Growing Spectrum of Autoimmune Nodopathies

Gupta, Pranjal; Mirman, Igal; Shelly, Shahar; Dubey, Divyanshu

doi:10.1007/s11910-023-01264-4

Cited by 11 publications

(9 citation statements)

References 75 publications

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“…Even if the response to any specific medication cannot be proven due to the lack of controlled clinical trials, early autoantibody-depleting treatment is strongly recommended based on pathophysiology and experiences from reported cases, to shorten disease duration, prevent axonal damage, and improve the outcome [6 ▪▪ ,44]. We propose the following treatment algorithm for adults: diagnose early, use plasma exchange as bridging treatment in patients with IgG4 autoantibodies, and plasma exchange or IVIg in patients with IgG1-3 autoantibodies, initiate antibody-depleting treatment early, in case of ongoing refractory, life-threatening course, or additional hemato-oncological disease, consider additional chemotherapy used for plasmocytoma in an individual risk/benefit analysis.…”

Section: Treatment Course and Prognosismentioning

confidence: 99%

“…The antibodies directly attack the node of Ranvier, leading to a disruption of the physiological architecture with paranodal dysjunction and conduction deficits [2,3,4]. A separate classification of these conditions was first proposed by Uncini et al [5] for neuropathies with antiganglioside antibodies, and later extended to antibodies against specific cell adhesion molecules at the node of Ranvier [2]: The most common paranodal antibodies target Neurofascin-155 (NF155) and Contactin-1, are of the IgG4 subclass and occur in cohorts previously classified as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with a frequency of 1–20%, with variations depending on the geographical region [2,6 ▪▪ ]. Less frequent targets are Contactin-1 associated protein 1 (Caspr1) and nodal targets [6 ▪▪ ,7–10].…”

Section: Introductionmentioning

confidence: 99%

“…A separate classification of these conditions was first proposed by Uncini et al [5] for neuropathies with antiganglioside antibodies, and later extended to antibodies against specific cell adhesion molecules at the node of Ranvier [2]: The most common paranodal antibodies target Neurofascin-155 (NF155) and Contactin-1, are of the IgG4 subclass and occur in cohorts previously classified as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with a frequency of 1–20%, with variations depending on the geographical region [2,6 ▪▪ ]. Less frequent targets are Contactin-1 associated protein 1 (Caspr1) and nodal targets [6 ▪▪ ,7–10]. Seropositive patients present with distinct symptoms such as acute onset, moderate to severe sensorimotor involvement, and additional features such as severe sensory ataxia, tremor, neuropathic pain, or concomitant glomerulonephritis in case of contactin-1 [2,6 ▪▪ ,11 ▪ ,12].…”

Section: Introductionmentioning

confidence: 99%

“…Less frequent targets are Contactin-1 associated protein 1 (Caspr1) and nodal targets [6 ▪▪ ,7–10]. Seropositive patients present with distinct symptoms such as acute onset, moderate to severe sensorimotor involvement, and additional features such as severe sensory ataxia, tremor, neuropathic pain, or concomitant glomerulonephritis in case of contactin-1 [2,6 ▪▪ ,11 ▪ ,12]. In contrast, antibodies against nodal NF186 were reported with heterogenous clinical presentation, from immune-mediated neuropathies to central nervous system disease [9,13–17].…”

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: Treatment Course and Prognosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Appeltshauser,

Doppler

2023

Current Opinion in Neurology

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“…In 2021, the most recent guidelines on the diagnosis and treatment of CIDP named this type of neuropathy as autoimmune nodopathy (AN) and recognized it as a relatively separate category of disease entity ( 2 , 4 ). Pathogenicity has been demonstrated for these antibodies, which have been identified as specific biomarkers for the diagnosis and treatment guidance of AN ( 5 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

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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Periphere neuroimmunologische Erkrankungen – neuropathologische Einsichten und klinische Perspektiven

Hoffmann,

Holzer,

Preuße

et al. 2024

Nervenarzt

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Growing Spectrum of Autoimmune Nodopathies

Cited by 11 publications

References 75 publications

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

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

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

Periphere neuroimmunologische Erkrankungen – neuropathologische Einsichten und klinische Perspektiven

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