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Aggressive Acquired Demyelinating Neuropathy Caused by NF-155: Initially Treatment-Resistant.

Selene Jamall¹, Nancy Baker, Gordon Peterson

¹Department of Neurology, Loma Linda University Medical Center, Loma Linda, CA.

Journal of Clinical Neuromuscular Disease

|November 14, 2023

Summary

Anti-neurofascin-155 IgG4 antibody disease can mimic acute inflammatory demyelinating polyradiculoneuropathy. Early identification of anti-NF-155 antibodies and prompt treatment with rituximab and steroids can improve patient outcomes.

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Area of Science:

Neurology
Immunology
Pathology

Background:

Anti-neurofascin-155 IgG4 (NF-155) antibody disease is a rare condition.
It is often associated with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Observation:

A 40-year-old male presented with acute onset weakness, areflexia, and sensory loss.
Initial treatment with IVIg led to temporary improvement followed by rapid decline.
The patient developed quadriplegia, areflexia, and oculobulbar paralysis.

Findings:

The patient tested positive for NF-155 antibodies.
Treatment with rituximab and steroids resulted in significant recovery.
This case highlights a variant presentation of NF-155 antibody disease.

Implications:

Early testing for NF-155 antibodies is crucial in cases of demyelinating neuropathy unresponsive to standard treatments.
Prompt diagnosis and targeted therapy with rituximab and steroids can lead to better recovery.
This underscores the importance of identifying specific antibody targets in autoimmune neuropathies.