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Journal of Neurophysiology Jan 2023Neurological manifestations associated with Coronavirus Disease-2019 (COVID-19) are commonly reported, but patients were not referred to perform the electrophysiological... (Review)
Review
Neurological manifestations associated with Coronavirus Disease-2019 (COVID-19) are commonly reported, but patients were not referred to perform the electrophysiological assessment. We aimed to review the existing literature on clinical studies on COVID-19 peripheral neuropathy to correlate patients' symptoms and characteristics with nerve conduction studies/electromyography (NCS/EMG) outcomes. This protocol is registered in the Open Science Framework (https://www.doi.org/10.17605/OSF.IO/ZF4PK). The systematic search included PubMed, ScienceDirect, and Google Scholar, for articles published from December 2019 to March 2022. A total of 727 articles were collected, and according to our inclusion and exclusion criteria, only 6 articles were included. Of 195 participants, only 175 underwent NCS/EMG assessment. Of these, 44 participants (25.1%) had abnormal EMG, 54 participants (30.8%) had abnormal motor NCS, and only 7 participants (4%) had abnormal sensory NCS. All cases presented with myopathy, while a limited number of cases presented with polyneuropathy. According to motor NCS and EMG, the most affected nerves were the tibial and peroneal in the lower extremities and the ulnar nerve in the upper extremities. Interestingly, the median nerve was reported to be associated with the severity and the rate of motor recovery of patients with COVID-19. COVID-19 generates a demyelinating motor neuropathy and myopathy. Clinicians are encouraged to refer patients with COVID-19 presenting with neurological symptoms to be assessed by electrophysiological methods to objectively determine the nature of their symptoms, follow their prognosis, and plan their rehabilitation.
Topics: Humans; Peripheral Nervous System Diseases; Neural Conduction; COVID-19; Polyneuropathies; Electromyography; Muscular Diseases
PubMed: 36475865
DOI: 10.1152/jn.00386.2022 -
Neurological Sciences : Official... Mar 2023Hereditary spastic paraplegia (HSP) is a rare genetic disorder associated with mutations in > 80 loci designated SPG (SPastic parapleGia). The phenotypic spectrum of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Hereditary spastic paraplegia (HSP) is a rare genetic disorder associated with mutations in > 80 loci designated SPG (SPastic parapleGia). The phenotypic spectrum of HSP can extend to include other neurologic features, including movement disorders. Our aim was to investigate genotype-phenotype associations in HSP with a focus on movement disorders.
METHODS
We performed a systematic review and individual participant data (IPD)-level meta-analysis by retrieving publications from Medline/EMBASE/Web of Science on HSP with a SPG genotype. Studies were included only if individual-level information was accessible and at least one patient with a movement disorder was reported for that genotype. Out of 21,957 hits, 192 manuscripts with a total of 1413 HSP cases were eligible. Data were compared between two HSP groups: manifested with (HSP-MD, n = 767) or without (HSP-nMD, n = 646) a movement disorder.
RESULTS
The HSP-MD group had an older age of onset (20.5 ± 16.0 vs. 17.1 ± 14.2 yr, p < 0.001) and less frequent autosomal dominant inheritance (7.6% vs. 30.1%, p < 0.001) compared to HSP-nMD. SPG7 (31.2%) and SPG11 (23.8%) were the most frequent genotypes in the HSP-MD group. HSP-MD with SPG7 had higher frequency of later onset during adulthood (82.9% vs. 8.5%), ataxia (OR = 12.6), extraocular movement disturbances (OR = 3.4) and seizure (OR = 3.7) compared to HSP-MD with SPG11. Conversely, SPG11 mutations were more frequently associated with consanguinity (OR = 4.1), parkinsonism (OR = 7.8), dystonia (OR = 5.4), peripheral neuropathy (OR = 26.9), and cognitive dysfunction (OR = 34.5).
CONCLUSION
This systematic IPD-level meta-analysis provides the largest data on genotype-phenotype associations in HSP-MD. Several clinically relevant phenotypic differences were found between various genotypes, which can possibly facilitate diagnosis in resource-limited settings.
