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Published on: August 9, 2019
Caspr2 antibodies in patients with thymomas
Angela Vincent1, Sarosh R Irani
1Department of Clinical Neurology, John Radcliffe Hospital, Oxford, United Kingdom. angela.vincent@imm.ox.ac.uk
Summary
Thymomas can be linked to rare autoimmune neurological disorders affecting the central and peripheral nervous systems. Autoantibodies targeting contactin-2 associated protein (Caspr2) are common in patients with thymic malignancies and these conditions.
Area of Science:
- Neuroimmunology
- Oncology
- Neurology
Background:
- Thymomas are associated with myasthenia gravis and other autoimmune disorders.
- Some patients with thymic tumors develop central nervous system (CNS) disorders, including acquired neuromyotonia, Morvan disease, and limbic encephalitis.
- These conditions involve peripheral nerves, CNS, or both, and are often linked to voltage-gated potassium channel autoantibodies.
Purpose of the Study:
- To investigate the target antigens of autoantibodies in patients with thymomas and associated neurological disorders.
- To explore the role of contactin-2 associated protein (Caspr2) in these conditions.
Main Methods:
- Review of recent studies on patients with thymomas and neurological conditions.
- Analysis of autoantibody targets in affected individuals.
Main Results:
- Voltage-gated potassium channel autoantibodies are not always directed against potassium channels.
- In some patients, these autoantibodies target contactin-2 associated protein (Caspr2), which is complexed with potassium channels.
- Caspr2 autoantibodies are prevalent in patients with thymic malignancies presenting with these neurological disorders.
Conclusions:
- Caspr2 is a significant autoantigen in a subset of patients with thymomas and associated neurological syndromes.
- Identification of Caspr2 as a target provides insights into the pathogenesis of these rare disorders.
- Treatment strategies may involve addressing the thymoma and targeting the autoimmune response against Caspr2.
