scholarly journals γ' Fibrinogen as a Predictor of Survival in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 8 ◽  
Author(s):  
Ana Catarina Pronto-Laborinho ◽  
Catarina S. Lopes ◽  
Vasco A. Conceição ◽  
Marta Gromicho ◽  
Nuno C. Santos ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rating Scale ◽  
Neurodegenerative Disorder ◽  
Positive Impact ◽  
Enzyme Linked Immunosorbent Assay ◽  
Fibrinogen Concentration ◽  
Respiratory Impairment ◽  
Increased Risk ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is an aggressive neurodegenerative disorder related to neuroinflammation that is associated with increased risk of thrombosis. We aimed to evaluate γ' fibrinogen plasma level (an in vivo variant of fibrinogen) as a biomarker in ALS, and to test its role as a predictor of disease progression and survival. Sixty-seven consecutive patients with ALS were followed and the results were compared with those from 82 healthy blood donors. Patients were clinically evaluated at the time of blood sampling and on follow-up (every 3 months for the beginning of the follow-up until death) by applying the revised ALS Functional Rating Scale. Human plasma γ' fibrinogen concentration was quantified using a specific two-site sandwich kit enzyme-linked immunosorbent assay. We found, for the first time, a positive association between γ' fibrinogen concentration and survival in ALS patients: patients with higher γ' fibrinogen plasma levels survived longer, and this finding was not influenced by confounders such as age, gender, respiratory impairment, or functionality (ALSFRS-R score). Since increased levels have a positive impact on outcome, this novel biomarker should be further investigated in ALS.

scholarly journals Utility of phrenic nerve ultrasound in amyotrophic lateral sclerosis

2020 ◽  
Author(s):  
Cezar Thomas Suratos ◽  
Naoko Takamatsu ◽  
Hiroki Yamazaki ◽  
Yusuke Osaki ◽  
Tatsuya Fukumoto ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Phrenic Nerve ◽  
Rating Scale ◽  
Neurodegenerative Disorder ◽  
Pulmonary Function Tests ◽  
Cross Sectional ◽  
The Right ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting the upper and lower motor neurons causing progressive weakness. It eventually involves the diaphragm which leads to respiratory paralysis and subsequently death. Phrenic nerve (PN) conduction studies and diaphragm ultrasound has been studied and correlated with pulmonary function tests in ALS patients. However, PN ultrasonography has not been employed in ALS. This study aims to sonographically evaluate the morphologic appearance of the PN of ALS patients. Thirty-eight ALS patients and 28 normal controls referred to the neurophysiology laboratory of two institutions were retrospectively included in the study. Baseline demographic and clinical variables such as disease duration, ALS Functional Rating Scale-Revised score, and ALS region of onset were collected. Ultrasound was used to evaluate the PN cross-sectional area (CSA) of ALS and control subjects. The mean PN CSA of ALS patients were 1.08 ± 0.39 mm on the right and 1.02 ± 0.34 mm on the left. The PN CSA of ALS patients were significantly decreased compared to controls (p value < 0.00001). The PN CSA of ALS patients was not correlated to any of the demographic and clinical parameters tested. This study demonstrates that ALS patients have a smaller PN size compared to controls using ultrasonography.

Risk factors for cognitive impairment in amyotrophic lateral sclerosis: a systematic review and meta-analysis

2021 ◽  
pp. jnnp-2020-325701
Author(s):  
Tianmi Yang ◽  
Yanbing Hou ◽  
Chunyu Li ◽  
Bei Cao ◽  
Yangfan Cheng ◽  
...  
Keyword(s):  
Risk Factors ◽  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Family History ◽  
Rating Scale ◽  
Or Education ◽  
Increased Risk ◽  
Bulbar Onset ◽  
History Of ◽  
Lateral Sclerosis

ObjectiveCognitive impairment is a common, far-reaching but imperceptible manifestation in patients with amyotrophic lateral sclerosis (ALS). We aimed to identify the risk factors for cognitive impairment in ALS.MethodsWe searched PubMed and EMBASE for cross-sectional, case–control and cohort studies that reported predictors of cognitive impairment in ALS. The obtained data were meta-analysed to generate overall ORs and 95% CIs.ResultsTwenty-seven eligible articles reporting on 6799 individuals were included out of 20 501 records. Nine predictors were identified: C9orf72 (OR 3.62, 95% CI 1.76 to 7.45), dysarthria (OR 2.25, 95% CI 1.20 to 4.22), family history of ALS (OR 1.76, 95% CI 1.18 to 2.61), predominant upper motor neuron (PUMN) phenotype (OR 1.73, 95% CI 1.09 to 2.73) and bulbar onset (OR 1.54, 95% CI 1.28 to 1.87) increased risk factors for cognitive impairment in ALS. ALS Functional Rating Scale-Revised scores, sex, age or education level were not significantly associated with cognitive impairment in ALS. In addition, C9orf72 (OR=5.94) and bulbar onset (OR=2.08) were strong predictors of ALS-frontotemporal dementia. Female sex conferred more susceptibility to executive cognitive impairment than male sex (OR=1.82).ConclusionsPatients with C9orf72 repeat expansion, dysarthria, family history of ALS, PUMN phenotype and bulbar onset had a high risk for cognitive impairment in ALS. These associations may contribute to understanding the heterogeneity of ALS.PROSPERO registration numberCRD42020201085.

