Prevalence of Amyotrophic Lateral Sclerosis — United States, 2015

Mehta, Paul; Kaye, Wendy; Raymond, Jaime; Punjani, Reshma; Larson, Theodore; Cohen, Jessica; Muravov, Oleg; Horton, D. Kevin

doi:10.15585/mmwr.mm6746a1

Cited by 157 publications

(144 citation statements)

References 5 publications

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“…Controls had higher educational levels, which contrasts to National ALS Registry data showing a higher rate of ALS in more educated persons. 51 While other ALS studies do have control groups with higher educational attainment, 29 the use of a primarily internet-based recruitment strategy for controls may have biased the group towards higher socioeconomic levels. Every model adjusted for education to control for potential confounding.…”

Section: Discussionmentioning

confidence: 99%

Association of Environmental Toxins With Amyotrophic Lateral Sclerosis

et al. 2016

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Section: Discussionmentioning

confidence: 99%

Association of Environmental Toxins With Amyotrophic Lateral Sclerosis

et al. 2016

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“…Neurodegeneration in ALS typically advances in a sequential fashion to the point of phrenic nerve involvement resulting in failure of respiratory effort and death before degenerative changes are seen elsewhere [3]. ALS affects approximately 3.9 in 100,000 people within the United States [4] with approximately equal occurrence worldwide and does not appear to be linked with environmental toxins. Studies indicate that ALS incidence is approximately 1.8 times greater in males than females for unknown reasons [5].…”

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confidence: 99%

Non-invasive MRI quantification of cerebrospinal fluid dynamics in amyotrophic lateral sclerosis patients

Sass

Khani

Romm

et al. 2020

Fluids Barriers CNS

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Background: Developing novel therapeutic agents to treat amyotrophic lateral sclerosis (ALS) has been difficult due to multifactorial pathophysiologic processes at work. Intrathecal drug administration shows promise due to close proximity of cerebrospinal fluid (CSF) to affected tissues. Development of effective intrathecal pharmaceuticals will rely on accurate models of how drugs are dispersed in the CSF. Therefore, a method to quantify these dynamics and a characterization of differences across disease states is needed. Methods: Complete intrathecal 3D CSF geometry and CSF flow velocities at six axial locations in the spinal canal were collected by T2-weighted and phase-contrast MRI, respectively. Scans were completed for eight people with ALS and ten healthy controls. Manual segmentation of the spinal subarachnoid space was performed and coupled with an interpolated model of CSF flow within the spinal canal. Geometric and hydrodynamic parameters were then generated at 1 mm slice intervals along the entire spine. Temporal analysis of the waveform spectral content and feature points was also completed. Results: Comparison of ALS and control groups revealed a reduction in CSF flow magnitude and increased flow propagation velocities in the ALS cohort. Other differences in spectral harmonic content and geometric comparisons may support an overall decrease in intrathecal compliance in the ALS group. Notably, there was a high degree of variability between cases, with one ALS patient displaying nearly zero CSF flow along the entire spinal canal. Conclusion: While our sample size limits statistical confidence about the differences observed in this study, it was possible to measure and quantify inter-individual and cohort variability in a non-invasive manner. Our study also shows the potential for MRI based measurements of CSF geometry and flow to provide information about the hydrodynamic environment of the spinal subarachnoid space. These dynamics may be studied further to understand the behavior of CSF solute transport in healthy and diseased states.

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“…There were methodological limitations to this study. Patient samples were drawn from individuals attending large ALS referral centers, and thus may not be representative of the ALS patient population as a whole, despite a similar age and gender distribution to that of the general ALS population . Data from the 2 studies were collected at different time periods, and it is possible that patient care or values relating to QOL changed in the interval.…”

Section: Discussionmentioning

confidence: 99%