Amyotrophic Lateral Sclerosis Progression Associated With Changes in Immune Status

Share this content:
It is not known how changes in immune cell status impact disease status in ALS.
It is not known how changes in immune cell status impact disease status in ALS.

Changes in the number of neutrophils and CD4 T cells are associated with progression of amyotrophic lateral sclerosis (ALS), according to results of a longitudinal cohort study published in JAMA Neurology.

In this study, researchers sought to identify immune system changes by evaluating peripheral inflammatory markers in patients with ALS (n=119) and healthy controls (n=35). Additionally, the researchers wished to identify whether or not these changes correlated with progression of ALS.

Investigators found that people with ALS had numerically higher average counts of total leukocytes (× 106/mL) compared with healthy controls (5.53 [0.16; 95% CI, 5.21-5.84] vs 4.57 [0.29; 95% CI, 3.94-5.11], respectively). Additionally, patients with ALS also had a greater number of neutrophils compared with controls (3.80 [0.12; 95% CI, 3.56-4.04] vs 2.87 [0.23; 95% CI, 2.40-3.35], respectively). CD16+ and CD16- monocytes were also higher among patients with ALS.

Change in the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) was also observed, demonstrating a significant association with immune system changes and ALS progression. These changes were most noticeable with CD4 T cells (-30.47 [95% CI, -46.02 to -14.94] per -3.72 × 104/mL [SD, 26.21 × 104/mL] per year) and neutrophils (-4.37 [95% CI, -6.60 to -2.14] per 11.47 × 104/mL [SD, 58.04 × 104/mL] per year).

This study examined correlations only and failed to establish a causative component related to immune system changes and ALS disease progression. The researchers suggested that “changes in peripheral immunity may be a consequence rather than a cause of disease.”


Murdock BJ, Zhou T, Kashlan SR, Little RJ, Goutman SA, Feldman EL. Correlation of peripheral immunity with rapid amyotrophic lateral sclerosis progression [published online September 25, 2017]. JAMA Neurol. doi:10.1001/jamaneurol.2017.225

You must be a registered member of Neurology Advisor to post a comment.

Next Article in Neuromuscular Disorders

Sign Up for Free e-newsletters

CME Focus