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Medizinische Klinik, Intensivmedizin... Oct 2017An average of 50-80% of patients treated in the intensive care unit is affected by disturbances of neuromuscular functions due to damage to the nerves and muscles, which... (Review)
Review
An average of 50-80% of patients treated in the intensive care unit is affected by disturbances of neuromuscular functions due to damage to the nerves and muscles, which has led to the terms critical illness polyneuropathy and myopathy. Both components occur in 30-50% of patients, while the others predominantly show a pure myopathy, while pure neuropathy is rare. Meanwhile, the descriptive term of the concept as intensive care unit-acquired weakness (ICUAW) is preferred. The most significant risk factors for the development of ICUAW are sepsis, multiorgan dysfunction and acute respiratory distress syndrome (ARDS). In at least one third of patients, persistent impairment by paralysis, sensory disturbances and balance problems persist when they leave the ICU. At approximately 10%, these leg-accentuated and highly everyday relevant disorders persist over the first year after ICU therapy. Pure myopathy rarely leads to residual disturbances, while the neuropathic component is responsible for long-term impairments.
Topics: Critical Illness; Humans; Intensive Care Units; Muscular Diseases; Polyneuropathies; Sepsis
PubMed: 28875277
DOI: 10.1007/s00063-017-0339-0 -
Deutsches Arzteblatt International Feb 2018Polyneuropathies (peripheral neuropathies) are the most common type of disorder of the peripheral nervous system in adults, and specifically in the elderly, with an... (Review)
Review
BACKGROUND
Polyneuropathies (peripheral neuropathies) are the most common type of disorder of the peripheral nervous system in adults, and specifically in the elderly, with an estimated prevalence of 5-8%, depending on age. The options for treatment depend on the cause, which should therefore be identified as precisely as possible by an appropriate diagnostic evaluation.
METHODS
This review is based on the current guidelines and on large-scale cohort studies and randomized, controlled trials published from 2000 to 2017, with an emphasis on non-hereditary types of polyneuropathy, that were retrieved by a selective search in PubMed.
RESULTS
Diabetes is the most common cause of polyneuropathy in Europe and North America. Alcohol-associated polyneuropathy has a prevalence of 22-66% among persons with chronic alcoholism. Because of the increasing prevalence of malignant disease and the use of new chemotherapeutic drugs, chemotherapy-induced neuropathies (CIN) have gained in clinical importance; their prevalence is often stated to be 30-40%, with high variation depending on the drug(s) and treatment regimen used. Polyneuropathy can also arise from genetic causes or as a consequence of vitamin deficiency or overdose, exposure to toxic substances and drugs, and a variety of immunological processes. About half of all cases of polyneu - ropathy are associated with pain. Neuropathic pain can be treated symptomatically with medication. Exercise, physiotherapy, and ergotherapy can also be beneficial, depending on the patient's symptoms and functional deficits.
CONCLUSION
A timely diagnosis of the cause of polyneuropathy is a prerequisite for the initiation of appropriate specific treatment. Patients with severe neuropathy of unidentified cause should be referred to a specialized center for a thorough diagnostic evaluation.
Topics: Female; Humans; Male; Neuralgia; Polyneuropathies
PubMed: 29478436
DOI: 10.3238/arztebl.2018.083 -
Journal of Applied Physiology... May 2021Critical illness-associated weakness (CIAW) is an umbrella term used to describe a group of neuromuscular disorders caused by severe illness. It can be subdivided into...
Critical illness-associated weakness (CIAW) is an umbrella term used to describe a group of neuromuscular disorders caused by severe illness. It can be subdivided into three major classifications based on the component of the neuromuscular system (i.e. peripheral nerves or skeletal muscle or both) that are affected. This includes critical illness polyneuropathy (CIP), critical illness myopathy (CIM), and an overlap syndrome, critical illness polyneuromyopathy (CIPNM). It is a common complication observed in people with critical illness requiring intensive care unit (ICU) admission. Given CIAW is found in individuals experiencing grave illness, it can be challenging to study from a practical standpoint. However, over the past 2 decades, many insights into the pathophysiology of this condition have been made. Results from studies in both humans and animal models have found that a profound systemic inflammatory response and factors related to bioenergetic failure as well as microvascular, metabolic, and electrophysiological alterations underlie the development of CIAW. Current management strategies focus on early mobilization, achieving euglycemia, and nutritional optimization. Other interventions lack sufficient evidence, mainly due to a dearth of large trials. The goal of this Physiology in Medicine article is to highlight important aspects of the pathophysiology of these enigmatic conditions. It is hoped that improved understanding of the mechanisms underlying these disorders will lead to further study and new investigations for novel pharmacologic, nutritional, and exercise-based interventions to optimize patient outcomes.
