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European Journal of Neurology Dec 2022We evaluated the clinical and neurophysiological efficacy of rituximab (RTX) in a neurophysiologically homogeneous group of patients with monoclonal gammopathy and...
Efficacy of rituximab in anti-myelin-associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow-up.
BACKGROUND AND PURPOSE
We evaluated the clinical and neurophysiological efficacy of rituximab (RTX) in a neurophysiologically homogeneous group of patients with monoclonal gammopathy and immunoglobulin M (IgM) anti-myelin-associated glycoprotein antibody (anti-MAG) demyelinating polyneuropathy.
METHODS
Twenty three anti-MAG-positive polyneuropathic patients were prospectively evaluated before and for 2 years after treatment with RTX 375 mg/m . The Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale (INCAT-ds), modified INCAT sensory score (mISS), Medical Research Council sum score, Patients' Global Impression of Change scale were used, IgM levels were assessed and extensive electrophysiological examinations were performed before (T0) and 1 year (T1) and 2 years (T2) after RTX treatment.
RESULTS
At T1 and T2 there was a significant reduction from T0 both in mISS and in INCAT-ds, with a p value < 0.001 in the inferential Friedman's test overall analysis. Ulnar nerve Terminal Latency Index and distal motor latency significantly changed from T0 to T1 and in the overall analysis (p = 0.001 and p = 0.002), and ulnar nerve sensory nerve action potential (SNAP) amplitude was significantly increased at T2 from T1, with a p value < 0.001 in the overall analysis. Analysis of the receiver-operating characteristic curves showed that a 41.8% increase in SNAP amplitude in the ulnar nerve at T2 from T0 was a fair predictor of a mISS reduction of ≥2 points (area under the curve 0.85; p = 0.005; sensitivity: 90.9%, specificity: 83.3%).
CONCLUSIONS
This study suggests that RTX is effective in patients with clinically active demyelinating anti-MAG neuropathy over 2 years of follow-up, and that some neurophysiological variables might be useful for monitoring this efficacy.
Topics: Humans; Rituximab; Follow-Up Studies; Polyneuropathies; Paraproteinemias; Immunoglobulin M; Autoantibodies
PubMed: 36083713
DOI: 10.1111/ene.15553 -
Basic & Clinical Pharmacology &... Aug 2014Chemotherapy-induced polyneuropathy (CIPN) is a common, but underestimated, clinical challenge. Incidence varies depending on many factors that are equally as important... (Review)
Review
Chemotherapy-induced polyneuropathy (CIPN) is a common, but underestimated, clinical challenge. Incidence varies depending on many factors that are equally as important as the type of chemotherapeutic agent itself. Moreover, the assessment of CIPN is still uncertain, as several of the most frequently used scales do not rely on a formal neurological evaluation and depend on patients' reports and examiners' interpretations. Therefore, the aim of this MiniReview was to introduce the most common chemotherapies that cause neuropathy, and in addition to this, highlight the most significant differences between the neuropathic pain scales, which are most commonly used for assessing CIPN.
Topics: Antineoplastic Agents; Humans; Incidence; Neurotoxicity Syndromes; Pain Measurement; Polyneuropathies; Surveys and Questionnaires
PubMed: 24796774
DOI: 10.1111/bcpt.12262 -
Journal of Neurology, Neurosurgery, and... Aug 2006Three polyneuropathy scores are described, which seem to be valid and sensible measures to score dysfunction and disability in patients with generalised motor...
Three polyneuropathy scores are described, which seem to be valid and sensible measures to score dysfunction and disability in patients with generalised motor neuropathies
Topics: Activities of Daily Living; Disabled Persons; Humans; Polyneuropathies; Reference Values; Running; Severity of Illness Index; Walking
PubMed: 16709581
DOI: 10.1136/jnnp.2006.093781 -
Neuroscience Letters Jan 2021Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will... (Review)
Review
Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. The intermediate steps between the loss of these functions and the initiation of axon degeneration are unknown but the importance of these processes provides insightful clues. Prevalent demyelinating diseases of the PNS include the inherited neuropathies Charcot-Marie-Tooth Disease, Type 1 (CMT1) and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and the inflammatory diseases Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Secondary axon degeneration is a common feature of demyelinating neuropathies and this process is often correlated with clinical deficits and long-lasting disability in patients. There is abundant electrophysiological and histological evidence for secondary axon degeneration in patients and rodent models of PNS demyelinating diseases. Fully understanding the involvement of secondary axon degeneration in these diseases is essential for expanding our knowledge of disease pathogenesis and prognosis, which will be essential for developing novel therapeutic strategies.
