Peripherin is a biomarker of axonal damage in peripheral nervous system disease

Brain, 2023 · DOI: https://doi.org/10.1093/brain/awad234 · Published: July 12, 2023

Simple Explanation

This study investigates peripherin as a potential biomarker for axonal damage in peripheral nervous system diseases. Peripherin is a protein found almost exclusively in peripheral nerve axons, making it a promising candidate. The researchers developed an immunoassay to measure peripherin levels in the blood and compared these levels across different neurological conditions. The study found that peripherin levels were significantly elevated in patients with Guillain-Barré syndrome (GBS), an acute peripheral nerve disorder, compared to those with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multiple sclerosis, dementia, and healthy controls. This suggests that peripherin is a specific marker for acute peripheral nerve axonal damage. Unlike neurofilament light chain (NfL), another biomarker for nerve damage, peripherin levels did not increase with age, which simplifies interpretation. The researchers suggest that peripherin, in combination with NfL, could improve the accuracy of diagnosing and differentiating between different types of neuropathies.

Study Duration

Not specified

Participants

45 GBS, 35 CIDP, 30 MS, 30 Dementia, and 24 healthy individuals

Evidence Level

Not specified

Key Findings

1
Peak peripherin levels were significantly higher in GBS patients compared to other groups, indicating its potential as a specific biomarker for acute PNS axonal damage.
2
Peripherin levels showed a rise-and-fall pattern in GBS patients, peaking within the first week of assessment, suggesting dynamic sensitivity to disease activity.
3
A combination of peak serum peripherin and NfL levels improved the specificity in differentiating GBS from CIDP, highlighting the potential for combined biomarker analysis.

Research Summary

This study introduces a sensitive immunoassay for measuring serum peripherin as a biomarker for peripheral nerve axonal injury. The research demonstrates that peripherin levels are significantly elevated in GBS patients compared to other neurological conditions and healthy controls, suggesting its specificity for acute PNS axonal damage. The study also reveals that peripherin levels exhibit a dynamic rise-and-fall pattern in GBS, peaking early after presentation and correlating with disease activity. This contrasts with NfL, another biomarker, and suggests that peripherin may offer more precise monitoring of disease progression and recovery in some cases. Furthermore, the research indicates that combining peripherin and NfL measurements improves the accuracy in differentiating GBS from CIDP, supporting the potential for multi-biomarker approaches in diagnosing and managing peripheral neuropathies.

Practical Implications

Diagnostic Tool

Peripherin can serve as a specific biomarker for diagnosing acute peripheral nerve axonal damage, particularly in GBS.

Disease Monitoring

Monitoring peripherin levels can help track disease activity and recovery in GBS patients, potentially guiding treatment decisions.

Differential Diagnosis

Combining peripherin and NfL measurements can improve the differentiation between GBS and CIDP, leading to more accurate diagnoses.

Study Limitations

1
Small group sizes in some analyses may limit statistical power.
2
The kinetics of serum peripherin distribution and elimination are not fully understood.
3
Unexplained high peripherin levels in some controls and CNS disease patients require further investigation.

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