Abstract Details

Title A Case of Septin-7 Antibody-associated Encephalitis Associated with Seizures and Squamous Cell Lung Carcinoma

Topic Autoimmune Neurology

Presentation(s) P5 - Poster Session 5 (5:30 PM-6:30 PM)

Poster/Presentation
Number 14-006

Objective To report a case of septin-7 antibody-associated encephalitis associated with seizures and squamous cell lung carcinoma.

Background Septin-7 IgG is a recently identified autoantibody with fewer than 20 total cases reported in the literature. This autoantibody has been associated with encephalopathy, myelopathy, cerebellar ataxia, and psychiatric changes. Associated malignancy has been reported in a minority of cases, specifically breast adenocarcinoma, non-Hodgkin lymphoma, carcinoid tumor, and myelodysplastic syndrome. To our knowledge, squamous cell lung carcinoma has not been reported in association with septin-7 autoimmunity.

Design/Methods We present a case of a 77-year-old woman with a history of recurrent left parieto-occipital intraparenchymal hemorrhage secondary to cirrhosis who presented with refractory focal onset seizures. Her seizure semiology consisted of behavioral arrest, yawning, and vomiting. Her seizures were initially attributed to her prior intraparenchymal hemorrhage and lowered seizure threshold from a urinary tract infection. However, her electroencephalography demonstrated bi-temporal epileptiform discharges and her seizures were refractory to multiple anti-epileptic agents. She also developed progressive encephalopathy and new neuropsychiatric symptoms of paranoia and agitation. Additional work-up revealed the presence of a cavitary lung mass.

Results Further evaluation revealed positivity for septin-7 IgG at high titers in both serum (1:30720) and cerebrospinal fluid (1:64) at Mayo Clinic Laboratory. A biopsy of the cavitary lung lesion was consistent with squamous cell carcinoma of the lung. High-dose methylprednisolone and intravenous immunoglobulin were initiated with moderate improvement in mental status and seizure frequency. Ultimately, the family decided to transition to hospice, and she passed away five weeks after her diagnosis.

Conclusions The full clinical and paraneoplastic spectrum of septin-7 IgG-associated central nervous system disorders is not yet fully defined. Our case highlights that presence of septin-7 IgG may be associated with squamous cell lung carcinoma, and seizures may be within the clinical phenotype.

Authors/Disclosures
Mallory Lowe, MD PRESENTER	Dr. Lowe has received research support from Kyverna Therapeutics.
Rumyar V. Ardakani, MD	Dr. Ardakani has nothing to disclose.
Barrie L. Zerwic, MD (University of Colorado, Anschutz Medical Campus)	Dr. Schmitt has nothing to disclose.
Paul D. Crane, MD (University of Colorado)	Dr. Crane has nothing to disclose.
Elizabeth Matthews, MD	Dr. Matthews has nothing to disclose.
Daniel Pastula, MD, MHS, FAAN (University of Colorado, Department of Neurology)	Dr. Pastula has nothing to disclose.
Amanda L. Piquet, MD, FAAN (University of Colorado)	The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. 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 $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet 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. 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. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. 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. The institution of Dr. Piquet has received research support from Kyverna . 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.

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