Amyotrophic lateral sclerosis: prospective study on respiratory parameters

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.

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

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.715842 ◽

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.

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Faculty Opinions recommendation of Respiratory impairment in a mouse model of amyotrophic lateral sclerosis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1053680.505587 ◽

2006 ◽

Author(s):

Gordon Mitchell

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Mouse Model ◽

Respiratory Impairment ◽

Lateral Sclerosis

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Biological follow-up in amyotrophic lateral sclerosis: decrease in creatinine levels and increase in ferritin levels predict poor prognosis

European Journal of Neurology ◽

10.1111/ene.12754 ◽

2015 ◽

Vol 22 (10) ◽

pp. 1385-1390 ◽

Cited By ~ 20

Author(s):

F. Patin ◽

P. Corcia ◽

B. Madji Hounoum ◽

C. Veyrat-Durebex ◽

E. Respaud ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Poor Prognosis ◽

Lateral Sclerosis

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Effect of one-year Dextromethorphan/Quinidine treatment on management of respiratory impairment in Amyotrophic Lateral Sclerosis

Respiratory Medicine ◽

10.1016/j.rmed.2021.106536 ◽

2021 ◽

pp. 106536

Author(s):

Jesus Sancho ◽

Santos Ferrer ◽

Enric Burés ◽

José Luis Díaz ◽

Teresa Torrecilla ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Respiratory Impairment ◽

One Year ◽

Lateral Sclerosis

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THE AMYOTROPHIC LATERAL SCLEROSIS/PARKINSONISMDEMENTIA COMPLEX: A TEN-YEAR FOLLOW-UP ON GUAM

American Journal of Epidemiology ◽

10.1093/oxfordjournals.aje.a120570 ◽

1966 ◽

Vol 83 (1) ◽

pp. 54-73 ◽

Cited By ~ 36

Author(s):

DWAYNE REED ◽

CHRIS PLATO ◽

TERESITA ELIZAN ◽

LEONARD T. KURLAND

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Lateral Sclerosis

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IL6 receptor358Ala variant and trans-signaling are disease modifiers in amyotrophic lateral sclerosis

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000631 ◽

2019 ◽

Vol 6 (6) ◽

pp. e631 ◽

Cited By ~ 8

Author(s):

Marlena Wosiski-Kuhn ◽

Mac Robinson ◽

Jane Strupe ◽

Phonepasong Arounleut ◽

Matthew Martin ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Disease Progression ◽

Interleukin 6 ◽

Serum Levels ◽

Interleukin 6 Receptor ◽

The Common ◽

Paired Serum ◽

Control Study ◽

Lateral Sclerosis

ObjectiveTo test the hypothesis that patients with amyotrophic lateral sclerosis (ALS) inheriting the common interleukin 6 receptor (IL6R) coding variant (Asp358Ala, rs2228145, C allele) have associated increases in interleukin 6 (IL6) and IL6R levels in serum and CSF and faster disease progression than noncarriers.MethodsAn observational, case-control study of paired serum and CSF of 47 patients with ALS, 46 healthy, and 23 neurologic disease controls from the Northeastern ALS Consortium Biofluid Repository (cohort 1) was performed to determine serum levels of IL6, sIL6R, and soluble glycoprotein 130 and compared across groups and IL6R genotype. Clinical data regarding disease progression from a separate cohort of 35 patients with ALS from the Wake Forest ALS Center (cohort 2) were used to determine change in ALSFRS-R scores by genotype.ResultsPatients with ALS had increased CSF IL6 levels compared with healthy (p < 0.001) and neurologic (p = 0.021) controls. Patients with ALS also had increased serum IL6 compared with healthy (p = 0.040) but not neurologic controls. Additive allelic increases in serum IL6R were observed in all groups (average increase of 52% with the presence of the IL6R C allele; p < 0.001). However, only subjects with ALS had significantly increased CSF sIL6R levels compared with controls (p < 0.001). When compared across genotypes, only patients with ALS inheriting the IL6R C allele exhibit increased CSF IL6. ALSFRS-R scores decreased more in patients with ALS with the IL6R C allele than in those without (p = 0.019).ConclusionsTheses results suggest that for individuals inheriting the IL6R C allele, the cytokine exerts a disease- and location-specific role in ALS. Follow-up, prospective studies are necessary, as this subgroup of patients may be identified as ideally responsive to IL6 receptor–blocking therapies.

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Prognostic value of weight loss in patients with amyotrophic lateral sclerosis: a population-based study

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2020-322909 ◽

2020 ◽

Vol 91 (8) ◽

pp. 867-875 ◽

Cited By ~ 1

Author(s):

Mark R Janse van Mantgem ◽

Ruben P A van Eijk ◽

Hannelore K van der Burgh ◽

Harold H G Tan ◽

Henk-Jan Westeneng ◽

...

Keyword(s):

Weight Loss ◽

Amyotrophic Lateral Sclerosis ◽

Body Weight ◽

Prognostic Value ◽

Population Based ◽

Data Set ◽

Population Based Study ◽

Risk Of Death ◽

Lateral Sclerosis

ObjectiveTo determine the prevalence and prognostic value of weight loss (WL) prior to diagnosis in patients with amyotrophic lateral sclerosis (ALS).MethodsWe enrolled patients diagnosed with ALS between 2010 and 2018 in a population-based setting. At diagnosis, detailed information was obtained regarding the patient’s disease characteristics, anthropological changes, ALS-related genotypes and cognitive functioning. Complete survival data were obtained. Cox proportional hazard models were used to assess the association between WL and the risk of death during follow-up.ResultsThe data set comprised 2420 patients of whom 67.5% reported WL at diagnosis. WL occurred in 71.8% of the bulbar-onset and in 64.2% of the spinal-onset patients; the mean loss of body weight was 6.9% (95% CI 6.8 to 6.9) and 5.5% (95% CI 5.5 to 5.6), respectively (p<0.001). WL occurred in 35.1% of the patients without any symptom of dysphagia. WL is a strong independent predictor of survival, with a dose response relationship between the amount of WL and the risk of death: the risk of death during follow-up increased by 23% for every 10% increase in WL relative to body weight (HR 1.23, 95% CI 1.13 to 1.51, p<0.001).ConclusionsThis population-based study shows that two-thirds of the patients with ALS have WL at diagnosis, which also occurs independent of dysphagia, and is related to survival. Our results suggest that WL is a multifactorial process that may differ from patient to patient. Gaining further insight in its underlying factors could prove essential for future therapeutic measures.

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Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-319611 ◽

2019 ◽

Vol 90 (6) ◽

pp. 666-673 ◽

Cited By ~ 15

Author(s):

Cristina Moglia ◽

Andrea Calvo ◽

Maurizio Grassano ◽

Antonio Canosa ◽

Umberto Manera ◽

...

Keyword(s):

Weight Loss ◽

Amyotrophic Lateral Sclerosis ◽

Median Survival ◽

Population Based ◽

Study Cohort ◽

Respiratory Impairment ◽

Clinical Correlates ◽

Severe Weight Loss ◽

Bulbar Onset ◽

Lateral Sclerosis

ObjectivesTo assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease.MethodsThe study cohort included all ALS patients in Piemonte/Valle d’Aosta in the 2007–2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated.ResultsOf the 712 patients, 620 (87.1%) were included in the study. Patients ’ survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20% of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients.ConclusionsThe rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.

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