Quantifying disease progression in amyotrophic lateral sclerosis using peripheral nerve sonography

2016 ◽  
Vol 54 (3) ◽  
pp. 391-397 ◽  
Author(s):  
Stefanie Schreiber ◽  
Verena Dannhardt-Stieger ◽  
Dorothea Henkel ◽  
Grazyna Debska-Vielhaber ◽  
Judith Machts ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Peripheral Nerve ◽  
Disease Progression ◽  
Lateral Sclerosis ◽  
Nerve Sonography

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.

Intravenous infusion of mesenchymal stem cells delays disease progression in the SOD1G93A transgenic amyotrophic lateral sclerosis rat model

2021 ◽  
Vol 1757 ◽  
pp. 147296
Author(s):  
Hirotoshi Magota ◽  
Masanori Sasaki ◽  
Yuko Kataoka-Sasaki ◽  
Shinichi Oka ◽  
Ryo Ukai ◽  
...  
Keyword(s):  
Stem Cells ◽  
Amyotrophic Lateral Sclerosis ◽  
Mesenchymal Stem Cells ◽  
Disease Progression ◽  
Rat Model ◽  
Intravenous Infusion ◽  
Lateral Sclerosis

Altered Metabolic Homeostasis in Amyotrophic Lateral Sclerosis: Mechanisms of Energy Imbalance and Contribution to Disease Progression

2016 ◽  
Vol 16 (5-6) ◽  
pp. 382-397 ◽  
Author(s):  
Zara A. Ioannides ◽  
Shyuan T. Ngo ◽  
Robert D. Henderson ◽  
Pamela A. McCombe ◽  
Frederik J. Steyn
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Metabolic Homeostasis ◽  
Energy Imbalance ◽  
Lateral Sclerosis

Developments in the assessment of non-motor disease progression in amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Adriano Chiò ◽  
Antonio Canosa ◽  
Andrea Calvo ◽  
Cristina Moglia ◽  
Alessandro Cicolin ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Lateral Sclerosis

Regionality of disease progression predicts prognosis in amyotrophic lateral sclerosis

2015 ◽  
Vol 16 (7-8) ◽  
pp. 442-447 ◽  
Author(s):  
Lisa A. van der Kleij ◽  
Ashley R. Jones ◽  
I Nick Steen ◽  
Carolyn A. Young ◽  
Pamela J. Shaw ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Lateral Sclerosis

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

2019 ◽  
Vol 6 (6) ◽  
pp. e631 ◽  
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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