-
Critical Care (London, England) 2008Critical illness polyneuropathy (CIP) and myopathy (CIM) are major complications of severe critical illness and its management. CIP/CIM prolongs weaning from mechanical... (Review)
Review
Critical illness polyneuropathy (CIP) and myopathy (CIM) are major complications of severe critical illness and its management. CIP/CIM prolongs weaning from mechanical ventilation and physical rehabilitation since both limb and respiratory muscles can be affected. Among many risk factors implicated, sepsis, systemic inflammatory response syndrome, and multiple organ failure appear to play a crucial role in CIP/CIM. This review focuses on epidemiology, diagnostic challenges, the current understanding of pathophysiology, risk factors, important clinical consequences, and potential interventions to reduce the incidence of CIP/CIM. CIP/CIM is associated with increased hospital and intensive care unit (ICU) stays and increased mortality rates. Recently, it was shown in a single centre that intensive insulin therapy significantly reduced the electrophysiological incidence of CIP/CIM and the need for prolonged mechanical ventilation in patients in a medical or surgical ICU for at least 1 week. The electrophysiological diagnosis was limited by the fact that muscle membrane inexcitability was not detected. These results have yet to be confirmed in a larger patient population. One of the main risks of this therapy is hypoglycemia. Also, conflicting evidence concerning the neuromuscular effects of corticosteroids exists. A systematic review of the available literature on the optimal approach for preventing CIP/CIM seems warranted.
Topics: Critical Illness; Humans; Intensive Care Units; Muscular Diseases; Polyneuropathies
PubMed: 19040777
DOI: 10.1186/cc7100 -
International Journal of Molecular... May 2023The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies,... (Review)
Review
The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome. The authors have undertaken a literature review of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain-Barre syndrome (GBS) and multifocal motor neuropathy (MMN). The molecular, electrophysiological and ultrasound features of these autoimmune polyneuropathies are analyzed, highlighting the differences in diagnosis and ultimately treatment. The immune dysfunction can lead to damage to the peripheral nervous system. In practice, it is suspected that these disorders are caused by autoimmunity to proteins located in the node of Ranvier or myelin components of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroups of treatment-naive motor neuropathies, including multifocal CIDP (synonyms: multifocal demyelinating neuropathy with persistent conduction block), which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features. Ultrasound is a reliable method for diagnosing immune-mediated neuropathies, particularly when alternative diagnostic examinations yield inconclusive results. In overall terms, the management of these disorders includes immunotherapy such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the development of more disease-specific immunotherapies should expand the therapeutic possibilities for these debilitating diseases.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Guillain-Barre Syndrome; Immunoglobulins, Intravenous; Polyneuropathies; Plasma Exchange
PubMed: 37298132
DOI: 10.3390/ijms24119180 -
American Journal of Transplantation :... Jun 2022Hereditary transthyretin-mediated (hATTR) amyloidosis, or ATTRv amyloidosis, is a progressive disease, for which liver transplantation (LT) has been a long-standing...
Hereditary transthyretin-mediated (hATTR) amyloidosis, or ATTRv amyloidosis, is a progressive disease, for which liver transplantation (LT) has been a long-standing treatment. However, disease progression continues post-LT. This Phase 3b, open-label trial evaluated efficacy and safety of patisiran in patients with ATTRv amyloidosis with polyneuropathy progression post-LT. Primary endpoint was median transthyretin (TTR) reduction from baseline. Twenty-three patients received patisiran for 12 months alongside immunosuppression regimens. Patisiran elicited a rapid, sustained TTR reduction (median reduction [Months 6 and 12 average], 91.0%; 95% CI: 86.1%-92.3%); improved neuropathy, quality of life, and autonomic symptoms from baseline to Month 12 (mean change [SEM], Neuropathy Impairment Score, -3.7 [2.7]; Norfolk Quality of Life-Diabetic Neuropathy questionnaire, -6.5 [4.9]; least-squares mean [SEM], Composite Autonomic Symptom Score-31, -5.0 [2.6]); and stabilized disability (Rasch-built Overall Disability Scale) and nutritional status (modified body mass index). Adverse events were mild or moderate; five patients experienced ≥1 serious adverse event. Most patients had normal liver function tests. One patient experienced transplant rejection consistent with inadequate immunosuppression, remained on patisiran, and completed the study. In conclusion, patisiran reduced serum TTR, was well tolerated, and improved or stabilized key disease impairment measures in patients with ATTRv amyloidosis with polyneuropathy progression post-LT (www.clinicaltrials.gov NCT03862807).
