Anti-NF155/NF186 IgG4 Antibody Positive Autoimmune Nodopathy

Wang, Lijun; Pan, Jing; Meng, Huanyu; Zhao, Yao; Zeng, Lili; Liu, Jun

doi:10.3390/brainsci12111587

Cited by 6 publications

(4 citation statements)

References 28 publications

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“…In contrast, tremor and ataxia, typical of NF155-associated disease, are not specifically associated with pan-neurofascin seropositivity, but were only reported in two male patients with GBS-like onset who did not show signs of severe motor involvement [25 ▪▪ ,27,28 ▪▪ ,32]. Both patients showed very low titers and noncomplement-activating IgG4 subclass, possibly explaining the benign phenotype compared with the severely affected patients described before.…”

Section: Clinical Features and Diagnostic Work-upmentioning

confidence: 74%

“…Multicenter studies are needed to evaluate the significance of antibodies targeting NF186 only, which are therefore not included in this review. Lately, antibodies targeting several neurofascin isoforms (‘pan-neurofascin’) have been described and linked to a unique phenotype: severe and therapy refractory Guillain-Barré syndrome (GBS)-like neuropathy with tetraplegia, cranial nerve involvement, autonomic dysfunction, and need of mechanical ventilation for months (see Table 1) [20–24,25 ▪▪ ,26,27,28 ▪▪ ,29–32]. This review summarizes insights on the molecular background, the clinical characteristics as well as diagnostic and therapeutic approaches for this rare and life-threatening condition…”

Section: Introductionmentioning

confidence: 99%

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Pan-Neurofascin autoimmune nodopathy – a life-threatening, but reversible neuropathy

Appeltshauser,

Doppler

2023

Current Opinion in Neurology

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Purpose of review Autoimmune nodopathies are immune-mediated neuropathies associated with antibodies targeting the peripheral node of Ranvier. Recently, antibodies against all neurofascin-isoforms (pan-neurofascin) have been linked to a clinical phenotype distinct from previously described autoimmune nodopathies. Here, we aim at highlighting the molecular background and the red flags for diagnostic assessment and provide treatment and surveillance approaches for this new disease. Recent findings Neurofascin-isoforms are located at different compartments of the node of Ranvier: Neurofascin-186 at the axonal nodal gap, and Neurofascin-155 at the terminal Schwann cell loops at the paranode. Pan-neurofascin antibodies recognize a common epitope on both isoforms and can access the node of Ranvier directly. Depending on their subclass profile, antibodies can induce direct structural disorganization and complement activation. Affected patients present with acute and immobilizing sensorimotor neuropathy, with cranial nerve involvement and long-term respiratory insufficiency. Early antibody-depleting therapy is crucial to avoid axonal damage, and remission is possible despite extended disease and high mortality. The antibody titer and serum neurofilament light chain levels can serve as biomarkers for diagnosis and therapy monitoring. Summary Pan-neurofascin-associated autoimmune nodopathies has unique molecular and clinical features. Testing should be considered in severe and prolonged Guillain-Barré-like phenotype.

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Section: Clinical Features and Diagnostic Work-upmentioning

confidence: 74%

Section: Introductionmentioning

confidence: 99%

Pan-Neurofascin autoimmune nodopathy – a life-threatening, but reversible neuropathy

Appeltshauser,

Doppler

2023

Current Opinion in Neurology

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“…Histologic examination of biopsied sural nerves in patients with NF155 + , in contrast to those with CIDP, show subperineurial edema and occasional paranodal demyelination without vasculitis, inflammatory cell infiltration, or onion bulbs. Loss of myelin fibers is minimal, even years after the onset of the disease ( 21 , 22 ).…”

Section: Discussionmentioning

confidence: 99%

“…Unlike postganglionic nerves, the preganglionic nerves are immersed in the CSF and are affected by its proteins and inflammatory factors. Notably, there are significant differences in CSF protein levels among these three diseases, with these levels being markedly higher in patients with NF155 + than in those with CIDP and MMN tending to be in the normal range ( 1 , 17 , 21 ). The correlation between CSF proteins and preganglionic nerve enlargement needs to be investigated in further studies.…”

Section: Discussionmentioning

confidence: 99%

Semiquantitative assessment of preganglionic nerves for chronic immune-mediated neuropathies using brachial plexus magnetic resonance imaging

Lu,

Wang,

et al. 2024

Quant Imaging Med Surg

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Background Brachial plexus magnetic resonance imaging (MRI) is an important noninvasive supplementary diagnostic method of chronic immune peripheral neuropathies, but few MRI studies on the preganglionic nerves have been conducted. This retrospective cross-sectional study aimed to establish a reliable assessment for brachial plexus preganglionic nerve thickness and to use this method to assess and compare nerve characteristics in various types of peripheral neuropathies. Methods Hospitalized patients diagnosed as positive for anti-neurofascin-155 (NF155)-positive autoimmune nodopathy (AN) (NF155 + ), chronic inflammatory demyelinating polyneuropathy (CIDP), or multifocal motor neuropathy (MMN) at Huashan Hospital of Fudan University in Shanghai, China, who underwent brachial plexus MRI between October 2011 and August 2023 were consecutively recruited for this study. We also recruited participants who underwent brachial plexus MRI during this period with no history of trauma, inflammation, tumors, compression, or degenerative conditions as healthy controls. According to our self-developed semiquantitative assessment of preganglionic nerves, we assessed the bilateral preganglionic C5–C8 nerves individually and scored the enlargement degree from 0 to 4 points. Furthermore, a sum score ≥20 was defined as definite enlargement. Results A total of 122 participants were enrolled, including 28 with NF155 + , 40 with CIDP, 15 with MMN, and 39 healthy controls. In the comparison of the single-nerve scores, we found that there was a significant difference distribution among the four groups (χ 2 test; P<0.001), with the patients with NF155 + exhibiting the highest scores in each of the bilateral C5–C8 nerves. In the comparison of the sum scores, a descending tendency was observed in patients NF155 + , CIDP, and MMN, with median scores of 11, 4, and 0 points, respectively (Kruskal-Wallis test; P=0.003, P<0.001, and P=0.005, respectively for NF155 + vs . CIDP, NF155 + vs . MMN, and CIDP vs . MMN). The proportion of definite enlargement in those with NF155 + was greater than that in healthy controls (21% vs . 0%; χ 2 test; P=0.004), and the sum score at 0 points was lower in the NF155 + group than in CIDP, MMN, and healthy control groups (7% vs . 37%, 87%, and 41%, respectively; χ 2 test; P<0.001). Conclusions This semiquantitative assessment can be a valuable tool for measuring preganglionic nerve enlargement, which was found to be decreased, respectively, in those with NF155 + , CIDP, and MMN. Pre...

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