scholarly journals Transplantation of Mesenchymal Stromal Cells in Patients with Amyotrophic Lateral Sclerosis: Results of Phase I/IIa Clinical Trial

2017 ◽  
Vol 26 (4) ◽  
pp. 647-658 ◽  
Author(s):  
Eva Syková ◽  
Petr Rychmach ◽  
Ivana Drahorádová ◽  
ŠImona Konrádová ◽  
Kateřina Růžičková ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Amyotrophic Lateral Sclerosis ◽  
Phase I ◽  
Lumbar Puncture ◽  
Stromal Cells ◽  
Adverse Reactions ◽  
Rating Scale ◽  
Neurodegenerative Disorder ◽  
Safe Procedure ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive untreatable neurodegenerative disorder, leading to the death of the cortical and spinal motoneurons (MNs). Bone marrow-derived mesenchymal stem/stromal cells (BM-MSCs) may represent a new approach to slowing down the progression of ALS by providing neurotrophic support to host MNs and by having an anti-inflammatory effect. We have designed a prospective, nonrandomized, open-label clinical trial (phase I/IIa, EudraCT No. 2011-000362-35) to assess the safety and efficacy of autologous multipotent BM-MSCs in ALS treatment. Autologous BM-MSCs were isolated and expanded under GMP conditions. Patients received 15 ± 4.5 × 106 of BM-MSCs via lumbar puncture into the cerebrospinal fluid. Patients were monitored for 6 months before treatment and then for an 18-month follow-up period. Potential adverse reactions were assessed, and the clinical outcome was evaluated by the ALS functional rating scale (ALSFRS), forced vital capacity (FVC), and weakness scales (WSs) to assess muscle strength on the lower and upper extremities. In total, 26 patients were enrolled in the study and were assessed for safety; 23 patients were suitable for efficacy evaluation. After intrathecal BM-MSC application, about 30% of the patients experienced a mild to moderate headache, resembling the headaches after a standard lumbar puncture. No suspected serious adverse reactions (SUSAR) were observed. We found a reduction in ALSFRS decline at 3 months after application ( p < 0.02) that, in some cases, persisted for 6 months ( p < 0.05). In about 80% of the patients, FVC values remained stable or above 70% for a time period of 9 months. Values of WS were stable in 75% of patients at 3 months after application. Our results demonstrate that the intrathecal application of BM-MSCs in ALS patients is a safe procedure and that it can slow down progression of the disease.

scholarly journals Changes in Retinal OCT and Their Correlations with Neurological Disability in Early ALS Patients, a Follow-Up Study

2019 ◽  
Vol 9 (12) ◽  
pp. 337 ◽  
Author(s):  
Pilar Rojas ◽  
Rosa de Hoz ◽  
Ana I. Ramírez ◽  
Antonio Ferreras ◽  
Elena Salobrar-Garcia ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rating Scale ◽  
P Value ◽  
Ophthalmological Examination ◽  
Fiber Layer ◽  
Neurological Disability ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Visual Changes

Background: To compare early visual changes in amyotrophic lateral sclerosis (ALS) patients with healthy controls in a baseline exploration, to follow-up the patients after 6 months, and to correlate these visual changes with neurological disability. Methods: All patients underwent a comprehensive neurological and ophthalmological examination. A linear mixed analysis and Bonferroni p-value correction were performed, testing four comparisons as follows: Control baseline vs. control follow-up, control baseline vs. ALS baseline, control follow-up vs. ALS follow-up, and ALS baseline vs. ALS follow-up. Results: The mean time from the diagnosis was 10.80 ± 5.5 months. The analysis of the optical coherence tomography (OCT) showed: (1) In ALS baseline vs. control baseline, a macular significantly increased thickness of the inner macular ring temporal and inferior areas; (2) in ALS follow-up vs. ALS baseline, a significant macular thinning in the inner and outer macular ring inferior areas; (3) in ALS follow-up vs. ALS baseline, a significant peripapillary retinal nerve fiber layer (pRNFL) thinning in the superior and inferior quadrants; and (4) ALS patients showed a moderate correlation between some OCT pRNFL parameters and Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score. Conclusion: The OCT showed retinal changes in patients with motoneuron disease and could serve as a complementary tool for studying ALS.