Topics: Amyloid Neuropathies, Familial; Humans; Liver Transplantation; Polyneuropathies; Prealbumin; Quality of Life; RNA, Small Interfering
PubMed: 35213769
DOI: 10.1111/ajt.17009 -
CMAJ : Canadian Medical Association... Oct 2016
Topics: Critical Illness; Electromyography; Humans; Muscle Weakness; Muscle, Skeletal; Neural Conduction; Polyneuropathies; Respiratory Muscles; Risk Factors; Severity of Illness Index; Ventilator Weaning
PubMed: 27378461
DOI: 10.1503/cmaj.151272 -
PloS One 2017Polyneuropathy is one of the most prevalent neurologic disorders. Although several studies explored the role of the neurological examination in polyneuropathy, they were... (Clinical Trial)
Clinical Trial
INTRODUCTION
Polyneuropathy is one of the most prevalent neurologic disorders. Although several studies explored the role of the neurological examination in polyneuropathy, they were mostly restricted to specific subgroups of patients and have not correlated examination findings with symptoms and electrophysiological results.
OBJECTIVES
To explore the sensitivity and specificity of different neurological examination components in patients with diverse etiologies for polyneuropathy, find the most sensitive combination of examination components for polyneuropathy detection, and correlate examination findings with symptoms and electrophysiological results.
METHODS
Patients with polyneuropathy attending the neuromuscular clinic from 01/2013 to 09/2015 were evaluated. Inclusion criteria included symptomatic polyneuropathy, which was confirmed by electrophysiological studies. 47 subjects with no symptoms or electrophysiological findings suggestive for polyneuropathy, served as controls.
RESULTS
The total cohort included 312 polyneuropathy patients, with a mean age of 60±14 years. Abnormal examination was found in 95%, most commonly sensory findings (86%). The most common abnormal examination components were impaired ankle reflexes (74%), vibration (73%), and pinprick (72%) sensation. Combining ankle reflex examination with vibration or pinprick perception had the highest sensitivity, of 88%. The specificities of individual examination component were generally high, excluding ankle reflexes (62%), and vibration perception (77%). Abnormal examination findings were correlated with symptomatic weakness and worse electrophysiological parameters.
CONCLUSION
The neurological examination is a valid, sensitive and specific tool for diagnosing polyneuropathy, and findings correlate with polyneuropathy severity. Ankle reflex examination combined with either vibration or pinprick sensory testing is the most sensitive combination for diagnosing polyneuropathy, and should be considered minimal essential components of the physical examination in patients with suspected polyneuropathy.
Topics: Aged; Electrophysiological Phenomena; Female; Humans; Male; Middle Aged; Polyneuropathies; Retrospective Studies; Sensitivity and Specificity
PubMed: 28249029
DOI: 10.1371/journal.pone.0171597 -
Revista Da Associacao Medica Brasileira... Mar 2019Peripheral neuropathy is a disorder that affects the cell body, axon or myelin of motor or peripheral sensory neurons and occurs in 60-100% of patients who are submitted... (Review)
Review
INTRODUCTION
Peripheral neuropathy is a disorder that affects the cell body, axon or myelin of motor or peripheral sensory neurons and occurs in 60-100% of patients who are submitted to dialysis due to chronic kidney disease. Uremic neuropathy (UN) is attributed to the accumulation of organic waste, evident in patients with reduced glomerular filtration rate.
OBJECTIVES
This review aims to make clinical characteristics of uremic neuropathy evident enabling early diagnosis and treatment.
METHODS
This is a literature review of articles published on PubMed over the last 10 years using "Uremic Neuropathy" as "Title/Abstract".
RESULTS
A total of nine articles that met the inclusion criteria were included. UN is a distal symmetric sensorimotor polyneuropathy that occurs due to the accumulation of uremic toxins associated with an oxidative stress-related free radical activity. Hyperkalemia is thought to play an important role in its pathophysiology. Diagnosis depends on nerve conduction studies, and treatment includes dialysis or renal transplant.
