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European Journal of Neurology Jun 2021Chronic axonal polyneuropathy is a common, usually multifactorial, disease for which there is no treatment yet available. We investigated the association between...
BACKGROUND AND PURPOSE
Chronic axonal polyneuropathy is a common, usually multifactorial, disease for which there is no treatment yet available. We investigated the association between cardiovascular health, defined by the health score of the American Heart Association, and chronic axonal polyneuropathy.
METHODS
Between June 2013 and January 2017, we investigated participants of the Rotterdam Study, a population-based cohort study. Participants were screened for polyneuropathy and categorized as having no, possible, probable or definite polyneuropathy. The cardiovascular health score (range 0-14; higher score reflecting better health) consisted of four health behaviours (diet, physical activity, smoking and body mass index) and three health factors (blood pressure, serum cholesterol and fasting glucose level).
RESULTS
We included 1919 participants, of whom 120 (6.3%) had definite polyneuropathy. The median (interquartile range [IQR]) age was 69.0 (58.6-73.7) years and 53.4% were women. A higher cardiovascular health score was associated with a lower prevalence of definite polyneuropathy (per point increase: odds ratio [OR] 0.90, 95% confidence interval [CI] 0.84-0.96). Optimal cardiovascular health (score≥10) was strongly associated with a lower prevalence of definite polyneuropathy (OR 0.55, 95% CI 0.32-0.90). An increase in health factors and health behaviour scores separately was associated with a lower prevalence of polyneuropathy (per point increase: OR 0.82, 95% CI 0.71-0.95 and OR 0.86, 95% CI 0.78-0.96, respectively). The association between a lower cardiovascular health score and lower sural nerve amplitude was not significant after correction for covariates (difference 0.07µV, 95% CI -0.02-0.17).
CONCLUSIONS
Better cardiovascular health, consisting of both modifiable health behaviours and health factors, is associated with a lower prevalence of chronic axonal polyneuropathy.
Topics: Aged; Body Mass Index; Cardiovascular Diseases; Cohort Studies; Exercise; Female; Humans; Polyneuropathies; Risk Factors; United States
PubMed: 33590563
DOI: 10.1111/ene.14777 -
Neurological Sciences : Official... Oct 2023Early diagnosis of hereditary ATTR polyneuropathy (ATTRv-PN) is important since treatment options have become available, which are most effective early in the disease...
BACKGROUND
Early diagnosis of hereditary ATTR polyneuropathy (ATTRv-PN) is important since treatment options have become available, which are most effective early in the disease course. ATTRv-PN is likely underdiagnosed as patients might be misdiagnosed with idiopathic polyneuropathy. It is uncertain if it is useful to test for TTR gene mutations in patients with a typical presentation for chronic idiopathic axonal polyneuropathy (CIAP) and which are the distinguishing clinical features.
METHODS
We carried out a retrospective cohort study to assess the yield of TTR gene sequencing in patients with polyneuropathy and assessed if the identified patients with ATTRv-PN had a clinical presentation typical of CIAP. Additionally, we assessed which clinical features, including previously defined red flag symptoms, can differentiate between patients with CIAP and ATTRv-PN and assessed the performance of the TTR suspicion index.
RESULTS
Out of 338 patients with polyneuropathy, 10 patients had a pathogenic TTR gene mutation (all p.Val50Met) and none had a clinical presentation typical of CIAP. Patients with ATTRv-PN more often had bilateral CTS, motor involvement of arms, cardiac involvement, family history suggestive of hATTRv, and autonomic symptoms than patients with CIAP. All patients with ATTRv-PN as well as 70% of patients with CIAP fulfilled the suspicion index.
CONCLUSION
Routine TTR gene sequencing in patients with a typical presentation for CIAP is not useful. However, red flag symptoms can differentiate patients with ATTRv-PN from patients with CIAP. We propose an adjusted version of the TTR suspicion index to increase diagnostic yield.
