Toward in vivo determination of peripheral nervous system immune activity in amyotrophic lateral sclerosis

Muscle Nerve. 2019 May;59(5):567-576. doi: 10.1002/mus.26444. Epub 2019 Mar 8.

Abstract

Introduction: We sought to identify patients with amyotrophic lateral sclerosis (ALS) who displayed suspected peripheral nervous system (PNS) inflammation to compare them to those with suspected PNS degeneration.

Methods: We measured sonographic median and ulnar nerve cross-sectional area (CSA) and cerebrospinal fluid albumin/serum albumin ratio (Qalb ) in patients with ALS to classify them as having suspected PNS degeneration (small CSA/low Qalb ) or inflammation (larger CSA/high Qalb ).

Results: Fifty-seven percent of patients had suspected PNS degeneration, 21% had suspected PNS inflammation, and 21% displayed suspected "normal PNS state." Suspected PNS degeneration was related to classic ALS, shorter disease duration, and a smaller hypoechoic nerve area. Suspected PNS inflammation was associated with men, longer disease duration, and a larger hypoechoic nerve area and was the dominant finding in superoxide dismutase 1 mutation carriers.

Discussion: Our simple approach might aid in the in vivo differentiation of supposed ALS subtypes, those with suspected PNS degeneration vs. inflammation, for stratification in clinical trials. Muscle Nerve 59:567-567, 2019.

Keywords: ALS; cerebrospinal fluid; peripheral nervous system; peripheral nervous system inflammation; peripheral neuropathy; ultrasound.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Albumins / cerebrospinal fluid
  • Amyotrophic Lateral Sclerosis / diagnostic imaging*
  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / immunology
  • Female
  • Humans
  • Inflammation / diagnostic imaging
  • Male
  • Median Nerve / diagnostic imaging*
  • Median Nerve / pathology
  • Middle Aged
  • Mutation
  • Organ Size
  • Peripheral Nervous System / diagnostic imaging
  • Peripheral Nervous System / immunology
  • ROC Curve
  • Serum Albumin
  • Superoxide Dismutase-1 / genetics
  • Ulnar Nerve / diagnostic imaging*
  • Ulnar Nerve / pathology
  • Ultrasonography

Substances

  • Albumins
  • Serum Albumin
  • Superoxide Dismutase-1