CONCLUSION
Clinical presentations of UN are broad and non-specific; nonetheless, it is important to detect early changes in order to avoid its progression. The earlier UN is diagnosed and treated, the more successful are the clinical outcomes.
Topics: Humans; Kidney Transplantation; Polyneuropathies; Renal Dialysis; Risk Factors; Uremia
PubMed: 30994849
DOI: 10.1590/1806-9282.65.3.469 -
Danish Medical Journal Oct 2012The objective was to search the literature with a view to providing a general description of critical illness myopathy/polyneuropathy (CIM/CIP), including its genesis... (Review)
Review
INTRODUCTION
The objective was to search the literature with a view to providing a general description of critical illness myopathy/polyneuropathy (CIM/CIP), including its genesis and prevention. Furthermore, it was our aim to determine whether new treatments have occurred in the past five years.
MATERIAL AND METHODS
PubMed, Cinahl and Swedmed+ were searched using the terms CIM, CIP and intensive care. The search was narrowed by adding the limits: humans, English, Danish, Norwegian, Swedish and, furthermore, articles had to have been published in the past five years as we aimed to focus on new knowledge.
RESULTS
A total of 74 articles were found. We excluded articles focusing on children and intensive care, tight insulin therapy in patients without CIM/CIP and articles focusing on Guillain-Barré syndrome, triage, bleeding, alcohol or meningitis. Of the remaining 36 articles, only five focused on CIM/CIP treatment. Their relevant original references were found and used too.
CONCLUSION
CIM/CIP is the most commonly occurring intensive care unit (ICU)-acquired neuromuscular dysfunction, and it is associated with a significant increase in length of stay, delayed weaning from mechanical ventilation, prolonged rehabilitation and, consequently, more expenses. To treat/prevent this condition, it seems reasonable to ensure maximal functional status for survivors of an ICU-stay by applying a multimodal therapeutic approach that includes intensive insulin therapy, minimal sedation and, as suggested by new evidence, early physiotherapy and electrical muscle stimulation.
Topics: Critical Care; Humans; Intensive Care Units; Muscular Diseases; Polyneuropathies
PubMed: 23158890
DOI: No ID Found -
Muscle & Nerve Dec 2009Neuromuscular disorders are common in human immunodeficiency virus (HIV); they occur at all stages of disease and affect all parts of the peripheral nervous system.... (Review)
Review
Neuromuscular disorders are common in human immunodeficiency virus (HIV); they occur at all stages of disease and affect all parts of the peripheral nervous system. These disorders have diverse etiologies including HIV itself, immune suppression and dysregulation, comorbid illnesses and infections, and side effects of medications. In this article, we review the following HIV-associated conditions: distal symmetric polyneuropathy; inflammatory demyelinating polyneuropathy; mononeuropathy; mononeuropathy multiplex; autonomic neuropathy; progressive polyradiculopathy due to cytomegalovirus; herpes zoster; myopathy; and other, rarer disorders.
Topics: HIV Infections; HIV-1; Humans; Polyneuropathies; Quality of Life
PubMed: 19771594
DOI: 10.1002/mus.21465 -
Scandinavian Journal of Pain Jan 2016Quantification of intraepidermal nerve fibre density (IENFD) is an important small fibre measure in distal symmetric polyneuropathies (DSP), but quantitative evaluation...
OBJECTIVES
Quantification of intraepidermal nerve fibre density (IENFD) is an important small fibre measure in distal symmetric polyneuropathies (DSP), but quantitative evaluation of additional structural and functional factors may help in elucidating the underlying mechanisms, and in improving the diagnostic accuracy in DSP. The literature reports a weak or moderate relationship between IENFD and spontaneous and evoked pain in neuropathies, but the relationship between functional and structural small fibre parameters in patients with DSP is unclear. The objectives of the current study, therefore, were to determine morphological and functional parameters related to small nerve fibres in subjects with distal symmetric polyneuropathy (DSP) and healthy controls, and to characterize the interplay among these parameters in these two groups.
MATERIALS AND METHODS
17 patients with painful DSP (≥4 on 0-10 numerical rating scale) and with symptoms and signs of small fibre abnormality (with or without large fibre involvement) and 19 healthy control subjects underwent comprehensive functional and structural small fibre assessments that included quantitative sensory testing, response to 30min topical application of 10% capsaicin and analysis of skin biopsy samples taken from the distal leg (IENFD, epidermal and dermal nerve fibre length densities (eNFLD, dNFLD) using global spatial sampling and axonal swelling ratios (swellings/IENFD and swellings/NFLD)).