Topics: Humans; Amyloid Neuropathies, Familial; Prealbumin; Retrospective Studies; Polyneuropathies; Disease Progression
PubMed: 37266816
DOI: 10.1007/s10072-023-06859-w -
Nutricion Hospitalaria Jul 2019Polyneuropathy in the critically ill patient was defined as a generalized weakness, acquired during Intensive Care Unit (ICU) admittance and attributed to lesion of the... (Review)
Review
Polyneuropathy in the critically ill patient was defined as a generalized weakness, acquired during Intensive Care Unit (ICU) admittance and attributed to lesion of the peripheral nerve. Research in this field progressed over time, revealing the crucial role of muscle injury in this disease, to the point of re-naming the disorder as ICU adquired weakness (ICUAW). Muscle damage is common in severe illness, and may be classified in qualitative (weakness) or quantitative (decrease in mass) muscle loss. The most frequent scenario in these patients, is simultaneous change in quality and quantity of muscle; resulting in a challenging and delayed recovery during hospital admittance and after discharge. Multiple causes have been identified in the pathogenesis of this disorder, such as: prolonged bed rest, inadequate intake of nutrients and exposure to drugs that affect muscle structure and contraction. The assessment of muscle mass using images provided by ultrasound or computerized tomography may guide follow up. The prevention and treatment of ICUAW requires a multimodal approach: early mobilization and exercise, appropriate nutritional prescription and, occasionally, muscle protein synthesis stimulants. Further studies will clarify more aspects regarding critically ill patients suffering from muscle injury, in order to better address prevention and treatment of ICUAW.
Topics: Critical Care; Critical Illness; Muscle Strength; Muscle Weakness; Muscle, Skeletal; Polyneuropathies
PubMed: 31189318
DOI: 10.20960/nh.02676 -
The Cochrane Database of Systematic... Jun 2017Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe... (Review)
Review
BACKGROUND
Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for evaluation of polyneuropathy. There is a need to gather and review emerging evidence on treatments, as the number of people affected is likely to increase in ageing populations. This is an update of a review first published in 2004 and previously updated in 2006, 2008, 2011 and 2013.
OBJECTIVES
To assess the effects of drug therapy for chronic idiopathic axonal polyneuropathy for reducing disability and ameliorating neurological symptoms and associated impairments, and to assess any adverse effects of treatment.
SEARCH METHODS
In July 2016, we searched Cochrane Central Register of Controlled Trials and the Cochrane Database of Systematic Reviews in the Cochrane Library, MEDLINE, Embase, and the Web of Science. We searched two trials registries for ongoing trials. We also handsearched the reference lists of relevant articles, reviews and textbooks identified electronically, and we would have contacted authors and other experts in the field to identify additional studies if this seemed useful.
SELECTION CRITERIA
We sought all randomised or quasi-randomised (alternate or other systematic treatment allocation) trials that examined the effects of any drug therapy in people with CIAP at least one year after the onset of treatment. People with CIAP had to fulfil the following criteria: age 40 years or older, distal sensory or sensorimotor polyneuropathy, absence of systemic or other neurological disease, chronic clinical course not reaching a nadir in less than two months, exclusion of any recognised cause of the polyneuropathy by medical history taking, clinical or laboratory investigations, and electrophysiological studies in agreement with axonal polyneuropathy, without evidence of demyelinating features. The primary outcome was the proportion of participants with a significant improvement in disability. Secondary outcomes were change in the mean disability score, change in the proportion of participants who make use of walking aids, change in the mean Medical Research Council sum score, degree of pain relief and/or reduction of other positive sensory symptoms, change in the proportion of participants with pain or other positive sensory symptoms, and frequency of adverse effects.
DATA COLLECTION AND ANALYSIS
Two review authors independently reviewed the results of the literature search and extracted details of trial methodology and outcome data of all potentially relevant trials.
MAIN RESULTS
We identified 39 studies and assessed them for possible inclusion in the review, but we excluded all of them because of insufficient quality or lack of relevance. We summarised evidence from non-randomised studies in the Discussion.
AUTHORS' CONCLUSIONS
Even though CIAP has been clearly described and delineated, no adequate randomised or quasi-randomised controlled clinical treatment trials have been performed. In their absence there is no proven efficacious drug therapy.
Topics: Aged; Axons; Chronic Disease; Gait Ataxia; Humans; Leg; Polyneuropathies
PubMed: 28631805
DOI: 10.1002/14651858.CD003456.pub3 -
Neurologic Clinics May 2013Chronic sensory or sensorimotor polyneuropathy is a common cause for referral to neurologists. Despite extensive diagnostic testing, up to one-third of these patients... (Review)
Review
Chronic sensory or sensorimotor polyneuropathy is a common cause for referral to neurologists. Despite extensive diagnostic testing, up to one-third of these patients remain without a known cause, and are referred to as having cryptogenic sensory peripheral neuropathy. Symptoms progress slowly. On examination, there may be additional mild toe flexion and extension weakness. Electrophysiologic testing and histology reveals axonal neuropathy. Prognosis is usually favorable, as most patients maintain independent ambulation. Besides patient education and reassurance, management is focused on pharmacotherapy for neuropathic pain and physical therapy for balance training, and, occasionally, assistive devices.
