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Updated: Aug 31, 2025

The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

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Published on: December 26, 2014

Autoimmune neuromyotonia.

Louis Comperat¹, Antoine Pegat^1,2, Jérôme Honnorat^1,3

¹French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon.

Current Opinion in Neurology

|August 22, 2022

Summary

This summary is machine-generated.

Autoimmune neuromyotonia, often linked to anti-CASPR2 antibodies, causes peripheral nerve hyperexcitability. Immune treatments show effectiveness in managing this rare neurological disorder.

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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4

Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4

Published on: August 21, 2017

Area of Science:

Neurology
Immunology
Neuroimmunology

Background:

Autoimmune neuromyotonia is a rare group of immune-mediated neurological disorders.
It is frequently associated with anti-contactin-associated protein-like 2 (CASPR2) antibodies.
Clinical and electrical signs of peripheral nerve hyperexcitability (PNH) are characteristic features.

Purpose of the Study:

To summarize current knowledge on immune-mediated neuromyotonia.
Focus on clinical presentations, pathophysiology, and management strategies.
Highlight the role of anti-CASPR2 antibodies in the disease.

Main Methods:

Review of clinical evidence and experimental findings.
Analysis of diagnostic criteria for autoimmune neuromyotonia.
Evaluation of treatment responses to immune-active therapies.

Main Results:

Neuromyotonia, a form of PNH, is a key feature in syndromes associated with anti-CASPR2 antibodies.
Syndromes include cramp-fasciculation, Isaacs, Morvan, and autoimmune limbic encephalitis.
Anti-CASPR2 antibodies are suggested to be directly pathogenic.

Conclusions:

Diagnosis requires identifying PNH signs, anti-CASPR2 antibodies, and specific electrodiagnostic abnormalities.
Paraneoplastic associations, particularly thymoma in Morvan syndrome, are possible.
Patients generally respond well to immune-active treatments such as steroids, IVIg, plasma exchange, and rituximab.