CIDP with anti-neurofascin 155 (NF155) antibodies

Evidence-based neurology checklist on cidp with anti-neurofascin 155 (nf155) antibodies: Pathology NF155 are antibodies to paranodal structures They are present in 7-14% of people with CIDP HLA DRB1*15 may increase the risk of developing the antibodies Clinical pattern Clinical features Nerve…

Pathology

  • NF155 are antibodies to paranodal structures
  • They are present in 7-14% of people with CIDP
  • HLA DRB1*15 may increase the risk of developing the antibodies

Clinical pattern

Clinical features

Nerve conduction studies (NCS)

Magnetic resonance imaging (MRI)

Other investigations

Treatment

Acronym

References

  1. Kuwahara M, Suzuki H, Oka N, et al. Electron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody. Muscle Nerve 2017; doi: 10.1002/mus.25757 (Epub ahead of print).
  2. Devaux JJ, Miura Y, Fukami Y, Inoue T, Manso C, Belghazi M, Sekiguchi K, Kokubun N, Ichikawa H, Wong AH, Yuki N. Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy. Neurology 2016; 86:800-807.
  3. Querol L, Rojas-García R, Diaz-Manera J, et al. Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm 2015; 2:e149. 
  4. Koike H, Kadoya M, Kaida KI, et al. Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. JNNP 2017; 88:465-473.
  5. Doppler K, Stengel H, Appeltshauser L, et al. Neurofascin-155 IgM autoantibodies in patients with inflammatory neuropathies. JNNP 2018; pii: jnnp-2018-318170 (Epub ahead of print).
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