Defining Area Postrema (AP) Attacks in Autoimmune AQP4 Channelopathies/Neuromyelitis Optica Spectrum Disorders (NMOSD) (P6.386)

Objective: To define the frequency, duration and severity of intractable nausea, vomiting or hiccups (INVH) in NMOSD Background: INVH are symptoms of inflammation targeted to AQP4-expressing astrocytes in the AP. Published data relating to frequency, duration, and severity of INVH are lacking. Design/Methods: Of 541 patients with AQP4-IgG-positive NMOSD in Mayo Clinic’s database, 200 reported INVH at some point. To date, 52 patients with a history of INVH have been surveyed by telephone. Results: INVH occurred in the first attack in 26 (50%) patients. All 3 symptoms (NVH) were present in 31 patients, 2 symptoms in 16 and only a single symptom in 5 (N [1], H [4]). Nausea was acute in onset in 32\48 patients (60%). The median duration of nausea was 19 days (range 3–90). Forty-four experienced vomiting with a median vomitus frequency of 6/day (range 2–20). Patients usually reported vomiting rapidly after eating liquid or solid. Sixteen were awakened by vomiting. Weight loss was reported in 16 patients (median 16lbs, range 3–70). Hiccups occurred in 35 and lasted all day (non-stop) for median of 30 days (range 3–90) in 9. Fifteen patients were awakened from sleep by hiccups. INVH led to hospitalization and IV rehydration in 32 patients. Twenty one (40%) patients presented to gastroenterologists and in 12 diagnosis assigned as gastritis (2), cyclic-vomiting syndrome (3), gastroparesis (3), cholecytitis (1) and Crohn’s disease (1), food poisoning (1), pancreatitis (1). All 21 patients receiving intravenous methylprednisolone or plasmapharesis showed resolution of NVH. INVH commonly preceded other symptoms of NMOSD exacerbation (optic neuritis in18, 34 %; transverse myelitis in 26, 50%, brainstem syndromes in 2, 4%) within median of 2 weeks (range 0–4) after INVH onset Conclusions: These data characterize the “area postrema” attack phenotype and will assist in development of diagnostic criteria and severity indices for such attacks Study Supported by: Mayo Clinic Foundation Disclosure: Dr. Shosha has nothing to disclose. Dr. Majed has nothing to disclose. Dr. Weinshenker has received personal compensation for activities with Novartis, Mitsubishi Pharmaceuticals, Medimmune and Alexion Pharmaceuticals. Dr. Weinshenker has received (royalty or license fee or contractual rights) payments from RSR Ltd., Oxford University and MVZ Labor PD and Mayo Foundation. Dr. Flanagan has nothing to disclose. Dr. Huebert has nothing to disclose. Dr. Sagen has nothing to disclose. Dr. Levy has received personal compensation from Alexion, Guidepoint Global, Genzyme and Acorda. Dr. Greenberg has received personal compensation for activities with Novartis Boston Pharmaceuticals, and MSAA. Dr. Greenberg has received personal compensation in an editorial capacity for JAMA Neurology. Dr. Greenberg has received research support from Medimmune, Acorda, and Chugai. Dr. Pittock9s institution has received compensation for Dr. Pittock9s activities with Alexion Pharmaceuticals, Medimmune and Chugai Pharma USA. Dr. Pittock and Dr. Pittock9s institution stand to receive patent payments that relate to patents for functional AQP4/NMO-IgG assays and NMO-IgG as a cancer marker. Dr. Pittock has received research support from NIH, Alexion Pharmaceuticals and Medimmune related to autoimmune NMO/AQP4 channelopathy.