was 52.4 mo (47.6-56.9) for the LA cohort, 64.7 mo (62.1-69.5) for the R/M cohort, and 63.1 mo (47.6-69.5) in the total population. ORR and DCR are shown in the table. Median DOR (range) was 47.2 mo (1.0+ to 49.9+) in the LA cohort, not reached (NR; 2.7 to 64.2+ mo) in the R/M cohort, and 52.5 mo (1.0+ to 64.2+) in the total population; the proportion of responders with responses ≥12 mo by Kaplan-Meier estimate were 84.8%, 77.8%, and 80.7%, respectively. Median (95% CI) PFS was 14.4 mo (5.5-43.6) in the LA cohort, 5.7 mo (3.1-8.5) in the R/M cohort, and 8.0 mo (5.3-14.4) in the total population; 12-mo rates were 56.7%, 37.3%, and 43.7%, respectively. Median (95% CI) OS was NR (33.3-NR) in the LA cohort, 23.8 mo (13.4-30.9) in the R/M cohort, and 29.8 mo (20.0-42.8) in the total population; 36-mo rates were 62.0%, 39.5%, and 47.0%, respectively. Grade 3-5 treatment-related AEs occurred in 11.3% of pts, and grade 3-5 immune-mediated AEs and infusion reactions occurred in 8.8% of pts. Two pts (1.3%) died due to a treatment-related AE (colitis, cranial nerve disorder). Conclusions: With a median follow-up of more than 5 years, pembro continued to show durable responses in pts with LA or R/M cSCC. No new safety signals were observed. Results from this study continue to support the use of pembro in this pt population. Clinical trial information: NCT03284424 . [Table: see text]

A phase II trial combining SHR-1316, bevacizumab, and cisplatin/carboplatin in patients with TNBC with active brain metastases.

e13133 Background: Triple-negative breast cancer (TNBC) has a high risk of brain metastases (BM), leading to poor survival. Treatment options for BMs are limited, and the combination of chemotherapy and immune checkpoint inhibitor is the standard-of-care for PD-L1 positive metastatic TNBC. Platinum-based systematic therapy is the mainstay of TNBC treatment and the addition of bevacizumab has shown improvement in the progression-free survival (PFS) of patients with BM. Therefore, this study aims to investigate the efficacy and safety of SHR-1316 (anti-PD-L1) in combination with bevacizumab and cisplatin/carboplatin in TNBC patients with BMs. Methods: In this single-center phase II trial, TNBC patients with active BM received SHR-1316, bevacizumab, and cisplatin/carboplatin. Prior treatment with bevacizumab or anti-PD-1/PD-L1 were not allowed. Prior platinum was allowed only in cases of previous platinum-sensitive response. The primary endpoint was objective response rate (ORR) of central nervous system (CNS), as assessed according to the response assessment in neuro-oncology brain metastases. The secondary endpoint included clinical benefit rate (CBR) of CNS, PFS, overall survival (OS), first progression site and safety. Results: As of January 12th, 2024, a total of 23 female patients with ECOG PS≥1(100%) were enrolled. The median number of previous lines of therapy for metastatic disease was 1 (0-3). All patients had active BM, of which 87% (20/23) were BM treatment-naïve and 13% (3/23) had BM progression after previous local therapy, 26.1% (6/23) had received a prior platinum agent. Of the 23 evaluable patients, the confirmed CNS ORR was 65.2% (95%CI: 42.7%-83.6%), with 3 complete response (CR), 12 partial response (PR), 7 stable disease (SD), and 1 progressive disease (PD). The median CNS-PFS was 11.2 months (95%CI, 5.8 to not reached), median PFS was 7.6 months (95%CI, 5.7-11.9), and median OS was 13.2 months (95%CI, 9.5 to not reached). Treatment-related adverse events (TRAEs) were reported in all 23 patients, and the most common TRAEs were neutrophil count decreased (69.6%, 16/23), anemia (65.2%, 15/23), asthenia (60.9%, 14/23), Hypomagnesaemia (56.5%, 13/23), leukopenia (52.2%, 12/23) and electrocardiogram T wave abnormal (52.2%, 12/23). The incidence of grade ≥3 TRAEs was 43.5% (10/23), including neutropenia (13%, 3/23), platelet count decreased (8.7%, 2/23), and hypertension (8.7%, 2/23). Only one patient had a grade 4 TRAE (lymphocytopenia), and SHR-1316 related serious adverse event (facial nerve disorder) occurred in only one patient. No grade 5 AEs were noted. Conclusions: SHR-1316 plus bevacizumab and cisplatin/carboplatin achieves a significant improvement in CNS ORR and brings PFS and OS benefits in TNBC patients with active BMs. As an effective and safe treatment, this triple-combination warrants further investigation. Clinical trial information: NCT04303988 .

