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Revue Neurologique Oct 2023Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system targets peripheral nervous system antigens and that respond... (Review)
Review
Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system targets peripheral nervous system antigens and that respond to immune therapies. This review focuses on Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, polyneuropathy associated with IgM monoclonal gammopathy, and autoimmune nodopathies. Autoantibodies targeting gangliosides, proteins in the node of Ranvier, and myelin-associated glycoprotein have been described in these disorders, defining subgroups of patients with similar clinical features and response to therapy. This topical review describes the role of these autoantibodies in the pathogenesis of autoimmune neuropathies and their clinical and therapeutic importance.
Topics: Humans; Autoantibodies; Guillain-Barre Syndrome; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyneuropathies
PubMed: 36907709
DOI: 10.1016/j.neurol.2023.02.064 -
Handbook of Clinical Neurology 2024Electrodiagnostic testing (EDX) has been the diagnostic tool of choice in peripheral nerve disease for many years, but in recent years, peripheral nerve imaging has been... (Review)
Review
Electrodiagnostic testing (EDX) has been the diagnostic tool of choice in peripheral nerve disease for many years, but in recent years, peripheral nerve imaging has been used ever more frequently in daily clinical practice. Nerve ultrasound and magnetic resonance (MR) neurography are able to visualize nerve structures reliably. These techniques can aid in localizing nerve pathology and can reveal significant anatomical abnormalities underlying nerve pathology that may have been otherwise undetected by EDX. As such, nerve ultrasound and MR neurography can significantly improve diagnostic accuracy and can have a significant effect on treatment strategy. In this chapter, the basic principles and recent developments of these techniques will be discussed, as well as their potential application in several types of peripheral nerve disease, such as carpal tunnel syndrome (CTS), ulnar neuropathy at the elbow (UNE), radial neuropathy, brachial and lumbosacral plexopathy, neuralgic amyotrophy (NA), fibular, tibial, sciatic, femoral neuropathy, meralgia paresthetica, peripheral nerve trauma, tumors, and inflammatory neuropathies.
Topics: Humans; Peripheral Nervous System Diseases; Ultrasonography; Magnetic Resonance Imaging; Electrodiagnosis
PubMed: 38697740
DOI: 10.1016/B978-0-323-90108-6.00001-6 -
Metabolic Brain Disease Aug 2023Endoplasmic stress response, the unfolded protein response (UPR), is a homeostatic signaling pathway comprising transmembrane sensors that get activated upon alterations... (Review)
Review
Endoplasmic stress response, the unfolded protein response (UPR), is a homeostatic signaling pathway comprising transmembrane sensors that get activated upon alterations in ER luminal environment. Studies suggest a relation between activated UPR pathways and several disease states such as Parkinson, Alzheimer, inflammatory bowel disease, tumor growth, and metabolic syndrome. Diabetic peripheral neuropathy (DPN), a common microvascular complication of diabetes-related chronic hyperglycemia, causes chronic pain, loss of sensation, foot ulcers, amputations, allodynia, hyperalgesia, paresthesia, and spontaneous pain. Factors like disrupted calcium signaling, dyslipidemia, hyperglycemia, inflammation, insulin signaling, and oxidative stress disturb the UPR sensor levels manifesting as DPN. We discuss new effective therapeutic alternatives for DPN that can be developed by targeting UPR pathways like synthetic ER stress inhibitors like 4-PhenylButyric acid (4-PBA), Sephin 1, Salubrinal and natural ER stress inhibitors like Tauroursodeoxycholic acid (TUDCA), Cordycepin, Proanthocyanidins, Crocin, Purple Rice extract and cyanidin and Caffeic Acid Phenethyl Ester (CAPE).
Topics: Humans; Diabetic Neuropathies; Endoplasmic Reticulum Stress; Unfolded Protein Response; Signal Transduction; Hyperglycemia; Diabetes Mellitus
PubMed: 37289403
DOI: 10.1007/s11011-023-01239-x -
Foot and Ankle Clinics Sep 2023Peripheral neuropathies of the foot and ankle can be challenging to diagnose clinically due to concomitant traumatic and nontraumatic or degenerative orthopedic... (Review)
Review
Peripheral neuropathies of the foot and ankle can be challenging to diagnose clinically due to concomitant traumatic and nontraumatic or degenerative orthopedic conditions. Although clinical history, physical examination, and electrodiagnostic testing comprised of nerve conduction velocities and electromyography are used primarily for the identification and classification of peripheral nerve disorders, MR neurography (MRN) can be used to visualize the peripheral nerves as well as the skeletal muscles of the foot and ankle for primary neurogenic pathology and skeletal muscle denervation effect. Proper knowledge of the anatomy and pathophysiology of peripheral nerves is important for an MRN interpretation.
