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Abstract Details

Clinicopathologic Correlation of Paraneoplastic Neuronal Intermediate Filament (NIF) Autoimmunity
Autoimmune Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
14-018

NIF IgG is a recently identified autoantibody that has been associated with a variety of neurological presentations including encephalopathy, cerebellar ataxia, and myelopathy. The majority of cases are associated with an underlying malignancy. To our knowledge, neuropathological examination of postmortem brain tissue in NIF-associated encephalitis has not been reported in the literature.

To describe a case of paraneoplastic neuronal intermediate filament (NIF) encephalitis associated with chronic lymphocytic leukemia (CLL), highlighting postmortem neuropathological findings.

A case report.

We present the case of a 76-year-old woman with a history of breast cancer and CLL (both in remission) who presented with several months of progressive cognitive decline, gait impairment, and prominent neuropsychiatric disturbances. Magnetic resonance imaging of the brain revealed two remote lacunar infarcts but was otherwise normal. Computed tomography imaging of the body revealed diffuse axillary, mesenteric, and retroperitoneal adenopathy concerning for CLL recurrence, and this was confirmed on peripheral flow cytometry of blood. Given the sudden onset of progressive and prominent neuropsychiatric changes without a clear etiology, a paraneoplastic evaluation was pursued. Serum autoantibody testing revealed positivity for NIF antibody by immunofluorescence assay and subsequent cell-based assay confirmed NIF heavy chain antibody at high titers (1:960). The patient was treated with a trial of high-dose intravenous methylprednisolone and plasma exchange with moderate clinical improvement. Ultimately, her cancer progressed, and she died approximately six months later. Neuropathological brain examination revealed the presence of inflammatory infiltrate in the cortex and cerebellum, and immunohistochemical analysis demonstrated reduced staining for intermediate filaments. There was also evidence of neurofibrillary tangles and neuritic plaques, findings typically seen in Alzheimer’s disease. There was no pathological evidence of CNS involvement of CLL.

Our case highlights neuropathological findings seen in paraneoplastic NIF autoimmunity and a possible association with CLL recurrence. 

Authors/Disclosures
Rumyar V. Ardakani, MD (Neuro-Infectious Diseases Group)
PRESENTER
Dr. Ardakani has nothing to disclose.
Yong Guo No disclosure on file
Eati Basal Eati Basal has nothing to disclose.
Samuel Aragon (University of Colorado Anschutz Medical Campus) No disclosure on file
Brooke Valdez (University of Colorado) Brooke Valdez has nothing to disclose.
Kelli Money, MD, PhD (UCHealth Neurology Clinic- Memorial Hospital North) Dr. Money has nothing to disclose.
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Samuel Guzman No disclosure on file
Amanda L. Piquet, MD, FAAN (University of Colorado) The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $0-$499 for serving as a Consultant for UCB. The institution of Dr. Piquet has received personal compensation in the range of $0-$499 for serving as a Consultant for EMD Serono. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Novartis. The institution of Dr. Piquet has received research support from Abbvie. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.