Abstract Details

Title Clinical and paraclinical features of non-paraneoplastic NIF-mediated disease associated with concurrent SARS-CoV-2 infection

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 059

Objective

To describe clinical and paraclinical features of non-paraneoplastic NIF-mediated disease associated with concurrent SARS-CoV-2 infection.

Background Neurologic syndromes associated with neuronal intermediate filament (NIF) immunoglobulin G (IgG) most often are characterized by encephalopathy, cerebellar ataxia, or myelopathy. NIF-IgG has been strongly correlated with the presence of an underlying malignancy, with neuroendocrine tumors being most prevalent. Despite the intracellular target of this antibody, patients with NIF-IgG mediated disease tend to improve clinically with immunotherapy. While some cases have been described in a parainfectious context, this is the first such case in the context of a SARS-CoV-2 infection.

Design/Methods NA

Results We reported a case of non-paraneoplastic NIF-mediated disease in the setting of SARS-CoV-2 infection. The patient presented with first time seizure. He was found to have frequent left temporal lobe spikes then two left temporal lobe seizures on neurotelemetry. Brain MRI displayed abnormal signal throughout the left hippocampus and mesial temporal lobe, without contrast enhancement. LP was subsequently performed. CSF showed elevated protein, 14-3-3, T-tau, interleukin 13, interleukin 2 receptor, and interleukin 6. The meningitis/encephalitis panel, and HSV-1/2 IgG were negative. Serum autoimmune encephalitis panel revealed a high-positive titer for anti-NIF 1:960, with concurrent NIF heavy chain cell-based assay positive. He improved with three days of IV steroids and treatment with levetiracetam and lacosamide. He has since been seizure free.

Conclusions NIF-mediated diseases usually present with encephalopathy, cerebellar ataxia, or myelopathy and are generally seen in the setting of malignancy. Our case illustrated an example of NIF-mediated disease presenting as seizure in the setting of infection. This highlights the importance of consideration of parainfectious autoimmunity.

Authors/Disclosures
Stefanie J. Rodenbeck, MD (Indiana University) PRESENTER	Dr. Rodenbeck has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen.
Lauren Schmidt, MD	Dr. Schmidt has nothing to disclose.
Jon A. Karel, MD (Indiana University School of Medicine)	Dr. Karel has nothing to disclose.

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