Topics: Humans; Peripheral Nervous System Diseases; Ankle; Magnetic Resonance Imaging; Peripheral Nerves; Magnetic Resonance Spectroscopy
PubMed: 37536819
DOI: 10.1016/j.fcl.2023.04.003 -
Seminars in Ultrasound, CT, and MR Aug 2023The Neuropathy Score Reporting and Data System (NS-RADS) is a newly developed MR imaging-based classification that standardizes reporting and multidisciplinary... (Review)
Review
The Neuropathy Score Reporting and Data System (NS-RADS) is a newly developed MR imaging-based classification that standardizes reporting and multidisciplinary communication for MR imaging diagnosis and follow-up of peripheral neuropathies. NS-RADS classification has shown to be accurate and reliable across different centers, readers' experience levels, and degrees of peripheral neuropathies, which include nerve injury, entrapment, neoplasm, diffuse neuropathy, post-interventional status, and temporal changes in muscle denervation. This article brings a practical review of NS-RADS classification, representative MR cases, and a step-by-step tutorial on how to approach this staging system. Readers can gain knowledge and apply it in their practice, aiming to standardize the communications between specialties and improve patient management.
Topics: Humans; Peripheral Nervous System Diseases; Magnetic Resonance Imaging
PubMed: 37437974
DOI: 10.1053/j.sult.2023.04.003 -
Journal of Diabetes and Its... May 2024We investigated associations between gastrointestinal symptoms - evaluated as a combined weighted symptom score (CWSS) - Diabetic autonomic neuropathy (DAN), and distal...
OBJECTIVE
We investigated associations between gastrointestinal symptoms - evaluated as a combined weighted symptom score (CWSS) - Diabetic autonomic neuropathy (DAN), and distal symmetrical polyneuropathy (DSPN) in type 1 and type 2 diabetes.
RESEARCH DESIGN AND METHODS
Cross-sectional study in a tertiary outpatient clinic. CWSS was calculated based on questionnaires: gastroparesis composite symptom index (GCSI) and gastrointestinal symptom rating score (GSRS). DAN and DSPN were addressed using the composite autonomic symptom score 31 (COMPASS-31) questionnaire, cardiac autonomic reflex tests (CARTs), electrochemical skin conductance (ESC), vibration perception threshold (VPT), Michigan Neuropathy Screening Instrument (MNSI), pain- and thermal sensation. Analyses were adjusted for age, sex, diabetes duration, smoking, LDL-cholesterol, HbA and systolic blood pressure. Type 1 and type 2 diabetes were evaluated separately.
RESULTS
We included 566 with type 1 diabetes and 377 with type 2 diabetes. Mean ± SD age was 58 ± 15 years and 565 (59.9 %) were women. A high CWSS was present in 143 (25 %) with type 1 and 142 (38 %) with type 2 diabetes. The odds of DAN by COMPASS-31 (p < 0.001) were higher in the high score group. For type 1 diabetes, odds of cardiac autonomic neuropathy were higher in the high CWSS group. The odds of DSPN by VPT and MNSI in type 1 diabetes, and by ESC, VPT and pain sensation in type 2 diabetes were higher in the high CWSS group.
CONCLUSIONS
A high symptom score was associated with neuropathy by COMPASS-31 and vibration perception. Gastrointestinal symptom burden associated inconsistently with other neuropathy tests between diabetes types.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Autonomic Nervous System Diseases; Cohort Studies; Cost of Illness; Cross-Sectional Studies; Denmark; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Diabetic Neuropathies; Gastrointestinal Diseases; Scandinavians and Nordic People; Severity of Illness Index; Surveys and Questionnaires; Symptom Burden
PubMed: 38615421
DOI: 10.1016/j.jdiacomp.2024.108745 -
Neurological Sciences : Official... Mar 2024Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease characterized by rapidly progressive dementia, motor impairments, and psychiatric symptoms. Sensory...