Topics: Animals; Arthrogryposis; Axons; Charcot-Marie-Tooth Disease; Demyelinating Diseases; Hereditary Sensory and Motor Neuropathy; Humans; Nerve Degeneration; Polyneuropathies; Schwann Cells
PubMed: 33359733
DOI: 10.1016/j.neulet.2020.135595 -
PloS One 2024To establish a simple electrophysiological scale for patients with distal symmetric axonal polyneuropathy, in order to promote standardized and informative...
OBJECTIVE
To establish a simple electrophysiological scale for patients with distal symmetric axonal polyneuropathy, in order to promote standardized and informative electrodiagnostic reporting, and understand the complex relationship between electrophysiological and clinical polyneuropathy severity.
METHODS
We included 76 patients with distal symmetric axonal polyneuropathy, from a cohort of 151 patients with polyneuropathy prospectively recruited from November 2016 to May 2017. Patients underwent nerve conduction studies (NCS), were evaluated by the Toronto Clinical Neuropathy Score (TCNS), and additional tests. The number of abnormal NCS parameters was determined, within the range of 0-4, considering low amplitude or conduction velocity in the sural and peroneal nerve.
RESULTS
Higher number of NCS abnormalities was associated with higher TCNS, indicating more severe polyneuropathy. Polyneuropathy severity per the TCNS was most frequently (63%-70%) mild in patients with a low (0-1) number of NCS abnormalities, and most frequently (57%-67%) severe in patients with a high number (3-4) of NCS abnormalities, while patients with an intermediate (2) number of NCS abnormalities showed mainly mild and moderate severity with equal distribution (40%).
CONCLUSIONS
A simple NCS classification system can objectively grade polyneuropathy severity, although significant overlap exists especially at the intermediate range, underscoring the importance of clinical based scoring.
Topics: Humans; Male; Female; Polyneuropathies; Middle Aged; Neural Conduction; Severity of Illness Index; Aged; Adult; Prospective Studies; Electrodiagnosis
PubMed: 38776287
DOI: 10.1371/journal.pone.0302491 -
Medical Science Monitor : International... Nov 2021BACKGROUND Chronic obstructive pulmonary disease (COPD) is a life-threatening and devastating disease associated with low-grade systemic inflammation. In adults, the...
BACKGROUND Chronic obstructive pulmonary disease (COPD) is a life-threatening and devastating disease associated with low-grade systemic inflammation. In adults, the most common disease of the peripheral nervous system is peripheral neuropathy. While most polyneuropathy has a mixed presentation, some cases are motor dominant and others are sensory dominant. We investigated polyneuropathy in patients with COPD and hypothesized that low-grade systemic inflammation and other pathologies in patients with COPD cause peripheral axonal polyneuropathy. MATERIAL AND METHODS We included 62 patients with COPD without any neurological signs or symptoms, and 30 healthy volunteers with no known neurological or pulmonary diseases as controls. There were 38 men in the COPD group and 17 men in the control group; the mean ages of the 2 groups were 64.88 and 62.7 years, respectively. According to the Global Initiative for Chronic Obstructive Lung Disease COPD report, all COPD patients were group D. After collecting demographic and clinical characteristics of the participants, we performed an electrophysiological examination to investigate polyneuropathy and pulmonary function test results. C-reactive protein, hemoglobin, creatinine, partial carbon dioxide pressure (pCO₂) levels were recorded. Electrophysiological examination was performed with a Medelec Synergy device using standard neurographic procedures, and the results were assessed. RESULTS Significant differences were found for forced expiratory volume in 1 sec (FEV1), %FEV1, forced vital capacity (FVC), %FVC, pCO₂, and hemoglobin and creatinine levels, but all participants had a creatinine level within the normal range. There was no difference in sensory neuropathy between the groups, but a significant difference was found in terms of motor neuropathy. CONCLUSIONS As noted in previous studies, systemic inflammation, increased oxidative stress, decreased oxygen pressure, and multiple comorbidities in patients with COPD may all contribute to the development of neuropathy.