RESULTS
DSP patients had reduced sensitivity to cold (median -11.07°C vs. -2.60, P≤0.001) and heat (median 46.7 vs. 37.70, P≤0.001), diminished neurovascular (median 184 vs. 278 mean flux on laser Doppler, P=0.0003) and pain response to topical capsaicin (median 10 vs. 35 on 0-100 VAS, P=0.0002), and lower IENFD, eNFLD and dNFLD values combined with increased swelling ratios (all P<0.001) compared to healthy controls. The correlation between structural and functional parameters was poor in DSP patients, compared with healthy controls. In healthy controls eNFLD and dNFLD, IENFD and eNFLD, IENFD and dNFLD all correlated well with each other (r=0.81; P≤0.001, r=0.58; P=0.009, r=0.60; P=0.007, respectively). In DSP, on the other hand, only eNFLD and dNFLD showed significant correlation (r=0.53, P=0.03). A diagnostic approach of combined IENFD and eNFLD utilization increased DSP diagnostic sensitivity from 82.0% to 100% and specificity from 84.0% to 89.5%.
CONCLUSIONS
This study presents a rigorous comparison between functional and morphological parameters, including parameters such as eNFDL and dNFLD that have not been previously evaluated in this context. The correlation pattern between functional and structural small fibre parameters is different in patients with DSP when compared to healthy controls. The findings suggest a more direct relationship between structure and function of nerve fibres in healthy controls compared to DSP. Furthermore, the findings suggest that combining IENFD with measurement of NFLD improves the diagnostic sensitivity and specificity of DSP.
IMPLICATIONS
Combining small fibre parameters may improve the diagnostic accuracy of DSP.
Topics: Case-Control Studies; Epidermis; Healthy Volunteers; Humans; Nerve Fibers; Peripheral Nervous System Diseases; Polyneuropathies
PubMed: 28361768
DOI: 10.1016/j.sjpain.2015.08.007 -
PloS One 2021Polyneuropathy is a debilitating condition characterized by distal sensory and motor deficits. Schwann cell dysfunction and axonal loss are integral factors in...
INTRODUCTION
Polyneuropathy is a debilitating condition characterized by distal sensory and motor deficits. Schwann cell dysfunction and axonal loss are integral factors in pathophysiology and disease progression of polyneuropathy.
AIMS
The aim of this study was the assessment of Schwann cell characteristics, nerve fibers and myelination parameters in polyneuropathy patients compared to controls.
METHODS
Nerve tissue was obtained from polyneuropathy patients (n = 10) undergoing diagnostic sural nerve biopsies. Biopsies of healthy peripheral nerves (n = 5) were harvested during elective sural nerve grafting for chronic peripheral nerve lesions. Exclusion criteria for the healthy control group were recent neurological trauma, diabetes, neurological and cardiovascular disease, as well as active malignancies and cytotoxic medication within the last 12 months. The over-all architecture of nerve sections and myelination parameters were histomorphometrically analyzed. Immunofluorescent imaging was used to evaluate Schwann cell phenotypes, senescence markers and myelination parameters.
RESULTS
Histomorphometric analysis of nerve biopsies showed significant axonal loss in polyneuropathy patients compared to controls, which was in accordance with the neuropathological findings. Immunofluorescent staining of Schwann cells and myelin basic protein indicated a significant impairment of myelination and lower Schwann cell counts compared to controls. Phenotypic alterations and increased numbers of non-myelinating p75-positive Schwann cells were found in polyneuropathy patients.
DISCUSSION
This study provided quantitative data of axonal loss, reduced myelination and Schwann cell dysfunction of polyneuropathy patients compared to neurologically healthy controls. Phenotypic alterations of Schwann cells were similar to those seen after peripheral nerve injury, highlighting the clinical relevance of Schwann cell dysfunction.
Topics: Adult; Axons; Female; Fluorescent Antibody Technique; Humans; Male; Middle Aged; Nerve Fibers, Myelinated; Polyneuropathies; Schwann Cells
PubMed: 34735549
DOI: 10.1371/journal.pone.0259654