Topics: Critical Care; Critical Illness; Humans; Intensive Care Units; Muscular Diseases; Neuromuscular Diseases; Polyneuropathies
PubMed: 33734888
DOI: 10.1152/japplphysiol.00019.2021 -
European Journal of Epidemiology Jan 2016Polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical distal numbness and paresthesia, often accompanied with pain and weakness.... (Review)
Review
Polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical distal numbness and paresthesia, often accompanied with pain and weakness. Although the disease is often encountered in neurological clinics and is well known by physicians, incidence and prevalence rates are not well known. We searched EMBASE, Medline, Web-of-science, Cochrane, PubMed Publisher, and Google Scholar, for population-based studies investigating the prevalence of polyneuropathy and its risk factors. Out of 5119 papers, we identified 29 eligible studies, consisting of 11 door-to-door survey studies, 7 case-control studies and 11 cohort/database studies. Prevalence of polyneuropathy across these studies varies substantially. This can partly be explained by differences in assessment protocols and study populations. The overall prevalence of polyneuropathy in the general population seems around 1% and rises to up to 7% in the elderly. Polyneuropathy seemed more common in Western countries than in developing countries and there are indications that females are more often affected than males. Risk factor profiles differ across countries. In developing countries communicable diseases, like leprosy, are more common causes of neuropathy, whereas in Western countries especially diabetes, alcohol overconsumption, cytostatic drugs and cardiovascular disease are more commonly associated with polyneuropathy. In all studies a substantial proportion of polyneuropathy cases (20-30%) remains idiopathic. Most of these studies have been performed over 15 years ago. More recent evidence suggests that the prevalence of polyneuropathy in the general population has increased over the years. Future research is necessary to confirm this increase in prevalence and to identify new and potentially modifiable risk factors.
Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Chronic Disease; Female; Humans; Incidence; Male; Polyneuropathies; Prevalence; Risk Factors; Sex Factors
PubMed: 26700499
DOI: 10.1007/s10654-015-0094-6 -
Journal of Neurology Jun 2021Amyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form... (Review)
Review
Amyloid transthyretin (ATTR) amyloidosis with polyneuropathy (PN) is a progressive, debilitating, systemic disease wherein transthyretin protein misfolds to form amyloid, which is deposited in the endoneurium. ATTR amyloidosis with PN is the most serious hereditary polyneuropathy of adult onset. It arises from a hereditary mutation in the TTR gene and may involve the heart as well as other organs. It is critical to identify and diagnose the disease earlier because treatments are available to help slow the progression of neuropathy. Early diagnosis is complicated, however, because presentation may vary and family history is not always known. Symptoms may be mistakenly attributed to other diseases such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), idiopathic axonal polyneuropathy, lumbar spinal stenosis, and, more rarely, diabetic neuropathy and AL amyloidosis. In endemic countries (e.g., Portugal, Japan, Sweden, Brazil), ATTR amyloidosis with PN should be suspected in any patient who has length-dependent small-fiber PN with autonomic dysfunction and a family history of ATTR amyloidosis, unexplained weight loss, heart rhythm disorders, vitreous opacities, or renal abnormalities. In nonendemic countries, the disease may present as idiopathic rapidly progressive sensory motor axonal neuropathy or atypical CIDP with any of the above symptoms or with bilateral carpal tunnel syndrome, gait disorders, or cardiac hypertrophy. Diagnosis should include DNA testing, biopsy, and amyloid typing. Patients should be followed up every 6-12 months, depending on the severity of the disease and response to therapy. This review outlines detailed recommendations to improve the diagnosis of ATTR amyloidosis with PN.
