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Abstract Details

Paraneoplastic Neurologic Syndromes with ANNA-2/anti-Ri: a Contrasting Story of Two Cases
Autoimmune Neurology
P5 - Poster Session 5 (11:45 AM-12:45 PM)
6-026
ANNA-2/anti-Ri is a marker of a T-cell-mediated paraneoplastic neurologic disease with a strong cancer association that can present with opsoclonus-myoclonus syndrome, ataxia, jaw dystonia, and laryngospasm.
To highlight the clinical spectrum of paraneoplastic neurologic disease associated with antineuronal-nuclear-autoantibody-type-2 (ANNA-2/anti-Ri).
Case report of two patients at a tertiary referral center. ANNA-2 was detected by tissue immunofluorescence and western blot.

Case 1: A 61-year-old woman presented with horizontal binocular diplopia, progressive trismus, jaw spasms causing tongue biting, and trouble eating over 5 weeks. Brain MRI revealed dorsal pons and medulla T2-hyperintensity without enhancement. CSF showed no cells, protein 70 mg/dL, and 4 oligoclonal bands. ANNA-2 was present in CSF (1:128) and serum (1:7680), and invasive ductal breast carcinoma (stage 2) was found and treated with right mastectomy and chemotherapy. She developed episodic cyanosis from laryngospasm. Botox moderately helped her jaw opening dystonia, and tracheostomy was recommended for laryngospasm. She did not have improvement with steroids, intravenous immunoglobulin, rituximab, plasma exchange, or cyclophosphamide.  

Case 2:  A 51-year-old woman with a history of recurrent right breast cancer previously treated with bilateral mastectomy, chemotherapy, radiation, and hormonal treatment developed opsoclonus-myoclonus and progressive imbalance over 3 months leading to wheelchair dependence. ANNA-2 was detected in CSF (1:4096) and serum (1:15360). She received seven plasma exchange treatments and a 1-month course of high-dose oral steroids, followed by 1-month tapering. FDG-PET revealed cervical lymph node avidity presumed to be breast cancer recurrence that was treated with chemotherapy. Overall, she reported 70-80% symptom improvement and could walk independently. She was maintained on monthly plasma exchange.

These two cases highlight the clinical spectrum of ANNA-2/anti-Ri paraneoplastic neurologic disorders. While outcome is often poor with ANNA-2/anti-Ri and other antibodies targeting intracellular antigens, our second case shows that some patients can have robust response to immunotherapy and cancer treatment.
Authors/Disclosures
Nanthaya Tisavipat, MD (Mayo Clinic)
PRESENTER
Miss Tisavipat has nothing to disclose.
Bryce Chang, MD (Mayo Clinic) Mr. Chang has nothing to disclose.
Farwa Ali, MD (Mayo Clinic) Dr. Ali has nothing to disclose.
Sean J. Pittock, MD (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech, Inc.. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics, Inc.. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Prime Therapeutics. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MedImmune/Viela Bio. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB, Inc. Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman/LaRoche AG. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for F. Hofman/LaRoche. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received research support from Grifols. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Viela Bio/MedImmune/Horizon. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. The institution of Dr. Pittock has received research support from NovelMed. The institution of Dr. Pittock has received research support from AstaZeneca. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic) The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan 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. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.