The effects of London air pollution on vegetation.

Imperial Users onl

Preparing for a U.S. National ALS Registry: Lessons from a pilot project in the State of Georgia

Our objective was to investigate the utility of existing data sources for identifying cases of amyotrophic lateral sclerosis (ALS) and related motor neuron diseases (MND) in the State of Georgia. Data were acquired from Medicare, Medicaid, Veterans Administration, Emory Healthcare, community neurologists, the ALS Association, and mortality records for ALS/MND patients residing in Georgia during 2001-2005. A neurologist used abstracted medical records to verify the diagnosis of ALS/MND. The positive predictive value (PPV) of an ICD code for a verified diagnosis of ALS was estimated. Simple 'rules' were developed to improve PPV. Results showed that a total of 2413 unique potential cases were identified in existing data sources. Medical records of 579 cases were available for review; the diagnosis of ALS (or a related MND) was confirmed in 486 (PPV = 84%) cases. Predictive rules, which permitted classification of ∼80% of the chart-reviewed population, improved PPV to 96-98%. In conclusion, existing data sources are useful for identifying cases of ALS/MND; most data sources contribute a substantial number of unique cases. Predictive algorithms may permit correct classification of a large proportion of cases without the need for verification based on medical record review

Utility of axial and radial diffusivity from diffusion tensor MRI as markers of neurodegeneration in amyotrophic lateral sclerosis

Objective: To investigate changes in the diffusion tensor imaging measures, axial diffusivity and radial diffusivity, in addition to the more commonly used fractional anisotropy and mean diffusivity, in patients with amyotrophic lateral sclerosis (ALS) using the voxel-based statistical analysis tool, tract based spatial statistics. Methods: We studied 12 patients with ALS and 19 normal controls using diffusion tensor imaging; tract based spatial statistics was applied to study changes in fractional anisotropy, mean diffusivity, axial diffusivity and radial diffusivity values in brain white matter tracts. ALS patients were evaluated using clinical examination, administration of the revised ALS functional rating scale and measurement of the forced vital capacity. Results: In ALS patients, we found significant increases in axial diffusivity, radial diffusivity, and mean diffusivity and significant decreases in fractional anisotropy. Increases in axial diffusivity and radial diffusivity were more widespread and more prominent in the corticospinal tract than the decreases in fractional anisotropy. The decreases in fractional anisotropy were evident only in the corona radiata and genu of the corpus callosum. Conclusion: In ALS, axial diffusivity and radial diffusivity may be useful diffusion tensor imaging-derived indices to consider in addition to fractional anisotropy and mean diffusivity to aid in demonstrating neurodegenerative changes

Diffusion tensor imaging reveals regional differences in the cervical spinal cord in amyotrophic lateral sclerosis

Therapeutic development in amyotrophic lateral sclerosis (ALS) is hampered by the lack of suitable biomarkers that might be sensitive to spatial and temporal patterns of neurodegeneration. Diffusion tensor imaging is a useful non-invasive tool that permits detection of microstructural tissue changes due, for example, to neurodegeneration. Even though the spinal cord bears the brunt of the disease process, diffusion tensor imaging has mainly been used to study white matter changes in the brain. The aim of this study was to examine the diffusion tensor imaging parameters of the cervical spinal cord (C1 through C6 segments) and brainstem (corticospinal tracts in the pyramids and pons) among ALS patients, to compare these to findings in age-matched healthy controls, and to correlate these differences with clinical measures of disease severity. Fractional anisotropy in the white matter of the cervical cord was 12% lower ( p < 0.01) in ALS patients ( n = 14) compared to age-matched healthy control subjects ( n = 15), and showed significant positive correlation with the average finger and foot tapping speed ( r = 0.61, p < 0.05) in ALS patients. Radial diffusivity in the cervical cord was 15% higher ( p < 0.05) in ALS patients compared to healthy control subjects. Radial diffusivity in the white matter of the cervical cord was significantly correlated with clinical measures of disease severity such as forced vital capacity (FVC % predicted, r = −0.69, p < 0.01), average finger and foot tapping speed from all four limbs ( r = −0.59, p < 0.05), and ALSFRS-R ( r = −0.55, p < 0.05) in ALS patients. There were no significant differences in mean diffusivity or axial diffusivity in the cervical spinal cord, or in any diffusion tensor imaging parameters measured in the brainstem. Analysis of diffusion tensor imaging parameters from individual cervical segments as well as profile plots along the length of the cervical cord showed larger differences in fractional anisotropy and radial diffusivity at more distal cervical segments, providing evidence that supports the “dying-back” hypothesis of neurodegeneration in ALS. ►1. DTI in the cervical cord showed reduced FA and increased in RD in ALS patients. ►2. DTI parameters correlated with clinical measures of disease severity in ALS. ►3. Distal cervical segments generally showed larger changes in RD and FA in ALS. ►4. These results may support the dying-back hypothesis of neurodegeneration in ALS. ►5. DTI may be a suitable imaging biomarker for monitoring disease progression in ALS

An Exploratory Study of Spiritual Care at the End of Life

PURPOSE Although spiritual care is a core element of palliative care, it remains unclear how this care is perceived and delivered at the end of life. We explored how clinicians and other health care workers understand and view spiritual care provided to dying patients and their family members

Electrical impedance myography as a biomarker to assess ALS progression

Electrical impedance myography (EIM), a non-invasive, electrophysiological technique, has preliminarily shown value as an ALS biomarker. Here we perform a multicenter study to further assess EIM’s potential for tracking ALS. ALS patients were enrolled across eight sites. Each subject underwent EIM, handheld dynamometry (HHD), and the ALS Functional Rating Scale-revised (ALSFRS-R) regularly. Techniques were compared by assessing the coefficient of variation (CoV) in the rate of decline and each technique’s correlation to survival. Results showed that in the 60 patients followed for one year, EIM phase measured from the most rapidly progressing muscle in each patient had a CoV in the rate of decline of 0.62, compared to HHD (0.82) and the ALSFRS-R (0.74). Restricting the measurements to the first six months gave a CoV of 0.55 for EIM, 0.93 for HHD, and 0.84 for ALSFRS-R. For both time-periods, all three measures correlated with survival. Based on these data, a six-month clinical trial designed to detect a 20% treatment effect with 80% power using EIM would require only 95 patients/arm compared to the ALSFRS-R, which would require 220 subjects/arm. In conclusion, EIM can serve as a useful ALS biomarker that offers the prospect of greatly accelerating phase 2 clinical trials