SHM Converge Scientific Abstract ePoster Gallery-Neuronal Intermediate Filament (Nif) Antibody Positive Paraneoplastic Syndrome From Ipilimumab/Nivolumab

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This poster reports a rare, severe neurologic paraneoplastic syndrome in a 64-year-old woman with metastatic neuroendocrine pancreatic carcinoma treated with one dose of ipilimumab plus nivolumab. These checkpoint inhibitors (anti-CTLA-4 and anti-PD-1) can activate immune responses against tumors but may also trigger autoimmune neurologic toxicity; the risk is higher with combination therapy.<br /><br />Within hours of treatment, the patient developed diplopia, fever, a fall, then rapidly progressive cranial nerve dysfunction and spastic weakness affecting all four limbs. Imaging showed widespread inflammatory involvement of the optic nerves, chiasm, cervical spinal cord, cauda equina, thoracic cord, and conus medullaris. CSF analysis revealed mild pleocytosis, high protein, elevated myelin basic protein, and negative infectious/cytologic studies. EEG showed nonspecific encephalopathy.<br /><br />She improved transiently with high-dose steroids, suggesting an immune-mediated process. However, she deteriorated again about 20 days later and died after transition to comfort care. Delayed CSF testing was positive for heavy- and light-chain neuronal intermediate filament (NIF) antibodies at a titer of 1:512, confirming a NIF antibody-positive paraneoplastic neurologic syndrome.<br /><br />The authors emphasize that early recognition of antibody-mediated neurologic syndromes in patients receiving immune checkpoint inhibitors is critical, because prompt diagnosis and treatment may improve outcomes. The case also illustrates the overlap between paraneoplastic autoimmunity and immune-related adverse events from checkpoint blockade.

Asset Subtitle

Neha Verma

Meta Tag

Author List Edwin Peguero, Neha Verma, Sepideh Mokhtari, Yolanda Pina

Category Clinical Vignettes

Concept Paraneoplastic Neurologic Syndrome

Concept Immune Checkpoint Inhibitor

Concept Negative Inspiratory Force

Concept Autoimmune Neurologic Toxicity

Concept Ipilimumab

Distinguished Non-Finalist

Presenter Organization Moffitt Cancer Center

Presenting Author Neha Verma

Track Adult

Keywords

paraneoplastic syndrome

neuroendocrine pancreatic carcinoma

ipilimumab

nivolumab

immune checkpoint inhibitors

neurologic toxicity

NIF antibodies

autoimmune encephalomyelitis

optic neuritis

corticosteroid treatment

Paraneoplastic Neurologic Syndrome

Immune Checkpoint Inhibitor

Negative Inspiratory Force

Autoimmune Neurologic Toxicity

Ipilimumab