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease characterized by rapidly progressive dementia, motor impairments, and psychiatric symptoms. Sensory disturbances were occasionally reported as well. The study aims to describe the sensory symptoms of the disease.
METHODS
The CJD Israeli National Database was screened for patients who presented sensory symptoms throughout the disease course. Symptoms, characteristics, and distribution were reviewed and the demographic and clinical data (sex, etiologies of the disease, age of onset, disease duration, neurological exam finding, tau protein level, EEG and MRI findings) were compared with the demographics and clinical data of CJD without sensory symptoms. Then, the patients with sensory symptoms were divided into patients with symptom distribution consistent with peripheral nervous system (PNS) involvement and central nervous system (CNS) involvement. The demographics and clinical data of the 2 groups were compared.
RESULTS
Eighty-four CJD patients with sensory symptoms and 645 CJD patients without sensory symptoms were included in the study. Sensory symptoms were more common in genetic E200K CJD patients (14.6% vs. 5.6% respectively, p = 0.0005) (chi-squared test). Numbness and neuropathic pain were the most common symptoms and distribution of symptoms of "stocking gloves" with decreased deep tendon reflexes suggesting peripheral neuropathy in 44% of the patients. In these patients, the classical EEG findings of Periodic Sharp Wave Complexes were less often found (58% vs. 22%, p = 0.02) (chi-squared test).
CONCLUSIONS
Sensory symptoms are more common in E200K patients and often follow peripheral neuropathy distribution that suggests PNS involvement.
Topics: Humans; Creutzfeldt-Jakob Syndrome; Neurodegenerative Diseases; Magnetic Resonance Imaging; Diagnosis, Differential; Sensation Disorders; Peripheral Nervous System Diseases
PubMed: 37828389
DOI: 10.1007/s10072-023-07093-0 -
Frontiers in Endocrinology 2023Diabetic neuropathy (DN) is a prevalent and debilitating complication of diabetes, imposing a significant burden on individuals and healthcare systems worldwide. This...
INTRODUCTION
Diabetic neuropathy (DN) is a prevalent and debilitating complication of diabetes, imposing a significant burden on individuals and healthcare systems worldwide. This study presents a comprehensive analysis of the global research landscape in DN, aiming to provide scientists, funders, and decision-makers with valuable insights into the current state of research and future directions.
METHODS
Through a systematic review of published articles, key trends in DN research, including epidemiology, diagnosis, treatment strategies, and gaps in knowledge, are identified and discussed.
RESULTS
The analysis reveals an increasing prevalence of DN alongside the rising incidence of diabetes, emphasizing the urgent need for effective prevention and management strategies. Furthermore, the study highlights the geographical imbalance in research activity, with a majority of studies originating from high-income countries.
DISCUSSION
This study underscores the importance of fostering international collaboration to address the global impact of DN. Key challenges and limitations in DN research are also discussed, including the need for standardized diagnostic criteria, reliable biomarkers, and innovative treatment approaches. By addressing these gaps, promoting collaboration, and increasing research funding, we can pave the way for advancements in DN research and ultimately improve the lives of individuals affected by this debilitating condition.
Topics: Humans; Diabetic Neuropathies; Forecasting; Prevalence; Diabetes Mellitus
PubMed: 38034004
DOI: 10.3389/fendo.2023.1220896 -
Scientific Reports Aug 2023Previous studies disclosed that a high thyroid stimulating hormone level is an independent risk factor for diabetes peripheral neuropathy (DPN) in subclinical...