Topics: Humans; Spastic Paraplegia, Hereditary; Paraplegia; Mutation; Movement Disorders; Phenotype; Proteins
PubMed: 36441344
DOI: 10.1007/s10072-022-06516-8 -
International Journal of Molecular... Nov 2022Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory... (Review)
Review
Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include , , and cytomegalovirus. is responsible for about a third of GBS cases. GBS due to is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.
Topics: Humans; Amantadine; Autoantibodies; Axons; Campylobacter jejuni; Guillain-Barre Syndrome
PubMed: 36430700
DOI: 10.3390/ijms232214222 -
Mediators of Inflammation 2022In this study, we conducted a systematic review and meta-analysis regarding the role of the neutrophil to lymphocyte ratio (NLR) in Guillain Barré syndrome (GBS). The... (Meta-Analysis)
Meta-Analysis Review
In this study, we conducted a systematic review and meta-analysis regarding the role of the neutrophil to lymphocyte ratio (NLR) in Guillain Barré syndrome (GBS). The most recent update to the search was on July 18, 2022, through the databases of Web of Science, PubMed, Embase, and Scopus. The Newcastle-Ottawa scale was used for quality assessment of included studies. Finally, 14 studies were included in the review, and among them, ten studies were included in the meta-analysis. Our results showed that NLR levels were significantly increased in the patients with GBS compared with healthy controls (SMD = 1.05; 95%CI = 0.59 to 1.50, < 0.001). After treatment, NLR levels were decreased to the extent that they became similar to healthy controls (SMD = -0.03, 95%CI = -0.29 to 0.22, = 0.204). Moreover, NLR was a stable predictor of outcome or response to treatment in such patients (SMD = 1.01, 95%CI = 0.65 to 1.37, < 0.001); the higher the NLR, the worse the outcome. In addition, patients who underwent mechanical ventilation had higher levels of NLR compared to those who did not (SMD = 0.93, 95%CI = 0.05 to 1.82, = 0.03). However, NLR levels were not different among distinct GBS subtypes, so it could not distinguish among them. In conclusion, our analysis indicates that the NLR levels are highly elevated in patients with GBS. Therefore, the NLR has the potential to be used as a biomarker to inform diagnosis, prognosis, or treatment responses in GBS, and future studies are warranted.
Topics: Biomarkers; Guillain-Barre Syndrome; Humans; Lymphocytes; Neutrophils; Prognosis
PubMed: 36133742
DOI: 10.1155/2022/3390831 -
Immunologic Research Dec 2022Since the beginning of worldwide vaccination against coronavirus disease 2019 (COVID-19), studies have reported a possible association between vaccination and... (Review)
Review
Since the beginning of worldwide vaccination against coronavirus disease 2019 (COVID-19), studies have reported a possible association between vaccination and Guillain-Barré syndrome (GBS). In this regard, we conducted a systematic review assessing different demographic, clinical, and neurophysiological aspects of patients with GBS following immunization with COVID-19 vaccines. A comprehensive search of PubMed, Web of Science, Scopus, and Google Scholar was performed. Articles in English between January 2020 and November 2021 were included. Data on demographics, clinical characteristics, vaccines information, treatment approaches, and outcomes were extracted. The data of a total of 88 patients out of 41 studies was included. The mean age of patients was 58.7 ± 16.6 years and 55 cases (62.5%) were male. AstraZeneca was the most-reported vaccine associated with GBS with 52 cases (59.1%) followed by Pfizer with 20 cases (22.7%). GBS occurred after the first dose of vaccination in 70 cases (79.5%). The mean time interval between vaccination and symptom onset was 13.9 ± 7.4 days. Limb weakness (47.7%), sensory disturbance (38.6%), and facial weakness (27.3%) were the most common reported symptoms, respectively. Albuminocytologic dissociation was seen in 65% of patients who underwent lumbar puncture (n = 65). Acute inflammatory demyelinating polyradiculopathy was the most common GBS subtype, which was reported in 38 patients (43.2%). While one-fifth of patients underwent intubation (n = 17), a favorable outcome was achieved in the majority of subjects (n = 46, 63%). Overall, a small rise in GBS incidence, following various COVID-19 vaccines, was observed. Notably, 85% of affected individuals experienced at least a partial recovery.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; COVID-19; COVID-19 Vaccines; Guillain-Barre Syndrome; Vaccination; Vaccines
PubMed: 36098903
DOI: 10.1007/s12026-022-09316-6 -
European Journal of Neurology Jan 2023Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND PURPOSE
Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement. pSS has been linked to various neurological manifestations, including peripheral neuropathy (PN). We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related PN.