Topics: Aged, 80 and over; Electrodiagnosis; Genetic Testing; Humans; Male; Neural Conduction; Pain Management; Polyneuropathies; Sensation Disorders
PubMed: 23642719
DOI: 10.1016/j.ncl.2013.01.008 -
Current Diabetes Reports Jun 2015Distal symmetric polyneuropathy (DSPN), the most common form of diabetic neuropathy, has a complex pathophysiology and can be a major source of physical and psychologic... (Review)
Review
Distal symmetric polyneuropathy (DSPN), the most common form of diabetic neuropathy, has a complex pathophysiology and can be a major source of physical and psychologic disability. The management of DSPN can be frustrating for both patient and physician. This article provides a general overview of typical patient pathways in DSPN, and highlights variations in diagnosis, management, and referral patterns among different providers. DSPN is managed in several settings by primary care physicians (PCPs), specialists, and nurse practitioners. The initial clinical management of the patient is often dependent on the presenting complaint, the referral pattern of the provider, level of comfort of the PCP in managing diabetic complications, and geographic access to specialists. The primary treatment of DSPN focuses mainly on glycemic control and adjustment of modifiable risk factors, but other causes of neuropathy should also be investigated. Several pharmacologic agents are recommended by treatment guidelines, and as DSPN typically exists with comorbid conditions, a multimodal therapeutic approach should be considered. Barriers to effective management include failure to recognize DSPN, and misdiagnosis. Patient education also remains important. Referral patterns vary widely according to geographic location, access to services, provider preferences, and comfort in managing complex aspects of the disease. The variability in patient pathways affects patient education, satisfaction, and outcomes. Standardized screening tools, a multidisciplinary team approach, and treatment algorithms for diabetic neuropathy should improve future care. To improve patient outcomes, DSPN needs to be diagnosed sooner and interventions made before significant nerve damage occurs.
Topics: Diabetic Neuropathies; Disease Management; Humans; Polyneuropathies
PubMed: 25899758
DOI: 10.1007/s11892-015-0609-2 -
Journal of the Peripheral Nervous... Dec 2020The aim of this study was to evaluate the presence and characterization of chemotherapy-induced neuropathy (CIPN) and neuropathic pain 5 years after adjuvant...
The aim of this study was to evaluate the presence and characterization of chemotherapy-induced neuropathy (CIPN) and neuropathic pain 5 years after adjuvant chemotherapy with docetaxel or oxaliplatin. Patients from an ongoing prospective study, who had received adjuvant chemotherapy with docetaxel or oxaliplatin in 2011 to 2012 were invited to participate. The patients underwent a thorough examination with interview, neurological examination, questionnaires, assessment tools, nerve conduction studies (NCS), quantitative sensory testing, MScan motor unit number estimation (MUNE), and corneal confocal microscopy (CCM). Patients were divided into no, possible, probable, and confirmed CIPN. Out of the 132 eligible patients, 63 agreed to participate: 28 had received docetaxel and 35 had received oxaliplatin. Forty-one percent had confirmed CIPN, 34% possible or probable CIPN, and 22% did not have CIPN. The CIPN was characterized mainly by sensory nerve fiber loss, with a more pronounced large fiber than small fiber loss but also some motor fiber loss identified on NCS and MUNE. In general, patients had mild neuropathy with relatively low scores on assessment tools and no association with mood and quality of life. CCM was not useful as a diagnostic tool. Of the patients with probable or confirmed CIPN, 30% experienced pain, which was most often mild, but still interfered moderately with daily life in 20% to 25% and was associated with lower quality of life. In conclusion CIPN was confirmed in 41% 5 years after chemotherapy. The neuropathy was generally mild, but in patients with neuropathic pain it was associated with lower quality of life.
