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Quantifying disease progression in amyotrophic lateral sclerosis.

Simon NG, Turner MR, Vucic S, Al-Chalabi A, Shefner J, Lomen-Hoerth C, Kiernan MC - Ann. Neurol. (2014)

Bottom Line: Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of disease progression, with inherent clinical heterogeneity making disease quantitation difficult.Recent advances in understanding pathogenic mechanisms linked to the development of ALS impose an increasing need to develop strategies to predict and more objectively measure disease progression.This review explores phenotypic and genetic determinants of disease progression in ALS, and examines established and evolving biomarkers that may contribute to robust measurement in longitudinal clinical studies.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, University of California, San Francisco, San Francisco, CA; Prince of Wales Clinical School, University of New South Wales, Randwick, Australia; Neuroscience Research Australia, Barker St, Randwick, Australia.

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Brain imaging markers of disease. (A) The corticospinal tracts (CST) can be reconstructed using diffusion tensor tractography. (B) A scatterplot of the extracted mean CST fractional anisotropy (FA) against the rate of decline of Amyotrophic Lateral Sclerosis (ALS) Functional Rating Scale (ALSFRS) score (points per month) shows a negative correlation, with potential to prognostically stratify patients (adapted from Fig 3 in Turner et al64). (C) Longitudinal gray matter changes are extensive in ALS, detected using voxel-based morphometry. They include extramotor frontal lobe regions and basal ganglia (regions of significantly reduced gray matter density common to a large group of ALS patients over time, shown in yellow–red scale overlaid on standard brain image in 3 planes, with anterior [A], posterior [P], right [R], and left [L] marked).
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fig02: Brain imaging markers of disease. (A) The corticospinal tracts (CST) can be reconstructed using diffusion tensor tractography. (B) A scatterplot of the extracted mean CST fractional anisotropy (FA) against the rate of decline of Amyotrophic Lateral Sclerosis (ALS) Functional Rating Scale (ALSFRS) score (points per month) shows a negative correlation, with potential to prognostically stratify patients (adapted from Fig 3 in Turner et al64). (C) Longitudinal gray matter changes are extensive in ALS, detected using voxel-based morphometry. They include extramotor frontal lobe regions and basal ganglia (regions of significantly reduced gray matter density common to a large group of ALS patients over time, shown in yellow–red scale overlaid on standard brain image in 3 planes, with anterior [A], posterior [P], right [R], and left [L] marked).


Quantifying disease progression in amyotrophic lateral sclerosis.

Simon NG, Turner MR, Vucic S, Al-Chalabi A, Shefner J, Lomen-Hoerth C, Kiernan MC - Ann. Neurol. (2014)

Brain imaging markers of disease. (A) The corticospinal tracts (CST) can be reconstructed using diffusion tensor tractography. (B) A scatterplot of the extracted mean CST fractional anisotropy (FA) against the rate of decline of Amyotrophic Lateral Sclerosis (ALS) Functional Rating Scale (ALSFRS) score (points per month) shows a negative correlation, with potential to prognostically stratify patients (adapted from Fig 3 in Turner et al64). (C) Longitudinal gray matter changes are extensive in ALS, detected using voxel-based morphometry. They include extramotor frontal lobe regions and basal ganglia (regions of significantly reduced gray matter density common to a large group of ALS patients over time, shown in yellow–red scale overlaid on standard brain image in 3 planes, with anterior [A], posterior [P], right [R], and left [L] marked).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4305209&req=5

fig02: Brain imaging markers of disease. (A) The corticospinal tracts (CST) can be reconstructed using diffusion tensor tractography. (B) A scatterplot of the extracted mean CST fractional anisotropy (FA) against the rate of decline of Amyotrophic Lateral Sclerosis (ALS) Functional Rating Scale (ALSFRS) score (points per month) shows a negative correlation, with potential to prognostically stratify patients (adapted from Fig 3 in Turner et al64). (C) Longitudinal gray matter changes are extensive in ALS, detected using voxel-based morphometry. They include extramotor frontal lobe regions and basal ganglia (regions of significantly reduced gray matter density common to a large group of ALS patients over time, shown in yellow–red scale overlaid on standard brain image in 3 planes, with anterior [A], posterior [P], right [R], and left [L] marked).
Bottom Line: Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of disease progression, with inherent clinical heterogeneity making disease quantitation difficult.Recent advances in understanding pathogenic mechanisms linked to the development of ALS impose an increasing need to develop strategies to predict and more objectively measure disease progression.This review explores phenotypic and genetic determinants of disease progression in ALS, and examines established and evolving biomarkers that may contribute to robust measurement in longitudinal clinical studies.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, University of California, San Francisco, San Francisco, CA; Prince of Wales Clinical School, University of New South Wales, Randwick, Australia; Neuroscience Research Australia, Barker St, Randwick, Australia.

Show MeSH
Related in: MedlinePlus