Previous studies disclosed that a high thyroid stimulating hormone level is an independent risk factor for diabetes peripheral neuropathy (DPN) in subclinical hypothyroidism (SCH) patients with type 2 diabetes mellitus (T2DM). However, whether thyroid metabolism has an effect on DPN in euthyroid T2DM patients remains unknown. The aim of this study was to identify the association between thyroid function and DPN in euthyroid T2DM patients. A set of 580 euthyroid T2DM patients was enrolled in the current study and stratified into DPN and Non-DPN groups. Mann-Whitney U test was performed to analyze the continuous variables of biochemical and thyroid metabolism indicators, and the Chi-square test was used to compare the categorical variables. Spearman correlation analysis was performed to analyze the relationship between clinical indicators and free thyroxine (FT4). By using the logistic regression analysis, the prevalence of DPN in different thyroid function indicators were evaluated. T2DM patients with DPN had obviously lower levels of aspartate aminotransferase (AST), alpha-hydroxybutyric dehydrogenase (α-HBDH), superoxide dismutase (SOD), calcium (Ca), creatinine (Cr), uric acid (UA), retinol binding protein (RBP), total protein (TP), albumin (ALB), alanine aminotransferase (ALT) and FT4 than the T2DM patients without DPN (P < 0.05). FT4 was associated with TP, prealbumin (PA), ALB, SOD, anion gap (AG), Ca, chlorine (Cl), UA, RBP, apoprotein A (Apo A), apoprotein B (Apo B), apoprotein E (Apo E), and total cholesterol (TC). According to the FT4 quartile, participants were sequentially divided into four groups to compare the prevalence of DPN between each group. The data suggested that the prevalence of DPN in these four groups was 53.79%, 53.28%, 54.97%, 38.10%, respectively. Moreover, compared with quartile 4, patients in quartile 1, 2, 3 all had a significantly higher risk of DPN (P = 0.007, P = 0.011, P = 0.004). The level of FT4 was negatively correlated with the prevalence of DPN in euthyroid T2DM patients.
Topics: Humans; Diabetes Mellitus, Type 2; Thyroid Gland; Risk Factors; Albumins; Calcium, Dietary; Apoproteins; Peripheral Nervous System Diseases
PubMed: 37596396
DOI: 10.1038/s41598-023-40752-y -
Obstetrics and Gynecology Dec 2023To investigate the efficacy of cryocompression therapy to prevent chemotherapy-induced peripheral neuropathy. (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVE
To investigate the efficacy of cryocompression therapy to prevent chemotherapy-induced peripheral neuropathy.
METHODS
This single-institution, randomized, self-controlled trial of cryocompression enrolled gynecologic cancer patients planned for five to six cycles neurotoxic chemotherapy. Exclusion criteria were prior neurotoxic chemotherapy or baseline peripheral neuropathy. Participants were randomized to cryocompression on dominant versus non-dominant hand and foot (treatment), with no intervention on the opposite side (control). Compression socks and gloves and ice bags were applied 15 minutes before, during, and 15 minutes after infusion. Primary outcome measures included the PNQ (Patient Neurotoxicity Questionnaire) and the Semmes-Weinstein monofilament test; secondary outcomes included the FACT/GOG-NTX (Functional Assessment of Cancer Therapy/Gynecologic Oncology Group - Neurotoxicity) and patient acceptability and tolerability. Sixty patients completing the study were necessary to detect a 70% reduction in the odds of PNQ grade C or higher peripheral sensory neuropathy with 80% power.
RESULTS
Ninety-one patients were enrolled from January 2021 to October 2022; 69 were eligible for final analysis. Of the 91 patients, 64.8% were White, 30.8% were Black, and 1.1% were Hispanic or Latina. With successive cycles, more patients had sensory PNQ grade C or higher neuropathy on the control side compared with the cryocompression side. Cryocompression decreased the odds of sensory neuropathy (PNQ grade C or higher) by 46% at final visit (odds ratio 0.54, 95% CI 0.31-0.94; P =.03). There was no difference in tactile sensitivity based on the monofilament test between sides at the final visit. At the final visit, average FACT/GOG-NTX-11 (Functional Assessment of Cancer Therapy/Gynecologic Oncology Group - Neurotoxicity 11 Item Version) scores were significantly lower on the cryocompression than the control side (estimate -0.97, 95% CI -1.89 to -0.06; P =.04), as were FACT/GOG-NTX-4 (Functional Assessment of Cancer Therapy/Gynecologic Oncology Group - Neurotoxicity 4 Item Version) scores (estimate -0.35, 95% CI -0.64 to -0.05; P =.02). More than 85% of patients assessed the intervention as acceptable and tolerable.
CONCLUSIONS
Cryocompression therapy reduces subjective chemotherapy-induced peripheral sensory neuropathy in patients who are receiving paclitaxel or cisplatin for gynecologic cancer.
CLINICAL TRIAL REGISTRATION
ClinicalTrials.gov , NCT04563130.
Topics: Humans; Female; Peripheral Nervous System Diseases; Paclitaxel; Neurotoxicity Syndromes; Genital Neoplasms, Female; Antineoplastic Agents
PubMed: 37883997
DOI: 10.1097/AOG.0000000000005419