ArticleJournal of neurology2019
Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome.
Article in Journal of neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it, 40 citations in OpenAlex.
- Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.Neurology(R) neuroimmunology & neuroinflammation · 2020Pooled it
- Paraneoplastic Intracellular Neuropathies: A Narrative Review.Neurology and therapy · 2026Review
- Isolated Peripheral Nervous System Presentation in Ma/Ma2-Associated Autoimmunity.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- Current and Future Biomarkers in the Diagnosis of Autoimmune Encephalitis: A Review of Biomarker Detection Techniques and Their Performance.Medicina (Kaunas, Lithuania) · 2026Review
- Paraneoplastic Brachial Amyotrophic Diplegia and Encephalitis With Ma2-IgG During Immune Checkpoint Inhibitor Use for Mesothelioma: A Case Report.JTO clinical and research reports · 2026Article
- Alternative diagnoses of suspected paraneoplastic neurological syndromes: a population-based study.Journal of neurology · 2026Article
- Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.Brain communications · 2026Article
- Paraneoplastic Brachial Amyotrophic Diplegia With Favorable Outcome and Anti-Ank3 Antibodies: A Case Report.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Neurodegeneration and the immune system: lessons from autoimmune encephalitis.Journal of neurology · 2025Review
- Paraneoplastic Calmodulin Kinase-Like Vesicle-Associated Protein (CAMKV) Autoimmune Encephalitis.Annals of neurology · 2024Article
- Autoantibodies in neuromuscular disorders: a review of their utility in clinical practice.Frontiers in neurology · 2024Review
- MR Imaging Findings in a Large Population of Autoimmune Encephalitis.AJNR. American journal of neuroradiology · 2023Article
- Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment.Biomedicines · 2023Review
- Identification of SKOR2 IgG as a novel biomarker of paraneoplastic neurologic syndrome.Frontiers in immunology · 2023Article
- Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians.Biomedicines · 2022Review
- Paraneoplastic neurological syndrome: growing spectrum and relevance.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Review
- How to diagnose and manage neurological toxicities of immune checkpoint inhibitors: an update.Journal of neurology · 2022Article
- Paraneoplastic neurological syndromes associated with non-Hodgkin lymphoma: a case series.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
- Neurologic Toxicity of Immune Checkpoint Inhibitors: A Review of Literature.Frontiers in pharmacology · 2022Review
- Anti-Ma2 Antibody-Associated Paraneoplastic Neurological Syndromes: A Pilot Study.Brain sciences · 2021Article
Corrections and comments
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Authors and funding
9 authors at 6 institutions in 2 countries.
Funding
Abstract
objectiveTo present clinical, radiological, and pathological features of a cohort of patients with motor neuron involvement in association with anti-Ma2 antibodies (Ma2-Ab).
methodsRetrospective case-series of patients with definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the literature.
resultsAmong 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identified three patients (9.1%) with a motor neuron syndrome (MNS). Seven additional cases were retrieved among the 75 Ma2-patients reported in the literature (9.3%). A total of ten patients are, therefore, described herein. MNS was evident as combined upper and lower MNS in four patients, isolated upper MNS in two, and isolated lower MNS in one; three patients were diagnosed with myeloradiculopathy. The most common MNS signs/symptoms were: hyperreflexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyramidal tract T2-weighted/FLAIR hyperintensities (three patients). Spine MRI found bilateral, symmetric, T2-weighted signal abnormalities in the anterior horn in two patients. CSF examination was abnormal in nine patients. Cancer was found in seven patients (four testicular, two lung, and one mesothelioma). Eight patients underwent first-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identified in the literature. Motor improvement was observed in 33% of our patients, and 20% in the literature series.
conclusionsMotor neuron involvement could complicate Ma2-Ab-associated PNS in almost 10% of patients and must be carefully studied to adapt treatment. This disorder differs from amyotrophic lateral sclerosis.
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