scholarly journals Botulinum toxin for the treatment of lower limb cramp pain in patients with Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Tejas Mehta ◽  
Richard Sommers ◽  
Raghav Govindarajan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Pain Score ◽  
Lower Limb ◽  
Rating Scale ◽  
Retrospective Chart Review ◽  
Pain Scale ◽  
Wilcoxon Test ◽  
Safe Procedure ◽  
Lateral Sclerosis

Background: Muscle cramps and pain associated with them can be seen in patients with amyotrophic lateral sclerosis (ALS) and are known to reduce the quality of life. Pharmacological treatment may not benefit all patients in treating these cramps. We assess the efficacy of Onabotulinum toxin A (BTX-A) in the treatment of lower limb cramps in patients with ALS. Methods: This retrospective chart review included a total of ten patients with ALS who suffered from pain due to lower limb cramps and were managed with BTX-A. Data including patient demographics, visual analog pain scale at different intervals during follow up, ALS functional rating scale and site of onset of ALS symptoms were documented. The pain score at baseline (before administration), at 3 months follow up and at 6 months follow up were compared using Wilcoxon test to assess BTX-A’s efficacy. Results: A significant improvement in average pain score due to cramps from baseline to the 6-month interval with a change of 3.1±0.7 (p<0.05,95%CI) was seen on the pain scale. No adverse events were noted during administration or post injections. Conclusion: Local BTX-A administration is an efficacious and safe procedure for improving pain associated with cramps in patients with ALS.

scholarly journals Amyotrophic lateral sclerosis: prospective study on respiratory parameters

2010 ◽  
Vol 68 (2) ◽  
pp. 258-262 ◽  
Author(s):  
Sara Regina Meira Almeida ◽  
Luciano Bruno C. Silva ◽  
Carlos Alberto M. Guerreiro ◽  
Anamarli Nucci
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Pulse Oximetry ◽  
Respiratory Impairment ◽  
Disease Evolution ◽  
Significant Parameter ◽  
Respiratory Parameters ◽  
Percentage Weight ◽  
Maximum Expiratory Pressure ◽  
Lateral Sclerosis

OBJECTIVE: To verify how efficient respiratory parameters are in the follow-up of subjects with amyotrophic lateral sclerosis (ALS) and to observe possible correlations between respiratory and nutritional functions. METHOD: Sixteen patients with probable or defined ALS were selected and evaluated over eight months using the following respiratory parameters: spirometry, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), arterial gasometry and pulse oximetry; and nutritional parameters such as body mass index (BMI) and percentage weight loss. RESULTS: PaCO2 was a significant parameter to follow up disease evolution (p=0.051). There was significant correlation between MIP and MEP (r: 0.83); BMI and MIP (r: 0.70); BMI and MEP (r: 0.72); pulse oximetry and forced vital capacity (r: 0.57). CONCLUSION: PaCO2 was shown to be an efficient and significant parameter in the measurement of respiratory impairment; the correlations among MIP, MEP and BMI indicated that these are significant parameters for periodic clinical evaluation.

scholarly journals Risk of amyotrophic lateral sclerosis and other motor neuron disease among men with benign prostatic hyperplasia: a population-based cohort study

BMJ Open ◽  
2019 ◽  
Vol 9 (7) ◽  
pp. e030015 ◽  
Author(s):  
Trine Toft Sørensen ◽  
Erzsébet Horváth-Puhó ◽  
Mette Nørgaard ◽  
Vera Ehrenstein ◽  
Victor W Henderson
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cohort Study ◽  
Benign Prostatic Hyperplasia ◽  
Primary Outcome ◽  
Population Based ◽  
Prostatic Hyperplasia ◽  
Increased Risk ◽  
Comparison Cohort ◽  
Lateral Sclerosis

ObjectivesAmyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder. Sleep disturbance may interfere with clearance of abnormal proteins that aggregate in neurodegenerative diseases. The objective of this study was to examine the association between benign prostatic hyperplasia (BPH), a common disorder causing nocturia and sleep disturbance, and risk of ALS and other motor neuron disease (MND). We hypothesised that men with BPH, in comparison to men in the general population, would be at increased risk.DesignThis is a nationwide, population-based cohort study.SettingThis study was conducted among the population of Denmark.ParticipantsWe used linked Danish medical databases to identify all men with a first-time diagnosis of BPH between 1 January 1980 and 30 November 2013 and no prior diagnosis of MND (BPH cohort, n=223 131) and an age-matched general population comparison cohort of men without BPH or MND (n=1 115 642).Primary outcome measureThe primary outcome is diagnosis of MND after the BPH diagnosis (index) date, with follow-up until MND diagnosis, emigration, death or 30 November 2013.ResultsWe used Cox regression to compute adjusted HR, comparing men with and without BPH. After 34 years of follow-up, there were 227 cases of MND in the BPH cohort (incidence rate 0.13/1000 person-years) and 1094 MND cases in the comparison cohort (0.12/1000 person-years; HR 1.05, 95% CI 0.90 to 1.22). Risk did not vary by follow-up time.ConclusionsBPH is not associated with an increased risk of ALS and other MND. Future studies should examine the relation between other disorders that disrupt sleep and MND risk in men and women.

Neurofilament Subunit L Levels in the Cerebrospinal Fluid and Serum of Patients with Amyotrophic Lateral Sclerosis

2018 ◽  
Vol 18 (2-3) ◽  
pp. 165-172 ◽  
Author(s):  
Zhong-Ying Gong ◽  
Gao-Peng Lv ◽  
Li-Na Gao ◽  
Yi Lu ◽  
Jie Guo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cerebrospinal Fluid ◽  
Disease Progression ◽  
Rating Scale ◽  
Enzyme Linked Immunosorbent Assay ◽  
Progression Rate ◽  
Serum Samples ◽  
High Level ◽  
Als Patients ◽  
Lateral Sclerosis

Background: There are no reliable biomarkers that could evaluate the disease burden in amyotrophic lateral sclerosis (ALS). Objectives: The aim of our study is to evaluate the changes in cerebrospinal fluid (CSF) and serum neurofilament subunit L (NF-L) in patients with ALS and to analyze the correlations between the levels of NF-L and clinical parameters. Method: CSF and serum samples were obtained from 80 ALS patients and 40 controls. The levels of NF-L in CSF and serum were assessed, and disease progression parameters including duration, revised ALS Functional Rating Scale (ALSFRS-r) score, disease progression rate (DPR), upper motor neuron (UMN) score, and survival were analyzed by registered neurologists. All samples were measured using a commercial enzyme-linked immunosorbent assay. Statistical analyses were performed using Prism software. Results: Compared to the controls, the ALS patients displayed significantly increased levels of NF-L; these values were negatively correlated with the ALSFRS-r score and positively correlated with the decrease in ALSFRS-r score, DPR, and UMN score. There was no correlation between levels of NF-L and duration. In addition, the cumulative survival rate in ALS patients with a low level of NF-L was higher than in patients with a high level of NF-L. Conclusions: NF-L levels increased in CSF and serum of patients with ALS. NF-L may thus be a neurodegenerative biomarker for predicting ALS severity and progression, and the survival of patients with this disease.

scholarly journals Clinical Determinants of Disease Progression in Amyotrophic Lateral Sclerosis—A Retrospective Cohort Study

2021 ◽  
Vol 10 (8) ◽  
pp. 1623
Author(s):  
Maria Viktoria Requardt ◽  
Dennis Görlich ◽  
Torsten Grehl ◽  
Matthias Boentert
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Disease Progression ◽  
Rating Scale ◽  
Rapid Decline ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Hazard Ratios ◽  
Sum Score ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that is ultimately fatal but characterized by substantial phenotypic heterogeneity, which is known to impact long-term course and survival. This study investigated clinical determinants of disease progression and outcome in a large cohort of patients with ALS. Methods: Retrospective analysis included comprehensive data from 625 patients who attended a tertiary ALS centre at least twice. Patients were stratified according to five distinct clinical phenotypes: classical ALS; bulbar ALS; ALS with frontotemporal dementia (ALS-FTD); upper motor neuron predominant (UMNP); and lower motor neuron predominant (LMNP). Results: This study confirmed higher age at symptom onset, shorter latency to diagnosis and more rapid decline in the revised ALS Functional Rating Scale sum score as predictors of poor prognosis. Hazard ratios for shorter survival were higher in patients with ALS-FTD versus classical ALS, and in patients with versus without chronic obstructive pulmonary disease (COPD). Mean survival was longest in the UMNP phenotype group. Conclusions: This study confirmed established predictors of shorter survival in ALS and showed that concomitant COPD in particular relates to poor outcome.

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