Paraneoplastic neuromyelitis optica spectrum disorders: a case series

Area of Science:

• Neurology
• Immunology
• Oncology

Background:

• Neuromyelitis optica spectrum disorders (NMOSD) are rare autoimmune diseases primarily affecting the optic nerves and spinal cord.
• A small percentage of NMOSD cases are associated with paraneoplastic (PN) syndromes, often linked to malignancies or benign tumors.
• Current treatment strategies for paraneoplastic NMOSD (PNNMOSD) lack consensus, with options including surgery and immunosuppression.

Purpose of the Study:

• To highlight the importance of considering paraneoplastic etiology in all new NMOSD diagnoses.
• To present three diverse cases of PNNMOSD, illustrating variations in demographics, clinical features, tumor associations, treatments, and outcomes.
• To raise clinician awareness regarding PNNMOSD and the significance of recognizing associated tumor symptoms.

Main Methods:

• Retrospective analysis of three newly diagnosed PNNMOSD cases.
• Review of patient demographics, clinical presentations, associated tumors, treatment regimens, and outcomes.
• Comparison of case features to identify variations and commonalities.

Main Results:

• The three PNNMOSD cases exhibited distinct demographic and clinical characteristics.
• Tumor associations varied, including malignancies and benign conditions.
• Treatment approaches and patient outcomes differed significantly among the cases.
• PNNMOSD can occur in patients younger than 50 and present with varied lesion patterns beyond spinal cord or brainstem.

Conclusions:

• A paraneoplastic etiology should be suspected in all new NMOSD diagnoses, irrespective of patient age or lesion location.
• Early identification of tumor-related symptoms is critical for diagnosing and managing PNNMOSD.
• Further research is needed to establish standardized treatment protocols for PNNMOSD.