Topics: Aged; Antineoplastic Agents; Diagnostic Techniques, Neurological; Docetaxel; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasms; Neuralgia; Oxaliplatin; Polyneuropathies; Prospective Studies; Quality of Life; Severity of Illness Index
PubMed: 32902058
DOI: 10.1111/jns.12413 -
Tidsskrift For Den Norske Laegeforening... Feb 2007Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many possible etiologies. This review presents diagnostic guidelines for... (Review)
Review
BACKGROUND
Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many possible etiologies. This review presents diagnostic guidelines for physicians without specialist competence in neuromuscular disorders.
MATERIAL AND METHOD
The article is based on a review of articles identified in PubMed using the search terms "peripheral neuropathy", "cause" and "investigation", relevant book chapters and own clinical experience.
RESULTS AND INTERPRETATION
All patients should undergo a routine investigation for the most common causes of polyneuropathy, such as diabetes, heredity, alcohol abuse, toxic medications and agents, symptoms of Sjögren's syndrome and renal failure. The following laboratory assessments should be done if the medical history proves negative: glucose, haemoglobin, leucocytes, thrombocytes, ESR, creatinin, ALAT, GT, vitamin B12, serum electrophoresis, TSH and thyroxin. If the routine investigation proves negative, a targeted approach based on clinical type and electrophysiological findings is recommended. The most common sensory type with slowly progressing, symmetric sensory symptoms beginning in the feet can often be classified as cryptogenic without further investigation. Polyneuropathies with subacute onset, progressive weakness, asymmetric symptoms, proximal weakness, selective involvement of sensory fibres or demyelinating pathology, or other organ manifestations, have different etiologies that necessitate individually focused investigations.
Topics: Chronic Disease; Clinical Competence; Comorbidity; Humans; Neurologic Examination; Polyneuropathies; Referral and Consultation; Sensation Disorders
PubMed: 17279107
DOI: No ID Found -
Neurology India 2011Multifocal motor neuropathy (MMN) is a unique disorder characterized by slowly progressive, asymmetric, distal and upper limb predominant weakness without significant... (Review)
Review
Multifocal motor neuropathy (MMN) is a unique disorder characterized by slowly progressive, asymmetric, distal and upper limb predominant weakness without significant sensory abnormalities. Electrophysiology is crucial to the diagnosis, revealing the hallmark partial conduction block. MMN is considered immune mediated due to the association with anti-GM1 antibodies and the response to immunomodulatory treatment. It is paramount to recognize MMN from other motor neuronopathies or peripheral neuropathies as it is treatable. Advances in pathogenesis, clinical features, electrophysiology, diagnostic studies and treatment are reviewed. References for this review were identified from literature search on Pubmed limited to dates from 1988 to 2011. Papers were selected if relevant to the review topic and published in English.
Topics: Diagnosis, Differential; Electrophysiology; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Motor Neuron Disease; Polyneuropathies
PubMed: 22019654
DOI: 10.4103/0028-3886.86544 -
Annals of Clinical and Translational... Oct 2020Previous studies in Parkinson's disease (PD) patients have demonstrated a high prevalence of polyneuropathy (PNP) and pronounced alpha-Synuclein pathology in dermal...
OBJECTIVE
Previous studies in Parkinson's disease (PD) patients have demonstrated a high prevalence of polyneuropathy (PNP) and pronounced alpha-Synuclein pathology in dermal nerve fibers already at early disease stages. The aim of this study was to analyze associations between the prevalence and severity of PNP with nonmotor and motor symptoms in PD patients.
METHODS
Fifty PD patients were characterized comprehensively for the presence of clinical symptoms (nonmotor and motor), electrophysiologic alterations and - for the first time - using high-resolution ultrasound of peripheral nerves.
RESULTS
Sixty-two percent of PD patients showed electrophysiological pathology of PNP. The prevalence of patient-reported PNP symptoms was 86% and was particularly present in patients with longer disease duration, compromised scores of nonmotor and motor symptoms as well as with a negative evaluation of quality of life. Seventy-five percent of patients showed morphologic alterations similar to axonal PNP in high-resolution ultrasound compared to healthy controls.
INTERPRETATION
The study demonstrates the high burden of peripheral nervous system disease in Parkinson's disease. It advocates further studies to delineate the underlying pathophysiological mechanisms in order to optimize treatment approaches for PD, including the associated PNP.
Topics: Aged; Female; Humans; Male; Middle Aged; Parkinson Disease; Peripheral Nervous System Diseases; Polyneuropathies; Prevalence; Quality of Life; Severity of Illness Index
PubMed: 32940017
DOI: 10.1002/acn3.51182