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

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.

• Authors: Stefanie Schreiber, Frank Schreiber, Cornelia Garz, Grazyna Debska-Vielhaber, Anne Assmann, Valentina Perosa, Susanne Petri, Reinhard Dengler, Peter Nestor, Stefan Vielhaber
• Journal: Muscle & nerve (Muscle Nerve) Vol. 59 Issue 5 Pg. 567-576 (05 2019) ISSN: 1097-4598 [Electronic] United States
• PMID: 30734322 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2019 Wiley Periodicals, Inc.

Chemical References

• Albumins
• Serum Albumin
• Superoxide Dismutase-1

Topics

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