Abstract Details

Title Paraneoplastic Neuromyelitis Optica Spectrum Disorder in a Patient with Endometrial Adenocarcinoma

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-018

Objective To present a case of paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) in a patient with endometrial cancer

Background Paraneoplastic neuromyelitis optica spectrum disorder (NMOSD), in relation to aquaporin-4 (AQU4) IgG, is an immune-mediated, erroneous attack on the spinal cord, optic nerves or brainstem, originally directed against the tumor. The reported incidence of tumor in patients with NMOSD associated with AQU4-IgG is about 5%.

Design/Methods A 51-year-old female recently diagnosed with stage IIIB endometrial carcinoma (metastatic disease to peritoneum, lung, and liver) presented for progressive weakness and paresthesia of the right arm and leg for three weeks. MRI of the spine revealed two peripheral areas of signal abnormality with associated enhancement: a longitudinally extensive cervical lesion, and a thoracic lesion involving one vertebral segment. A brain MRI revealed no evidence of lesion burden. Cerebrospinal fluid revealed normal protein and glucose, without cells, an elevated IgG index and synthesis rate, without oligoclonal bands, and negative infectious workup. She denied prior history of vision changes or focal weakness or numbness of the extremities. AQU4 antibody titers from the serum was > 80, confirmed with Mayo NMO IgG antibody titer > 1000. She was treated with intravenous methylprednisolone over five days with notable improvement in her right sided weakness and paresthesia. She is currently undergoing palliative chemotherapy with taxol/carboplatin. AQU4-IgG staining of the endometrial biopsy was pending at the time of this abstract submission.

Results NA

Conclusions This is the first case to describe such a case of NMOSD in a patient with endometrial adenocarcinoma. NMOSD, unlike idiopathic NMO, is more likely to be paraneoplastic in patients aged over 50 years at onset of symptoms, especially in female patients. It is advisable that AQU-4-IgG be added as an onconeural antibody as part of the paraneoplastic workup, though its clinical utility does warrant additional investigation.

Authors/Disclosures
Andre Granger, MD, MBA (Mayo Clinic) PRESENTER	Dr. Granger has nothing to disclose.
Elina Zakin, MD, FAAN (NYU Grossman School of Medicine)	The institution of Dr. Zakin has received research support from American Board of Psychiatry and Neurology.

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