Clinical Case Report on Electroacupuncture Combined with Warm Acupuncture for the Treatment of Painful Ophthalmoplegia (Tolosa-hunt Syndrome)

Tolosa-Hunt Syndrome (THS) is a rare and complex clinical syndrome characterized by severe headache, orbital pain, and multiple cranial nerve palsies. The precise etiology of THS remains unclear, though it is hypothesized to be related to immune system deficiencies, viral infections, or nonspecific inflammation. Due to its low incidence, THS is infrequently encountered in clinical practice. This case report presents a 39-year-old female patient with THS who was successfully treated with a combination of electroacupuncture and warm acupuncture. The patient's symptoms included left eyelid ptosis, left-sided headache, and reduced eye movement, which initially responded to high-dose steroid therapy but recurred upon cessation. After discontinuing steroid treatment, the patient received comprehensive acupuncture therapy aimed at soothing the liver and gallbladder, tonifying Qi, activating blood, and unblocking channels to relieve pain. Over the course of more than 30 acupuncture sessions, the patient's symptoms significantly improved, with complete resolution of pain and restoration of eye function. This case highlights the potential of acupuncture as an effective alternative treatment for THS, providing valuable insights for the management of this rare condition. Future research should further explore the mechanisms and efficacy of acupuncture in treating THS and other cranial nerve disorders.

Amish Infantile Epilepsy in an Indian Child

AbstractConsanguineous marriages in India continue to give rise to a wide spectrum of recessively inherited disorders that require a broader base of knowledge. A 2-year-old boy presented with global development delay, persistent vomiting, drug refractory seizures, deafness, and central hypotonia. He had profound bilateral hearing loss, barium swallow showed severe reflux but magnetic resonance imaging brain was normal, leading to a diagnostic dilemma. A normal electromyogram with nerve conduction velocity ruled out disorders of muscle and nerve. Whole-exome sequencing showed salt and pepper development regression syndrome but phenotypically, he did not have the classic skin changes. He has shown mild improvement in cognition, mobility, and weight gain with citicoline, antireflux medications, antiseizure medications, and a protein diet. Accurate diagnosis based on cohort of symptoms and appropriate early intervention can help improve the quality of life in such children.

Effect of Timing of Rehabilitation Nursing Intervention on Children with Acute Viral Encephalitis

Background: Viral encephalitis (VE) refers to an acute intracranial inflammation mainly caused by various viral infections. Objectives: Viral encephalitis (VE) refers to an acute inflammation of the brain primarily caused by various viral infections. Methods: Data from 60 children with acute viral encephalitis (VE) who received 4 weeks of rehabilitation nursing from July 2019 to July 2020 were collected for retrospective analysis. These children were divided into two groups: An early intervention group (n = 30), where rehabilitation nursing started in the second week after admission, and a late intervention group (n = 30), where rehabilitation nursing began in the fourth week after admission. Motor function, daily living abilities, and limb spasticity were compared before and four weeks after nursing. Additionally, the outcomes of rehabilitation nursing—including recovery times from convulsions, cranial nerve disorders, and consciousness disorders—were also compared. Results: The scores for motor function and daily living ability were significantly higher, while the score for limb spasticity was lower in the early intervention group compared to the late intervention group (P < 0.05). Additionally, the early intervention group experienced a significantly shorter recovery time from convulsions, cranial nerve disorders, and consciousness disorders than the late intervention group (P < 0.05). Conclusions: Early rehabilitation nursing is effective in enhancing motor function, relieving limb spasticity, and improving the nursing outcomes for children with acute viral encephalitis (VE).