Topics: Adult; Amyloid Neuropathies, Familial; Brazil; Consensus; Humans; Japan; Polyneuropathies; Portugal; Prealbumin; Sweden
PubMed: 31907599
DOI: 10.1007/s00415-019-09688-0 -
Diabetes Research and Clinical Practice Apr 2022Diabetic sensorimotor polyneuropathy (DSPN) affects around one third of people with diabetes and accounts for considerable morbidity, increased risk of mortality,... (Review)
Review
Diabetic sensorimotor polyneuropathy (DSPN) affects around one third of people with diabetes and accounts for considerable morbidity, increased risk of mortality, reduced quality of life, and increased health care costs resulting particularly from neuropathic pain and foot ulcers. Painful DSPN is encountered in 13-26% of diabetes patients, while up to 50% of patients with DSPN may be asymptomatic. Unfortunately, DSPN still remains inadequately diagnosed and treated. Herein we provide international expert consensus recommendations and algorithms for screening, diagnosis, and treatment of DSPN in clinical practice derived from a Delphi process. Typical neuropathic symptoms include pain, paresthesias, and numbness particularly in the feet and calves. Clinical diagnosis of DSPN is based on neuropathic symptoms and signs (deficits). Management of DSPN includes three cornerstones: (1) lifestyle modification, optimal diabetes treatment aimed at near-normoglycemia, and multifactorial cardiovascular risk intervention, (2) pathogenetically oriented pharmacotherapy (e.g. α-lipoic acid and benfotiamine), and (3) symptomatic treatment of neuropathic pain including analgesic pharmacotherapy (antidepressants, anticonvulsants, opioids, capsaicin 8% patch and combinations, if required) and non-pharmacological options. Considering the individual risk profile, pain management should not only aim at pain relief, but also allow for improvement in quality of sleep, functionality, and general quality of life.
Topics: Consensus; Diabetes Mellitus; Diabetic Neuropathies; Humans; Neuralgia; Polyneuropathies; Quality of Life
PubMed: 34547367
DOI: 10.1016/j.diabres.2021.109063 -
F1000Research 2019Intensive care unit-acquired weakness (ICU-AW) is the most common neuromuscular impairment in critically ill patients. We discuss critical aspects of ICU-AW that have... (Review)
Review
Intensive care unit-acquired weakness (ICU-AW) is the most common neuromuscular impairment in critically ill patients. We discuss critical aspects of ICU-AW that have not been completely defined or that are still under discussion. Critical illness polyneuropathy, myopathy, and muscle atrophy contribute in various proportions to ICU-AW. Diagnosis of ICU-AW is clinical and is based on Medical Research Council sum score and handgrip dynamometry for limb weakness and recognition of a patient's ventilator dependency or difficult weaning from artificial ventilation for diaphragmatic weakness (DW). ICU-AW can be caused by a critical illness polyneuropathy, a critical illness myopathy, or muscle disuse atrophy, alone or in combination. Its diagnosis requires both clinical assessment of muscle strength and complete electrophysiological evaluation of peripheral nerves and muscles. The peroneal nerve test (PENT) is a quick simplified electrophysiological test with high sensitivity and good specificity that can be used instead of complete electrophysiological evaluation as a screening test in non-cooperative patients. DW, assessed by bilateral phrenic nerve magnetic stimulation or diaphragm ultrasound, can be an isolated event without concurrent limb muscle involvement. Therefore, it remains uncertain whether DW and limb weakness are different manifestations of the same syndrome or are two distinct entities. Delirium is often associated with ICU-AW but a clear correlation between these two entities requires further studies. Artificial nutrition may have an impact on ICU-AW, but no study has assessed the impact of nutrition on ICU-AW as the primary outcome. Early mobilization improves activity limitation at hospital discharge if it is started early in the ICU, but beneficial long-term effects are not established. Determinants of ICU-AW can be many and can interact with each other. Therefore, future studies assessing early mobilization should consider a holistic patient approach with consideration of all components that may lead to muscle weakness.
Topics: Critical Illness; Diaphragm; Hand Strength; Humans; Intensive Care Units; Muscle Weakness; Muscular Atrophy; Muscular Diseases; Polyneuropathies; Respiration, Artificial
PubMed: 31069055
DOI: 10.12688/f1000research.17376.1 -
Journal of Neurology Jan 2019POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder,... (Review)
Review
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.
