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Printable Handouts
Navigable Slide Index
- Introduction
- Overview
- Inflammatory neuropathies
- Clinical spectrum
- Clinical presentation
- Spectra
- Acute inflammatory neuropathies
- Chronic inflammatory neuropathies
- Pattern in “typical” cases
- GBS versus CIDP
- Targets and mechanisms
- Potential mechanisms
- Potential effector mechanisms for nerve injury
- Methods to study or define pathological mechanisms
- Early evidence for neuropathy-associated anti-carbohydrate antibodies
- IgG antibodies to ganglioside in GBS
- Anti-ganglioside antibodies in the inflammatory neuropathies
- A true case of molecular mimicry?
- The node as a target for inflammatory neuropathy-associated antibodies
- Induced pluripotent SC derived sensory neurons
- Ganglioside antibody targeting nodal axolemma
- Calcium influx in sensory soma
- Calcium fluxes are a prelude to axonal degeneration
- Analysis of the electrophysiological effects of disialosyl antibodies and complement
- Reversible conduction failure vs. axonal degeneration
- GM1 & sulfatide antibodies may target glia directly
- Glial injury is followed by axolemmal nanoruptures, calcium influx, and secondary axonal degeneration
- Myelin proteins and T cells?
- The node of Ranvier
- Identification of specific antigens by immunoprecipitation
- Nodal/paranodal reactive antibodies in patients with inflammatory neuropathies
- Nodal/paranodal antibody syndromes: autoimmune nodopathies
- Nodal and paranodal antibody-associated neuropathies
- IgG4
- IgG4 does not activate complement
- Anti-contactin IgG4 antibodies have a (potentially) pathogenic “blocking” effect
- Anti-contactin IgG4 antibodies can disrupt nodal architecture
- Anti-neurofascin-155 IgG4 antibodies prevent paranodal complex formation in vivo
- Contrasting pathological importance of monovalent versus bivalent paranodal antibodies
- CNTN1 immune complexes drive nephropathy?
- CNTN1 positive immune complexes are found in glomeruli
- Anti-CNTNI IgG3 induces acute conduction block and motor deficits in a passive transfer rat model
- panNF antibodies activate complement and disrupt myelin, nodal, and paranodal structure
- Established and upcoming therapies
- Established therapies
- Plasma exchange
- IVIg
- Rituximab
- Treatments and their effects on neuropathies
- Autoimmune nodopathies: treatment implications
- Rituximab appears effective
- Other approaches
- Imlifidase: an approach to eliminate pathogenic IgG
- 15-HMedIdeS-09: imlifidase in GBS patients
- FcRN inhibition
- ARGX-113-1802/Adhere
- Complement inhibition with a C5 convertase blocking mAb precent MAC pore deposition
- Eculizumab maintains nerve terminal integrity and prevents functional impairment
- Complement inhibition in GBS
- ANX005
- Protection of axonal and nodal integrity via overexpression of calpastatin
- Summary
Topics Covered
- Inflammatory neuropathies
- Acute inflammatory neuropathies
- Chronic inflammatory neuropathies
- Guillain-Barré syndrome (GBS)
- Chronic inflammatory demyelinating polyradicuoloneuropathy (CIDP)
- Anti-ganglioside antibodies
- GM1 and sulfatide antibodies
- The node of Ranvier
- IgG4
- CNTN1 immune complexes
- Established and upcoming therapies
Links
Series:
- The Immune System - Key Concepts and Questions
- Periodic Reports: Advances in Clinical Interventions and Research Platforms
Categories:
Therapeutic Areas:
Talk Citation
Rinaldi, S. (2025, April 30). Immunology of the peripheral nervous system: the inflammatory neuropathies [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved January 28, 2026, from https://doi.org/10.69645/RLTQ4692.Export Citation (RIS)
Publication History
- Published on April 30, 2025
Financial Disclosures
- Dr. Rinaldi has received honoraria for lectures given at the request of Excemed, Fresenius, CSL Behring, UCB, argenx, the Beijing Association of Holistic and Integrated Medicine, and the Irish Institute of Clinical Neuroscience. He has been a paid consultant for argenx, Annexon, Dianthus, Takeda and Hansa Biopharma. He runs a not-for-profit diagnostic testing service for nodal/paranodal antibodies.
Other Talks in the Series: The Immune System - Key Concepts and Questions
Other Talks in the Series: Periodic Reports: Advances in Clinical Interventions and Research Platforms
Transcript
Please wait while the transcript is being prepared...
0:00
Hello. I'm Simon Rinaldi.
I'm an Associate
Professor of Neurology,
and the lecture today
is going to be about
immunology of the
peripheral nervous system
with a particular focus on the
inflammatory neuropathies.
0:13
In this lecture,
I'm going to cover
the clinical spectrum of the
inflammatory neuropathies,
targets and mechanisms
of these disorders
in an immunological sense and
also talk about established
and upcoming therapies.
0:25
The inflammatory neuropathies
can broadly be defined
as a range of disorders
characterised by
immune-mediated damage
to peripheral nerves.
Within this broad definition,
there are a wide range of
different possibilities,
different disorders and different
immune-mediated mechanisms.
0:47
We think about the
clinical presentation of
neuropathies in general,
and inflammatory
neuropathies, in particular,
can be associated with
a range of symptoms.
We can think of positive
sensory features,
such as tingling,
burning and pain;
and negative sensory
features such as numbness,
loss of sensation, loss of
dexterity and imbalance.
There can also be motor
involvement with loss of strength,
and the cranial nerves and
autonomic nervous system
can also be involved.
This also translates
into the range of signs
that can be seen on
clinical examination.
This includes weakness, which
can be distal or proximal,
wasting, cranial nerve
dysfunction, fasciculations,
reduced muscle tone,
suppression of
the deep tendon reflexes,
evidence of sensory loss,
sensory ataxia, and
autonomic dysfunction.
1:31
The inflammatory neuropathies
can be considered
and placed on a range
of different spectra.
For example, some are acute,
reaching nadir
within four weeks;
whereas, others have ongoing
progression and nerve injury
beyond eight weeks and will
be considered chronic.
Similarly, they can
be monophasic with
a single nadir and improvement,
or they can be relapsing, remitting,
or chronically progressive.
They may be focal,
multifocal, or generalised.
They may be pure motor, pure
sensory, or sensory-motor,
and they may affect proximal
or distal muscles, or both.
Furthermore, the pathology can
affect the myelin
sheath and cause
demyelinating neuropathies
or be primarily
targeted at the axon and
cause axonal neuropathies.