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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.
Objectives:
Ma/Ma2-associated neurologic autoimmunity is characterized by CNS involvement. However, isolated peripheral nervous system (PNS) presentations have been rarely reported. We aimed to describe the frequency and syndromes of patients with Ma/Ma2 antibodies and isolated PNS involvement.
Methods:
We performed a nested case series within multicenter cohorts of patients with Ma/Ma2-associated neurologic syndromes, confirmed by tissue-based assay and line-blot. Patients with isolated PNS presentations were included.
Results:
Among 212 patients with Ma/Ma2 antibody-associated neurologic syndromes, 7 (3%) presented with isolated PNS involvement (median age, 68 years; 4/7 female). PNS syndromes included sensory neuronopathy (3 patients), myeloradiculopathy (1), radiculoplexopathy (1), motor neuronopathy (1), and multiple mononeuropathy. All patients were anti-Ma2-positive, whereas Ma antibodies (reactive against Ma1 and Ma2 proteins) were detected in 2 (33%)/6 tested patients. An associated cancer was identified in 6 (86%)/7 patients: pleural mesothelioma; oral squamous cell; testicular, lung, and breast cancer; and B-cell lymphoma. Five patients received immunotherapy, cancer treatment, or both. After a median follow-up of 23 months, symptoms improved or stabilized in 3 patients and progressed in 4.
Discussion:
Isolated PNS involvement is a rare manifestation of Ma/Ma2 associated autoimmunity. Ma/Ma2 antibody testing should be considered in neuronopathies and unexplained non-length-dependent neuropathies.
Insights
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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