Topics: Humans; POEMS Syndrome
PubMed: 30498913
DOI: 10.1007/s00415-018-9110-6 -
International Journal of Molecular... Apr 2023Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation... (Review)
Review
Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection. Various animal models have contributed and helped to elucidate the pathophysiological mechanisms in acute and chronic inflammatory polyradiculoneuropathies (Guillain-Barre Syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, respectively). The presence of specific anti-glycoconjugate antibodies indicates an underlying process of molecular mimicry and sometimes assists in the classification of these disorders, which often merely supports the clinical diagnosis. Now, the electrophysiological presence of conduction blocks is another important factor in characterizing another subgroup of treatable motor neuropathies (multifocal motor neuropathy with conduction block), which is distinct from Lewis-Sumner syndrome (multifocal acquired demyelinating sensory and motor neuropathy) in its response to treatment modalities as well as electrophysiological features. Furthermore, paraneoplastic neuropathies are also immune-mediated and are the result of an immune reaction to tumour cells that express onconeural antigens and mimic molecules expressed on the surface of neurons. The detection of specific paraneoplastic antibodies often assists the clinician in the investigation of an underlying, sometimes specific, malignancy. This review aims to discuss the immunological and pathophysiological mechanisms that are thought to be crucial in the aetiology of dysimmune neuropathies as well as their individual electrophysiological characteristics, their laboratory features and existing treatment options. Here, we aim to present a balance of discussion from these diverse angles that may be helpful in categorizing disease and establishing prognosis.
Topics: Animals; Guillain-Barre Syndrome; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyneuropathies; Autoantibodies; Inflammation; Neuritis
PubMed: 37108447
DOI: 10.3390/ijms24087288 -
JAMA Nov 2015Peripheral neuropathy is a highly prevalent and morbid condition affecting 2% to 7% of the population. Patients frequently experience pain and are at risk of falls,... (Review)
Review
IMPORTANCE
Peripheral neuropathy is a highly prevalent and morbid condition affecting 2% to 7% of the population. Patients frequently experience pain and are at risk of falls, ulcerations, and amputations. We aimed to review recent diagnostic and therapeutic advances in distal symmetric polyneuropathy, the most common subtype of peripheral neuropathy.
OBSERVATIONS
Current evidence supports limited routine laboratory testing in patients with distal symmetric polyneuropathy. Patients without a known cause should undergo a complete blood cell count, comprehensive metabolic panel, vitamin B12 measurement, serum protein electrophoresis with immunofixation, fasting glucose measurement, and glucose tolerance test. The presence of atypical features such as asymmetry, non-length dependence, motor predominance, acute or subacute onset, and prominent autonomic involvement should prompt a consultation with a neurologist or neuromuscular specialist. Electrodiagnostic tests and magnetic resonance imaging of the neuroaxis contribute substantial cost to the diagnostic evaluation, but evidence supporting their use is lacking. Strong evidence supports the use of tricyclic antidepressants, serotonin norepinephrine reuptake inhibitors, and voltage-gated calcium channel ligands in the treatment of neuropathic pain. More intensive glucose control substantially reduces the incidence of distal symmetric polyneuropathy in patients with type 1 diabetes but not in those with type 2 diabetes.
CONCLUSIONS AND RELEVANCE
The opportunity exists to improve guideline-concordant testing in patients with distal symmetric polyneuropathy. Moreover, the role of electrodiagnostic tests needs to be further defined, and interventions to reduce magnetic resonance imaging use in this population are needed. Even though several efficacious medications exist for neuropathic pain treatment, pain is still underrecognized and undertreated. New disease-modifying medications are needed to prevent and treat peripheral neuropathy, particularly in type 2 diabetes.
Topics: Antidepressive Agents; Calcium Channel Blockers; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Electrodiagnosis; Humans; Hypoglycemic Agents; Magnetic Resonance Imaging; Polyneuropathies; Practice Guidelines as Topic; Serotonin and Noradrenaline Reuptake Inhibitors
PubMed: 26599185
DOI: 10.1001/jama.2015.13611