Abstract Details

Title ANNA-2 Autoantibody Syndrome Associated with Remote Cervical Carcinoma and Good Response to Immunomodulatory Therapy

Topic Neuro-oncology

Presentation(s) P01 - (-)

Poster/Presentation
Number 110

Objective

Background ANNA-2 (anti-Ri) is a rare autoantibody first described in association with opsoclonus-myoclonus and breast carcinoma. It most commonly presents with a multifocal syndrome involving the brainstem, spinal cord and cerebellum in association with cancers of the breast and lung; it has rarely been described with cervical carcinoma.

Design/Methods Our patient was a 56 year-old female who initially presented with tinnitus, dysarthria, leg cramping, myoclonus most prominent at night and with startle, and trouble with fine motor tasks. Her medical history was significant for a remote history of cervical cancer 15 years prior to presentation which was treated with hysterectomy and unilateral oophorectomy with no recurrence. Exam was significant for emotional lability, nystagmus, myoclonic jerks, increased lower extremity tone, axial rigidity, and ataxia on gait exam. Serologic studies showed an elevated ANNA-2 (anti-Ri) antibody titer of 1:30,720; MRI brain and total spine were unrevealing.

Results An extensive workup for malignancy including multiple PET scans, a CT chest/abdomen/pelvis, mammography, and right upper quadrant ultrasound showed no evidence of neoplasm. The patient underwent therapy with intravenous immune globulin, steroids and rituximab. She had significant improvement in her clinical syndrome and decrease in antibody titers. On five-year follow up, no additional neoplasm was identified. Her last examination showed normal mentation and no myclonus; she remains with lower limb rigidity and ataxia. Her current ANNA-2 titer is 1:690.

Conclusions We present a rare patient in whom a clinical condition characterized by encephalopathy, diffuse myoclonus, ataxia and leg rigidity associated with high ANNA-2 titers developed 15 years after hysterectomy for cervical cancer. Extensive work up over 5 years showed no evidence for additional neoplasm. Aggressive medical treatment resulted in marked improvement of symptoms.

Authors/Disclosures
Duarte G. Machado, MD, FAAN (Hartford Healthcare Neurology) PRESENTER	Dr. Machado has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Supernus. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurocrine. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Adamas. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Ipsen. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Kyowa Kirin. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merz. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Neurocrine. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sunovion. Dr. Machado has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva.
Jacqueline Montes, PT, EdD, NCS (Columbia University Medical Center)	Ms. Montes has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Ms. Montes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for F. Hoffman LaRoche. Ms. Montes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Scholar Rock. Ms. Montes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. The institution of Ms. Montes has received research support from NIH/NICHD. The institution of Ms. Montes has received research support from Muscular Dystrophy Association. The institution of Ms. Montes has received research support from Cure SMA.
Anna Sorokin, MD	No disclosure on file
Bahman Jabbari, MD, FAAN	Dr. Jabbari has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbvie. Dr. Jabbari has received publishing royalties from a publication relating to health care.

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