METHODS
A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis.
RESULTS
The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%-20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies-particularly trigeminal-are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin.
CONCLUSIONS
PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration.
Topics: Female; Humans; Middle Aged; Male; Sjogren's Syndrome; Peripheral Nervous System Diseases; Vasculitis; Immunoglobulins, Intravenous
PubMed: 36086910
DOI: 10.1111/ene.15555 -
Pain and Therapy Dec 2022Immunoglobulins (IG) are widely used for the treatment of a variety of immune-mediated diseases. The exact mechanism of action remains unknown, but IG modulate the...
BACKGROUND
Immunoglobulins (IG) are widely used for the treatment of a variety of immune-mediated diseases. The exact mechanism of action remains unknown, but IG modulate the expression and function of Fc receptors, interfere with complement activation and production of cytokines, neutralize pathogenic autoantibodies, and affect the activation and effector functions of B and T lymphocytes. Immunoglobulins are usually delivered intravenously, and are effective in ameliorating motor symptoms, and/or preventing disease progression in immune-mediated neuropathies, including Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.
OBJECTIVE
The aim of this systematic review and meta-analysis was to study the potential of IG for the treatment of painful peripheral neuropathy (PPN). The outcome of interest was the percentage of patients with PPN who achieved pain relief following IG administration.
METHODS
We performed a systematic literature search on March 17, 2022, in the PubMed database without any publication date restrictions. We also looked for unpublished or ongoing trials in clinicaltrials.org. Pain reduction following IG treatment had to be within the aims (primary or secondary).
RESULTS
The aforementioned literature search strategy revealed five studies (two open-label, three randomized placebo-controlled) eligible to be included. The pooled estimate of the percentage of patients with PPN who received immunoglobulins and reported pain relief was found to be 65% (95% CI 58-71%). The likelihood of achieving pain relief with immunoglobulin treatment was 2.9 times higher (95% CI 1.6-5.2) compared to placebo (p = 0.0003).
CONCLUSION
The use of IG for the treatment of pain due to peripheral neuropathy has a potential therapeutic benefit. Further studies across patients with different types of painful peripheral neuropathy are needed to better characterize this effect. Registration number on PROSPERO: CRD42022319614.
PubMed: 35925489
DOI: 10.1007/s40122-022-00416-7 -
Physical Therapy Oct 2022The purpose of this study was to critically appraise and summarize the evidence for reliability of muscle strength and muscle power assessment in patients with...
OBJECTIVE
The purpose of this study was to critically appraise and summarize the evidence for reliability of muscle strength and muscle power assessment in patients with neuromuscular diseases (NMDs) using isokinetic dynamometry.
METHODS
PubMed, CINAHL, and Embase electronic databases were searched from inception to March 8, 2022. Studies designed to evaluate reliability of muscle strength and power measurements using isokinetic dynamometry were included in this review. First, the methodological quality of the studies was assessed according to the Consensus-Based Standards for the Selection of Health Measurement Instruments guidelines. Next, the quality of measurement properties was determined. Finally, the methodological quality and quality of measurement properties of the studies were combined to obtain a best-evidence synthesis.
RESULTS
A best-evidence synthesis of reliability was performed in 11 studies including postpoliomyelitis syndrome (n = 5), hereditary motor and sensory neuropathy (n = 2), motor neuron diseases (n = 1), myotonic dystrophy (n = 1), and groups of pooled NMDs (n = 2). A best-evidence synthesis on measurement error could not be performed. Quality of evidence on reliability ranged from high in postpoliomyelitis syndrome to very low in hereditary motor and sensory neuropathy, motor neuron diseases, and groups of pooled NMDs. The most frequently used outcome measure was peak torque, which was reliable in all populations (intraclass correlation coefficient >0.7).