Topics: Female; Humans; Male; Middle Aged; Polyneuropathies; Pulmonary Disease, Chronic Obstructive
PubMed: 34750341
DOI: 10.12659/MSM.932690 -
Scientific Reports Jun 2023Postmenopausal status is a risk factor for distal sensory polyneuropathy-the most common type of peripheral neuropathy. We aimed to investigate associations between...
Association of reproductive factors and exogenous hormone use with distal sensory polyneuropathy among postmenopausal women in the United States: results from 1999 to 2004 NHANES.
Postmenopausal status is a risk factor for distal sensory polyneuropathy-the most common type of peripheral neuropathy. We aimed to investigate associations between reproductive factors and history of exogenous hormone use with distal sensory polyneuropathy among postmenopausal women in the United States using data from the National Health and Nutrition Examination Survey 1999-2004, and to explore the modifying effects of ethnicity on these associations. We conducted a cross-sectional study among postmenopausal women aged ≥ 40 years. Women with a history of diabetes, stroke, cancer, cardiovascular disease, thyroid disease, liver disease, weak or failing kidneys, or amputation were excluded. Distal sensory polyneuropathy was measured using a 10-g monofilament test, and a questionnaire was used to collect data on reproductive history. Multivariable survey logistic regression was used to test the association between reproductive history variables and distal sensory polyneuropathy. In total, 1144 postmenopausal women aged ≥ 40 years were included. The adjusted odds ratios were 8.13 [95% confidence interval (CI) 1.24-53.28] and 3.18 (95% CI 1.32-7.68) for age at menarche < 11 years and time since menopause > 20 years, respectively, which were positively associated with distal sensory polyneuropathy; adjusted odds ratios were 0.45 for the history of breastfeeding (95% CI 0.21-0.99) and 0.41 for exogenous hormone use (95% CI 0.19-0.87) were negatively associated. Subgroup analysis revealed ethnicity-based heterogeneity in these associations. Age at menarche, time since menopause, breastfeeding, and exogenous hormone use were associated with distal sensory polyneuropathy. Ethnicity significantly modified these associations.
Topics: Female; Humans; United States; Postmenopause; Nutrition Surveys; Reproductive History; Cross-Sectional Studies; Menopause; Risk Factors; Menarche; Polyneuropathies; Hormones
PubMed: 37286578
DOI: 10.1038/s41598-023-35934-7 -
Supportive Care in Cancer : Official... Mar 2023Chemotherapy-related polyneuropathy (CIPN) is a very common, often dose-limiting side effect that affects the patients' quality of life. Treatment usually consists of a...
PURPOSE
Chemotherapy-related polyneuropathy (CIPN) is a very common, often dose-limiting side effect that affects the patients' quality of life. Treatment usually consists of a combination of medicinal, medical, and individualized treatment approaches, although the effectiveness of these therapies is insufficient for many patients. The aim of this article is to review and evaluate the impact of CIPN on patients' daily lives and possible effective treatment approaches.
METHODS
A standardized questionnaire was developed based on ten anonymous telephone interviews with CIPN patients. The content of the questionnaire was divided into 5 categories: demographics, clinical presentation, everyday symptoms, treatment of CIPN symptoms, and medical care. Mostly closed questions were used but multiple choice and individual additions by free text answers were possible.
RESULTS
CIPN limits patients' quality of life over a long period of time. In addition to diurnal and situational fluctuations, the emotional burden negatively affects patients' daily lives in many ways. From the patients' point of view, the individually implemented therapy measures were most effective in treating their complaints. But even the combination of different therapy methods insufficiently alleviates the symptoms of the patients.
CONCLUSION
It is important and necessary to comprehensively inform patients about CIPN as a possible side effect, to point out prevention strategies, and to critically examine and evaluate different therapy approaches. In this way, misunderstandings of the doctor-patient relationship can be avoided. In addition, patient satisfaction and quality of life can be increased in the long term.
