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Published on: October 6, 2023
Isolated Peripheral Nervous System Presentation in Ma/Ma2-Associated Autoimmunity
Andreu Vilaseca1,2, Macarena Villagrán-García3,4, Antonio Farina3,4
1Center for Multiple Sclerosis and Autoimmune Neurology, Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN.
Ma/Ma2 antibodies rarely cause isolated peripheral nervous system (PNS) autoimmunity. This study found PNS syndromes like sensory neuronopathy in 3% of patients, often linked to cancer.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Ma/Ma2-associated neurologic autoimmunity typically affects the central nervous system (CNS).
- Isolated peripheral nervous system (PNS) involvement is infrequently reported in Ma/Ma2 autoimmunity.
Purpose of the Study:
- To determine the frequency of isolated PNS presentations in patients with Ma/Ma2 antibodies.
- To describe the clinical syndromes associated with isolated PNS involvement in Ma/Ma2 autoimmunity.
Main Methods:
- A nested case series design was employed within multicenter cohorts.
- Patients with confirmed Ma/Ma2 antibodies (tissue-based assay and line-blot) and isolated PNS involvement were included.
Main Results:
- Seven out of 212 patients (3%) exhibited isolated PNS involvement.
- Reported PNS syndromes included sensory neuronopathy, myeloradiculopathy, radiculoplexopathy, motor neuronopathy, and multiple mononeuropathy.
- Cancer was identified in 86% of affected patients, with various types including mesothelioma, lung, and breast cancer.
Conclusions:
- Isolated PNS involvement is a rare but recognized manifestation of Ma/Ma2-associated autoimmunity.
- Consider Ma/Ma2 antibody testing for patients presenting with neuronopathies and unexplained non-length-dependent neuropathies.
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