About this Research Topic
The spectrum of immune-mediated neuropathies is broad, and the different subtypes are still under investigation. Although adherence to the EFNS/PNS guidelines for chronic inflammatory demyelinating polyneuropathy (CIDP) significantly minimises the percentage of misdiagnoses, there remains a small number of patients who meet the criteria for CIDP but may have an alternative diagnosis ('true mimics of CIDP'), such as CANOMAD (chronic atactic neuropathy, ophthalmoplegia, immunoglobulin M [IgM] paraprotein, cold agglutinins, and disialosyl antibodies), POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) and neurolymphomatosis.
The discovery of specific pathomechanisms, including novel target antigens, in a heterogeneous group of immune-mediated neuropathies is of great importance for diagnosis and appropriate treatment. The identification of pivotal particulars of cell adhesion molecules, which form a complex with other adhesion molecules, ion channels, and the cytoskeleton to form nodal and paranodal compartments, has changed the understanding of autoimmune nodopathies (AN). Findings regarding the initial axon segment and the paranodal area may also have implications for the classification of individual-acquired neuropathies.
The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroup of treatment-naive motor neuropathies, including multifocal CIDP, which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features.
Molecular and electrophysiological diagnostic methods are still being investigated to diagnose specific polyneuropathies. The non-availability of multipurpose biological biomarkers, difficulties of electrodiagnosis, and inconclusive MRI of cervical/lumbar nerve roots and brachial/lumbar plexus or nerve ultrasound extend the duration of diagnosis and inclusion of appropriate treatment. Nerve biopsy in patients with immune-mediated neuropathy can identify endoneurial mononuclear cell infiltration, macrophage-associated demyelination, emyelinated and to a lesser extent remyelinated nerve fibers, onion bulb formation, loss of transverse bands or paranodal loop detachment. However, whether the inclusion or absence of nerve biopsy lesions impacts diagnostic precision and patient outcomes.
The Research Topic aims to attempt to systematize the pathomechanism, diagnosis, and treatment of patients with immune-mediated neuropathy.
The discovery of specific pathomechanisms, including novel target antigens, in a heterogeneous group of immune-mediated neuropathies is of great importance for diagnosis and appropriate treatment. The identification of pivotal particulars of cell adhesion molecules, which form a complex with other adhesion molecules, ion channels, and the cytoskeleton to form nodal and paranodal compartments, has changed the understanding of autoimmune nodopathies (AN). Findings regarding the initial axon segment and the paranodal area may also have implications for the classification of individual-acquired neuropathies.
The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroup of treatment-naive motor neuropathies, including multifocal CIDP, which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features.
Molecular and electrophysiological diagnostic methods are still being investigated to diagnose specific polyneuropathies. The non-availability of multipurpose biological biomarkers, difficulties of electrodiagnosis, and inconclusive MRI of cervical/lumbar nerve roots and brachial/lumbar plexus or nerve ultrasound extend the duration of diagnosis and inclusion of appropriate treatment. Nerve biopsy in patients with immune-mediated neuropathy can identify endoneurial mononuclear cell infiltration, macrophage-associated demyelination, emyelinated and to a lesser extent remyelinated nerve fibers, onion bulb formation, loss of transverse bands or paranodal loop detachment. However, whether the inclusion or absence of nerve biopsy lesions impacts diagnostic precision and patient outcomes.
The Research Topic aims to attempt to systematize the pathomechanism, diagnosis, and treatment of patients with immune-mediated neuropathy.
Keywords: immune-mediated neuropathies, acute and chronic inflammatory demyelinating polyneuropathy, autoimmune nodopathies, nodo-paranodopathy, acute and chronic inflammatory axonal polyneuropathy
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