Abstract Details

Title Paraneoplastic Opsoclonus-Myoclonus Syndrome As Presenting Symptom of Primary Gallbladder Adenocarcinoma

Topic Neuro-oncology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 13-008

Objective To report a unique case of multiple auto-antibody positive opsoclonus-myoclonus syndrome (OMS) associated with adenocarcinoma of the gallbladder.

Background Opsoclonus-Myoclonus Syndrome (OMS) is a rare disorder characterized by combination of involuntary multidirectional saccadic eye movements (opsoclonus), limb and truncal myoclonus, ataxia and cognitive decline. It is recognized to have both paraneoplastic and non-paraneoplastic causes, and is most commonly seen in pediatric neuroblastoma. Multiple associated malignancies and auto-antibodies have been described in adults, most commonly Anti-Ri/ANNA-2 antibodies associated with breast and lung cancers. There are no prior case reports in the literature to date of gallbladder malignancy as underlying cause of paraneoplastic OMS.

Design/Methods Single institution retrospective case report.

Results A 75-year-old woman with no known malignancy presented with 6 months of progressive dizziness, falls, cognitive decline, involuntary movements, diplopia and “bouncing” vision. Neurological examination was notable for disorientation, emergence of primitive reflexes, myoclonic jerks and involuntary eye movements consistent with opsoclonus. MRI Brain revealed bilateral mesial temporal T2 FLAIR hyperintensities. Cerebrospinal fluid analysis was positive for ANNA-1, Ma1 and Ma2/Ta antibodies. Serum paraneoplastic panel was positive for anti-Ma1 (>1:3200), anti-Amphiphysin ( >1:3200), and Anti-GAD-65 (0.10) antibodies. Systemic staging demonstrated elevated tumor marker CA 19-9 (134.9 U/mL) and CT C/A/P revealed an enhancing mass in gallbladder. Patient underwent biopsy of a porta hepatis lymph node with pathology positive for metastatic adenocarcinoma consistent with gallbladder primary. The patient was treated with IV corticosteroids and IVIG with significant neurological recovery with improvement in opsoclonus, myoclonus and cognitive status.

Conclusions OMS is a recognized paraneoplastic syndrome in adults and children, with an increasingly broad range of cancers and autoantibodies found underlying this clinical syndrome. Presentation with OMS should prompt comprehensive neurologic and malignancy screening. We now add gallbladder cancer as a potential underlying malignancy to this syndrome.

Authors/Disclosures
Lauren Brandes, MD PRESENTER	Dr. Brandes has nothing to disclose.
Megan M. Mantica, MD (University of Pittsburgh)	Dr. Mantica has nothing to disclose.

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