Topics: Humans; Antineoplastic Agents; Quality of Life; Physician-Patient Relations; Peripheral Nervous System Diseases; Polyneuropathies; Drug-Related Side Effects and Adverse Reactions; Neoplasms
PubMed: 36971861
DOI: 10.1007/s00520-023-07688-5 -
Journal of the Neurological Sciences Oct 2019Hereditary transthyretin-mediated amyloidosis (hATTR amyloidosis) is a rare, life-threatening disease, caused by point mutations in the transthyretin gene. It is a... (Review)
Review
Hereditary transthyretin-mediated amyloidosis (hATTR amyloidosis) is a rare, life-threatening disease, caused by point mutations in the transthyretin gene. It is a heterogeneous, multisystem disease with rapidly progressing polyneuropathy (including sensory, motor, and autonomic impairments) and cardiac dysfunction. Measures used to assess polyneuropathy in other diseases have been tested as endpoints in hATTR amyloidosis clinical trials (i.e. Neuropathy Impairment Score [NIS], NIS-lower limb, and NIS + 7), yet the unique nature of the polyneuropathy in this disease has necessitated modifications to these scales. In particular, the heterogeneous impairment and the aggressive disease course have been key drivers in developing scales that better capture the disease burden and progression of polyneuropathy in hATTR amyloidosis. The modified NIS + 7 (mNIS + 7) scale was specifically designed to assess polyneuropathy impairment in patients with hATTR amyloidosis, and has been the primary endpoint in two recent, phase III studies in this disease. The mNIS + 7 uses highly standardized, quantitative, and referenced assessments to quantify decreased muscle weakness, muscle stretch reflexes, sensory loss, and autonomic impairment. Physicians using this scale in clinical trials should be specifically trained and monitored to minimize variability. This article discusses the different scales that have been/are being used to assess polyneuropathy in patients with hATTR amyloidosis, their correlation with other disease assessments, and reflects on how and why scales have evolved to the latest iteration of mNIS + 7.
Topics: Amyloid Neuropathies, Familial; Humans; Polyneuropathies; Symptom Assessment
PubMed: 31445300
DOI: 10.1016/j.jns.2019.116424 -
British Journal of Clinical Pharmacology Sep 2017In a previous study, we found a positive association between statin use and polyneuropathy risk. Other studies reported equivocal results. The present study aimed to...
AIM
In a previous study, we found a positive association between statin use and polyneuropathy risk. Other studies reported equivocal results. The present study aimed to confirm our findings with a design similar to that used in our previous study but with a larger data set.
METHODS
We searched medical registry data to identify patients diagnosed with incident polyneuropathy of no known cause (idiopathic polyneuropathy) between 1999 and 2013; we verified diagnoses through medical records. For each case, we recruited 20 general population controls with no previous history of polyneuropathy. Controls were matched to their respective case for age and gender. We ascertained the prior statin use of cases and controls through a prescription registry. Based on this information, exposure to statins was categorized into 'ever use' or 'never use'. Ever use of statins was classified by how recently they had been used ('current use' or 'past use'); current use was further classified into long-term use (5+ years) and high- or low-intensity use. We used conditional logistic regression to calculate odds ratios (ORs) with 95% confidence intervals (CIs) to examine associations between polyneuropathy and statin use.
RESULTS
We included 370 validated cases and 7400 controls. Ever use of statins was not associated with an elevated risk of polyneuropathy (OR 1.14, 95% CI 0.84, 1.54). Similarly, we found no associations between polyneuropathy risk and current use (OR 1.11, 95% CI 0.79, 1.53), long-term use (OR 1.13, 95% CI 0.66, 1.92) or high-intensity statin use (OR 1.05, 95% CI 0.59, 1.84).
CONCLUSION
Statin use was not associated with an increased risk of idiopathic polyneuropathy.
Topics: Aged; Case-Control Studies; Denmark; Female; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Male; Middle Aged; Polyneuropathies; Registries
PubMed: 28370351
DOI: 10.1111/bcp.13298