Highlights
- •Electron microscopic observations suggest that both patients present lesions in favor of a paranodopathy.
- •Some paranodes seem to be associated with macrophages.
- •These lesions are in favor of a complement-mediated dysfunction/disruption of the nodal region.
- •This may be due to antibodies against gangliosides which are mainly located at the level of the paranode (axolemma).
- •Miller Fisher syndrome and CANDA should be classified as paranodopathies.
Abstract
Objective
To study the pathological characteristics of acute and chronic ataxic peripheral neuropathy
at the level of the node of Ranvier.
Study design and setting
We performed the pathological study (nerve biopsy of a sural nerve) of two patients,
one with an acute form of ataxic peripheral neuropathy called ‘Miller Fisher syndrome’
(MFS), the other one with a chronic form called ‘chronic ataxic neuropathies with
disialosyl antibodies’ (CANDA).
Results
A dysimmune process involving peripheral nerves commences in myelin, at the internodal
area or/and in the paranodal and nodal regions. Our electron microscopic observations
suggest that both patients present lesions in favor of a paranodopathy.
Conclusion
Many of the immune-mediated peripheral neuropathies are now classified as nodoparanodopathies.
This subtype of auto-immune neuropathy may present various clinical phenotypes such
as ‘Acute Motor Axonal Neuropathy’ (AMAN), ‘Acute Motor and Sensory Neuropathy’ (AMSAN)
or ‘chronic inflammatory demyelinating polyradiculoneuropathy’ (CIDP), and are associated
with anti-disialosyl antibodies. In our two cases, some paranodes seem to be associated
with macrophages and we hypothesized that these lesions are in favor of a complement-mediated
dysfunction/disruption of the nodal region due to disialosyl antibodies against gangliosides
which are mainly located at the level of the axolemma of the paranode.
Keywords
Abbreviations:
AIDP (acute inflammatory demyelinating polyradiculoneuropathy), AMAN (Acute Motor Axonal Neuropathy), AMSAN (Acute Motor and Sensory Neuropathy), CANDA (chronic ataxic neuropathies with disialosyl antibodies), CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein), CIDP (chronic inflammatory demyelinating polyradiculoneuropathy), EM (electron microscopy), ENMG (electroneuromyogram), GBS (Guillain-Barré syndrome), IV (index value), MAC (membrane attack complex), MFS (Miller Fisher syndrome), MGUS (Monoclonal Gammopathy of Undetermined Significance)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: May 10, 2022
Accepted:
May 6,
2022
Received in revised form:
May 4,
2022
Received:
April 8,
2022
Identification
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