Automimmune Nodo-paranodopathies

1. Autoimmune
Nodo-paranodopathies
Ade Wijaya, MD – June 2025

2. All autoimmune
neuropathies in which the
dysfunction/disruption of
the nodal region
Uncini A, Susuki K, Yuki N. Nodo-paranodopathies: beyond the
demyelinating and axonal classification in anti-ganglioside
antibodymediated neuropathies. Clin Neurophysiol. 2013;48:1928-1934.
Uncini A, Kuwabara S. Nodopathies of the peripheral nerve: an emerging
concept. J Neurol Neurosurg Psychiatry. 2015;86:1186- 1195. Uncini A,
Vallat JM. Autoimmune nodo-paranodopathies of peripheral nerve: the
concept is gaining ground. J Neurol Neurosurg Psychiatry. 2018;89:627-635

3. 3 Presentation title
Clinical presentation
20XX
• Similar to GBS / CIDP with ‘atypical’ features:
Aggressive onset and severe disease course, cranial
neuropathies, tremor, pronounced sensory ataxia, respiratory
and autonomic insufficiency, and little or no response to IVIg
Johnson CB, Fehmi J, Rinaldi S. The immunology and neuropathology of the autoimmune nodopathies. Journal of Neuroimmunology. 2025 Jun 9:578665.

4. 4 Presentation title 20XX
Johnson CB, Fehmi J, Rinaldi S. The immunology and neuropathology of the autoimmune nodopathies. Journal of Neuroimmunology. 2025 Jun 9:578665.

5. Proteinuria is a possible biomarker of
autoimmune nodopathies associated with
autoantibodies targeting CNTN1 or NF155.
Urinalysis results should be carefully
checked for quick differentiation of
autoimmune nodopathies from CIDP.
Patients who present with nephrotic
syndrome should be tested for anti-CNTN1
IgG4 antibodies, and patients who exhibit
mild proteinuria should be tested for anti-
NF155 IgG4 antibodies
“
”
5 Presentation title 20XX
Funakoshi K, Kokubun N, Suzuki K, Yuki N. Proteinuria is a key to suspect autoimmune nodopathies. European Journal of Neurology. 2024 Oct;31(10):e16406.

6. 6 Presentation title
Neurophysiology
• Conduction block without temporal dispersion, ‘axonal
conduction block’
• Reversible conduction failure
• Conduction block may be more common in AN than in CIDP,
particularly at proximal sites
20XX
Funakoshi K, Kokubun N, Suzuki K, Yuki N. Proteinuria is a key to suspect autoimmune nodopathies. European Journal of Neurology. 2024 Oct;31(10):e16406.

7. 7 Presentation title
Pathological Features
• Skin biopsies: lengthening of the node and loss of paranodal
NF155/Caspr1 staining
• Ultrastructural studies: dehiscence of the paranodal myelin
loops from the underlying axolemma, and the absence of
macrophage mediated stripping of internodal myelin
20XX
Cortese A, Lombardi R, Briani C, Callegari I, Benedetti L, Manganelli F, Luigetti M, Ferrari S, Clerici AM, Marfia GA, Rigamonti A. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP:
clinical relevance of IgG isotype. Neurology: Neuroimmunology & Neuroinflammation. 2019 Nov 21;7(1):e639.
Koike H, Kadoya M, Kaida KI, Ikeda S, Kawagashira Y, Iijima M, Kato D, Ogata H, Yamasaki R, Matsukawa N, Kira JI. Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-
neurofascin-155 and anti-contactin-1 antibodies. Journal of Neurology, Neurosurgery & Psychiatry. 2017 Jun 1;88(6):465-73.

8. 8 Presentation title
Radiology
• MRI : nerve root enlargement or enhancement
20XX
Liu B, Zhou L, Sun C, Wang L, Zheng Y, Hu B, Qiao K, Zhao C, Lu J, Lin J. Clinical profile of autoimmune nodopathy with anti neurofascin 186 antibody. Annals of Clinical and Translational Neurology. 2023
‐
Jun;10(6):944-52.

9. 9
Summary
Autoimmune nodo-paranodopathies are
autoimmune neuropathies affectiong nodal
regions.
Similar presentation with CIDP with more
aggressive course and less responsive to IVIG.
Proteinuria, electrodiagnostic testing and
ultimately antibody testing could help to
differentiate with CIDP.
Rituximab is effective, plasma exchange may be
effective

10. Thank you
Click icon to add picture