CONCLUSION
The quality of evidence for reliability of isokinetic dynamometry was found to vary substantially among different NMDs. High quality of evidence has been obtained only in patients with postpoliomyelitis syndrome. Further research is needed in the majority of known NMDs to determine reliability and validity of isokinetic dynamometry.
IMPACT
The ability of isokinetic dynamometers to capture clinically relevant changes in muscle strength and muscle power in NMDs remains unclear. Isokinetic dynamometry results in NMDs should be interpreted with caution.
Topics: Humans; Muscle Strength Dynamometer; Reproducibility of Results; Postpoliomyelitis Syndrome; Muscle Strength; Neuromuscular Diseases; Hereditary Sensory and Motor Neuropathy; Muscles; Muscle, Skeletal
PubMed: 35899532
DOI: 10.1093/ptj/pzac099 -
Frontiers in Pharmacology 2022Diabetes mellitus (DM) is one of the leading causes of morbidity and mortality worldwide. DM patients with diabetic neuropathy (DN) usually present with distal pain,...
Diabetes mellitus (DM) is one of the leading causes of morbidity and mortality worldwide. DM patients with diabetic neuropathy (DN) usually present with distal pain, sensorimotor polyneuropathy, postural hypotension, or erectile dysfunction. They also may present with other nerve pathologies such as inflammatory neuropathies and carpal tunnel syndrome. We conducted a systematic review and meta-analysis to assess the benefits of using sodium-glucose co-transporter-2 inhibitors (SGLT2Is) to manage DN. An extensive systematic literature review was conducted to include all articles published up to 24 February 2022. All clinical studies included patients with DM and reported the outcomes of SGLT2I on diabetes-associated neuropathy. Six studies were identified for meta-analysis, including a total of 5312 diabetic patients. The average age of the included patients ranged from 41 to 74 years and 34-73 years in the SGLT2I treatment and control groups, respectively. SGLT2I moderately improved the manifestations of diabetic peripheral neuropathy events and nerve conduction velocity. Furthermore, the SGLT2I treatment group had a statistically significant higher mean heart-to-mediastinum ratio (MD 0.41; 95% 0.17, 0.64; = 0.0006). However, the mean heart rates (MD -4.51; 95% -10.05, 1.04; = 0.11) and wash out rates (MD 2.13; 95% -8.48, 12.75; = 0.69) were not significantly different between the two groups. SGLT2Is could therefore be considered neuroprotective in patients with DN, possibly by considerably increasing the sensory and motor nerve conduction velocity, improving the clinical manifestations of DPN, and reducing sympathetic nervous system activity. http://www.crd.york.ac.uk/prospero/, identifier CRD42022312828.
PubMed: 35899123
DOI: 10.3389/fphar.2022.926717 -
Diagnostics (Basel, Switzerland) Jul 2022Chronic Inflammatory Demyelinating Polyneuropathy is an immune-mediated pathology of the peripheral nerves and nerve roots that leads to weakness and sensory symptoms.... (Review)
Review
Chronic Inflammatory Demyelinating Polyneuropathy is an immune-mediated pathology of the peripheral nerves and nerve roots that leads to weakness and sensory symptoms. Given its clinical heterogeneity, often times diagnosis is challenging. Even though nerve conduction studies and clinical features are the main criteria used for diagnosis, supplementary investigations, such as nerve biopsies, cerebral spinal fluid examination and magnetic resonance studies, may be used in order to confirm the diagnosis. Given the fact that the hallmark in CIDP physiopathology is the demyelination process, nerve biopsies are used to demonstrate and assess the magnitude of the phenomenon. The question and the main interest of this review is whether histopathological findings are relevant for the diagnosis and can be useful in disease assessment.
PubMed: 35885595
DOI: 10.3390/diagnostics12071691