Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese

Wang, Wenqing; Liu, Chang; Li, Wei; Zhang, Dong; Sangbong, Yi; Zheng, Jieyun; Shan, Jingli; Zhao, Yuhan; Yan, Chuanzhu; Wang, Qinzhou

doi:10.1002/acn3.51550

Cited by 12 publications

(7 citation statements)

References 28 publications

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“…CSF albuminocytologic dissociation is a common phenomenon in CIDP, and the vast majority of patients with AN with anti-NF155 antibodies had markedly elevated CSF protein levels−up to more than 3.8 g/L−which were much higher than those in serologically negative patients with CIDP. At the meantime, magnetic resonance imaging (MRI) of peripheral nerves showed thickening of nerve roots in the cervical plexus or lumbosacral plexus, which is similar to what has been reported in previous studies ( 8 , 12 ) ( Figure 2 ). The reason for the elevated CSF protein levels remains unclear and may be related to thickening of the nerve root.…”

Section: Discussionsupporting

confidence: 88%

Clinical characteristics of patients with autoimmune nodopathy with anti-neurofascin155 antibodies

Zhang,

Hou,

Wei

et al. 2024

Front. Immunol.

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BackgroundAccording to the latest guidelines on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), patients with CIDP with anti-neurofascin 155 (NF155) antibodies are referred to as autoimmune nodopathy (AN), an autoimmune disorder distinct from CIDP. We aimed to compare the clinical data of patients with AN with anti-NF155 antibodies with those of anti-NF155 antibodies-negative patients with CIDP, and to summarize the clinical characteristics of patients with AN with anti-NF155 antibodies.MethodsNine patients with AN with anti-NF155 antibodies and 28 serologically negative patients with CIDP were included in this study. Diagnosis was made according to the diagnostic criteria in the European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) guidelines on CIDP published in 2021. Demographics, clinical manifestations, electrophysiological examination, cerebrospinal fluid (CSF) tests, and response to treatment were retrospectively analyzed.ResultsCompared with serologically negative patients with CIDP, those patients with AN with anti-NF155 antibodies were younger (p=0.007), had a younger onset age (p=0.009), more frequent ataxia (p=0.019), higher CSF protein levels (p=0.001), and more frequent axon damage in electrophysiology (p=0.025). The main characteristics of patients with AN with anti-NF155 antibodies include younger age and onset age, limb weakness, sensory disturbance, ataxia, multiple motor−sensory peripheral neuropathies with demyelination and axonal damage on electrophysiological examination, markedly elevated CSF protein levels, and varying degrees of response to immunotherapy.ConclusionsPatients with AN with anti-NF155 antibodies differed from serologically negative patients with CIDP in terms of clinical characteristics. When AN is suspected, testing for antibodies associated with the nodes of Ranvier is essential for early diagnosis and to guide treatment.

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Section: Discussionsupporting

confidence: 88%

Clinical characteristics of patients with autoimmune nodopathy with anti-neurofascin155 antibodies

Zhang,

Hou,

Wei

et al. 2024

Front. Immunol.

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“…It has demonstrated sufficient efficacy and good tolerability in many autoimmune diseases. Rituximab also induced clinical remission in approximately 80% of patients with anti-NF155 nodopathy both at common doses (375 mg/m 2 weekly for 4 weeks followed by additional doses) and low doses (0.1 g and 0.5 g, IV) ( 4 , 6 , 7 ). However, rituximab can elicit anti-drug antibodies due to the high immunogenicity resulting from its structure.…”

Section: Discussionmentioning

confidence: 99%

Anti-rituximab antibodies in patients with refractory autoimmune nodopathy with anti-neurofascin-155 antibody

Bai

Yan

et al. 2023

Front. Immunol.

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BackgroundRecent studies have reported that similar to other IgG4 autoimmune diseases, such as muscle-specific kinase antibody-associated myasthenia gravis, most anti-neurofascin-155 (anti-NF155) nodopathies respond well to rituximab treatment, regardless of the dosage. However, there are still a few patients for which rituximab is ineffective for unknown reasons. Currently, there are no studies on the mechanism of ineffective treatment with rituximab.MethodsA 33-year-old Chinese man presenting with numbness, tremor, and muscle weakness for 4 years was recruited for this study. Anti-NF155 antibodies were identified by cell-based assay and confirmed by immunofluorescence assay on teased fibers. The anti-NF155 immunoglobulin (IgG) subclasses were also detected by immunofluorescence assay. Anti-rituximab antibodies (ARAs) were quantitatively analyzed using enzyme-linked immunosorbent assay (ELISA), and peripheral B cell counts were determined by flow cytometry.ResultsThe patient exhibited anti-NF155 IgG4-antibody positivity. After the first round of rituximab infusion, the patient showed stratified outcomes with improvements in numbness, muscle weakness and ambulation. However, after three rounds of rituximab infusion, the patient’s symptoms deteriorated, and the numbness, tremor and muscle weakness returned. No obvious improvement was found after plasma exchange and another round of rituximab treatment. 14 days after the last treatment with rituximab, ARAs were detected. And the titers gradually decreased on day 28 and 60 but remained higher than normal. Peripheral CD19+ B cell counts were less than 1% within the 2-month period following the final rituximab administration.ConclusionsIn this study, ARAs presented in a patient with anti-NF155 nodopathy undergoing rituximab treatment and showed an unfavorable impact on rituximab efficacy. This is the first case to report the occurrence of ARAs in patients with anti-NF155 antibodies. We suggest that ARAs should be tested early during the initial intervention, especially in patients who respond poorly to rituximab treatment. In addition, we believe it is necessary to investigate the association between ARAs and B cell counts, their effect on clinical efficacy, and their potential adverse reactions in a larger cohort of patients with anti-NF155 nodopathy.

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“…Abnormalities on NCS/EMG included prolonged distal and F-wave latencies, slow conduction velocities, conduction block, and temporal dispersion [ 10 ]. Paranodal antibodies have mostly been reported in treatment-refractory CIDP with prominent distal involvement, ataxia, and disabling tremor [ 26 , 27 ]. In our cases, we found acute onset demyelinating or axonal neuropathy and polyradiculopathy without demyelination.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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Clinical and diagnostic features of anti‐neurofascin‐155 antibody‐positive neuropathy in Han Chinese

Cited by 12 publications

References 28 publications

Clinical characteristics of patients with autoimmune nodopathy with anti-neurofascin155 antibodies

Clinical characteristics of patients with autoimmune nodopathy with anti-neurofascin155 antibodies

Anti-rituximab antibodies in patients with refractory autoimmune nodopathy with anti-neurofascin-155 antibody

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

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