Anti-Neurofascin Antibodies Associated with White Matter Diseases of the Central Nervous System: A Red Flag or a Red Herring?

Autoantibodies against nodal and paranodal proteins, specifically anti-neurofascin antibodies (ANFAs), have been recently described in central and peripheral nervous system demyelinating disorders. We retrospectively reviewed the charts of six individuals evaluated at our Multiple Sclerosis Program...

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Bibliographic Details
Published in:	Brain sciences Vol. 12; no. 9; p. 1124
Main Authors:	Gupta, Navnika, Shirani, Afsaneh, Arcot Jayagopal, Lakshman, Piccione, Ezequiel, Hartman, Elizabeth, Zabad, Rana Khalil
Format:	Journal Article
Language:	English
Published:	Basel MDPI AG 24-08-2022 MDPI
Subjects:	anti-neurofascin antibodies Antibodies Autoantibodies Brain Brief Report Central nervous system Central nervous system diseases Cerebrospinal fluid combined central and peripheral demyelination Coronaviruses COVID-19 demyelinating diseases Demyelination Disease Electromyography Guillain-Barre syndrome Immunoglobulin G Immunoglobulin M Laboratories Magnetic resonance imaging Multiple sclerosis Muscle strength Myelitis Nervous system Neutrophils nodes of Ranvier Ostomy paranodes Pathology Patients Polyneuropathy Proteins Spinal cord Substantia alba Tracheotomy Ventilation White people
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Summary:	Autoantibodies against nodal and paranodal proteins, specifically anti-neurofascin antibodies (ANFAs), have been recently described in central and peripheral nervous system demyelinating disorders. We retrospectively reviewed the charts of six individuals evaluated at our Multiple Sclerosis Program who tested positive for serum ANFAs on Western blot. We describe these patients’ clinical and diagnostic findings and attempt to identify features that might guide clinicians in checking for ANFAs. In our series, the women-to-men ratio was 2:1. At presentation, the median age was 60 years (range 30–70). The clinical presentation was pleiotropic and included incomplete transverse myelitis (n = 3), progressive myelopathy (n = 1), recurrent symmetric polyneuropathy (n = 1), and nonspecific neurological symptoms (n = 1). Atypical features prompting further workup included coexisting upper and lower motor neuron features, older age at presentation with active disease, atypical spinal cord MRI features, and unusual cerebrospinal fluid findings. The serum ANFAs panel was positive for the NF-155 isoform in five patients (IgM n = 2; IgG n = 2; both n = 1) and the NF-140 isoform in two (IgG n = 2). Larger studies are needed to assess the relevance of ANFAs in demyelinating nervous system diseases, their impact on long-term clinical outcomes, and associated therapeutic implications.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	2076-3425 2076-3425
DOI:	10.3390/brainsci12091124