A case with Neurofascin-155 IgG antibody-associated combined central and peripheral demyelination: Successfully treated with anti-CD20 monoclonal antibody

Sokmen, Okan; Demırci, Mehmet; Tan, Ersin

doi:10.1016/j.clineuro.2021.106961

Cited by 7 publications

(6 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…One case of facial diplegia has been reported. 6 Only one of these cases was treated with rituximab, 7 as another recovered with IV corticosteroids and azathioprine, 8 and another with IV PLEX alone. 9 Interestingly, there are several cases of NF-155 antibody–positive patients, often with much milder presentations, who recovered with combined treatment with prednisone, IVIg, and/or PLEX.…”

Section: Discussionmentioning

confidence: 99%

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

Jamall,

Baker,

Peterson

et al. 2023

Journal of Clinical Neuromuscular Disease

View full text Add to dashboard Cite

Objectives: Anti–neurofascin-155 IgG4 (NF-155) antibody disease has previously been associated with a subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy. We report a case of NF-155 positive polyneuropathy that initially presented as an acute inflammatory demyelinating polyradiculoneuropathy. The patient responded appropriately to treatment but subsequently progressed over a 3-month period, resulting in quadriplegia, areflexia, and oculobulbar paralysis. Methods: Case report and literature review. Results: A 40-year-old male presented with acute bilateral arm and thigh weakness, areflexia, and distal sensory loss. Treatment with intravenous immunoglobulin (IVIg) for acute acquired demyelinating neuropathy resulted in initial improvement but subsequent decline. Lack of response to additional IVIg and plasmapheresis (PLEX) prompted testing for NF-155. Treatment with rituximab and steroids resulted in virtually complete recovery. Conclusions: Early testing for nodal and paranodal proteins is indicated in patients who present with acute acquired demyelinating neuropathy but fail to respond to conventional treatments, such as IVIg or PLEX. Identification of nodal and paranodal antibodies should prompt treatment with rituximab and steroids to increase likelihood of recovery.

show abstract

Section: Discussionmentioning

confidence: 99%

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

Jamall,

Baker,

Peterson

et al. 2023

Journal of Clinical Neuromuscular Disease

View full text Add to dashboard Cite

show abstract

“…Neurofascin (NF) is a cell surface protein involved in forming nerve bundles, which plays an important role in the formation and stability of Ranvier's nodes. NF can interfere with the conduction of nerve impulses and may be involved in the pathophysiological process of demyelinating diseases such as MS, CIDP and CCPD [17][18][19].…”

Section: Neuroscience An Interesting Case Of Combined Central and Per...mentioning

confidence: 99%

“…Under CCPD, demyelinating changes occur simultaneously or successively in the peripheral nervous system and the central nervous system, which is rare in the clinic. CCPD is a pedigree disease and cannot be considered a simple combination of MS and CIDP [10][11][12][13][14][15]. At present, there is no unified diagnostic standard for CCPD.…”

Section: Introductionmentioning

confidence: 99%

Untitled

2022

J. Multiscale Neurosci.

View full text Add to dashboard Cite

We report the diagnostic process of a rare patient with a combined central and peripheral demyelinating disease with antibodies against paranodal protein neurofascin 186 (anti-NF186)-the patient presented with weakness in both lower limbs. The clinical process and evidence are similar to subacute combined degeneration. However, thoracic spinal MRI shows T1 equal signal, and T2 high signal lesions in thoracic vertebrae 5-7 mainly involve white matter. The Aquaporin 4 antibody of the central nervous system indicates the disease is of an optic neuromyelitis pedigree. The antibodies against paranodal protein neurofascin 186 indicate demyelinating peripheral nerve pathogenesis. The final diagnosis was a combined central and peripheral demyelinating disease based on the results of various examinations. The patient's condition improved after methylprednisolone therapy and intravenous immunoglobulin.

show abstract

“…Therapies like rituximab, plasma exchange (PE), and immunoadsorption (IA) have been effective in treating NF155+ AN patients ( 2 , 8 – 10 ). However, recurrent symptoms and poor response in some cases emphasize the need for more targeted interventions.…”

Section: Introductionmentioning

confidence: 99%

Case Report: Telitacicept in treating a patient with NF155+ autoimmune nodopathy: a successful attempt to manage recurrent elevated sero-anti-NF155 antibodies

Ren,

Chen,

Yang

2023

Front. Immunol.

View full text Add to dashboard Cite

This report presents a case of a neurofascin-155 (NF155)+ autoimmune nodopathy (AN) patient who exhibited resistance to conventional treatments but responded positively to telitacicept therapy. Telitacicept, a dual inhibitor of B lymphocyte stimulator (BLyS) and A proliferation-inducing ligand (APRIL), suppressed the development and survival of plasma cells and mature B cells. The patient’s unique clinical features were consistent with NF155+ AN, showing limited response to standard treatments like rituximab and a recurrent significant increase in anti-NF155 antibody titers. Administering telitacicept (160mg, ih) led to an improvement in clinical symptoms, inflammatory neuropathy cause and treatment (INCAT) scale and inflammatory Rasch-built overall disability scale (I-RODS), and stabilized anti-NF155 antibody levels without a rebound. This case demonstrates telitacicept as a potential novel therapy for NF155+ AN, particularly when conventional treatments fail. Further investigation into its safety, efficacy, dosage, and treatment cycle in NF155+ AN is warranted.

show abstract

A case with Neurofascin-155 IgG antibody-associated combined central and peripheral demyelination: Successfully treated with anti-CD20 monoclonal antibody

Cited by 7 publications

References 4 publications

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

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

Untitled

Case Report: Telitacicept in treating a patient with NF155+ autoimmune nodopathy: a successful attempt to manage recurrent elevated sero-anti-NF155 antibodies

Contact Info

Product

Resources

About