The combination of electrotherapy with pelvic floor muscle training in the treatment of a patient with postcovid erectile dysfunction. Clinical case

The combination of electrotherapy with pelvic floor muscle training in the treatment of a patient with postcovid erectile dysfunction. Clinical case

Immune dysregulation in chronic inflammatory demyelinating polyneuropathy

ObjectiveChronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with an incompletely understood underlying pathophysiology. This investigation focused on defining B and T cell frequencies, T cell functional capacity and innate immune system analysis in patients with CIDP. MethodsBy using multi-parameter flow cytometry, we examined the phenotype and function of PBMCs in 25 CIDP patients who were relatively clinically stable on treatment who met EFNS/PNS criteria, 21 patients with genetically confirmed hereditary neuropathy and 25 healthy controls. We also evaluated the regulatory T cell (Treg) inhibitory capacity by co-culturing Treg and effector T cells. ResultsProinflammatory CD4 T cells, especially type 1 helper T cell (Th1) and CD8 T cells in patients with CIDP were found to have an enhanced capacity to produce inflammatory cytokines. There was no difference in frequency of Th17 regulatory cells in CIDP patients versus healthy controls, however, Treg function was impaired in CIDP patients. There was no remarkable difference in innate immune system measures. Within B cell subsets, transitional cell frequency was decreased in CIDP patients. InterpretationPatients with CIDP clinically stable on treatment continued to show evidence of a proinflammatory state with impaired Treg function. This potentially implies an inadequate suppression of ongoing inflammation not addressed by standard of care therapies as well as persistent activity of disease while on treatment. Targeting T cells, especially inhibiting Th1 and polyfunctional CD8 T cells or improving Treg cell function could be potential targets for future therapeutic research.

Peripheral Nerve and Muscle Disorders Associated with Sjögren's Syndrome

Sjögren's syndrome is often accompanied by various neurological complications, among which peripheral neuropathy is the most common. A variety of clinical phenotypes of peripheral neuropathy, including axonal polyneuropathy and sensory ataxic neuropathy are reported in the literature. We present an overview of the pathophysiology and differential diagnosis of each phenotype. Immunotherapy using corticosteroids and high-dose intravenous immunoglobulin therapy tends to elicit varied therapeutic responses depending on the peripheral neuropathy phenotype. We also discuss myositis, a possible complication of Sjögren's syndrome.

Flexible Peripheral Nerve Interfacing Electrode for Joint Position Control in Closed-Loop Neuromuscular Stimulation.

Addressing peripheral nerve disorders with electronic medicine poses significant challenges, especially in replicating the dynamic mechanical properties of nerves and understanding their functionality. In the field of electronic medicine, it is crucial to design a system that thoroughly understands the functions of the nervous system and ensures a stable interface with nervous tissue, facilitating autonomous neural adaptation. Herein, we present a novel neural interface platform that modulates the peripheral nervous system using flexible nerve electrodes and advanced neuromodulation techniques. Specifically, we have developed a surface-based inverse recruitment model for effective joint position control via direct electrical nerve stimulation. Utilizing barycentric coordinates, this model constructs a three-dimensional framework that accurately interpolates inverse isometric recruitment values across various joint positions, thereby enhancing control stability during stimulation. Experimental results from rabbit ankle joint control trials demonstrate our model's effectiveness. In combination with a proportional-integral-derivative (PID) controller, it shows superior performance by achieving reduced settling time (less than 1.63 s), faster rising time (less than 0.39 s), and smaller steady-state error (less than 3 degrees) compared to the legacy model. Moreover, the model's compatibility with recent advances in flexible interfacing technologies and its integration into a closed-loop controlled functional neuromuscular stimulation (FNS) system highlight its potential for precise neuroprosthetic applications in joint position control. This approach marks a significant advancement in the management of neurological disorders with advanced neuroprosthetic solutions.

Median Nerve Anatomy and Entrapment Syndromes

Peripheral neuropathies, including entrapment or injury, are quite common and are encountered frequently. To ensure accurate diagnosis and effective treatment for these conditions, it is essential to have a comprehensive understanding of peripheral nerve anatomy and assessment techniques. This may involve the use of methods like ultrasonography, as well as requiring clinical proficiency. The median nerve, which is one of the main nerves in the upper extremity, primarily controls motor function in the flexor muscles of the forearm and hand. It is responsible for actions such as wrist flexion and the movement of the first three fingers. The purpose of this article is to enhance understanding and improve the diagnosis of peripheral nerve disorders, with a particular focus on neuropathies associated with the median nerve.