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• W1935202080 abstract "OBJECTIVE: To report the clinical characteristics, type and frequency of hydrocephalus in aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica (NMO) spectrum disorders. BACKGROUND: optica (NMO) is an inflammatory demyelinating autoimmune disease of the central nervous system that is characterized by relapsing attacks preferentially involving the optic nerves and spinal cord. The disease-specific autoantibody marker of NMO, NMO-immunoglobulin G (IgG), binds to the extracellular domain of the AQP4 water channel which is concentrated in polarized foot processes of astrocytes at CNS tissue -fluid interfaces (blood-brain and brain-cerebrospinal fluid) and encircling glutamatergic synapses and nodes of Ranvier. DESIGN/METHODS: Obstructive hydrocephalus was identified in the index case. We therefore reviewed, with a study neurologist and neuroradiologist, all available head MRIs from AQP4-IgG-seropositive cases identified in the Mayo Clinic NMO clinical biospecimens database (125 fulfilled NMO diagnostic criteria; 45 had an NMO spectrum disorder). RESULTS: Hydrocephalus was identified in 2 of 177 AQP4-IgG positive patients (frequency 1.1%), in addition to the index case. In all three patients, imaging findings were consistent with obstructive hydrocephalus: third and lateral ventricles were enlarged, and the fourth ventricle and aqueduct were relatively small. CONCLUSIONS: Obstructive hydrocephalus is encountered in NMO spectrum disorders more frequently than observed in the general adult population. This likely reflects the vulnerability of the cerebral aqueduct of Sylvius to stenosis in the setting of immunopathology targeting AQP4-rich periventricular and periependymal tissues. Study Supported by: Supported by the National Institutes of Health (R01 NS065829) and the Guthy Jackson Foundation. Disclosure: Dr. Lucchinetti stands to receive royalty payments from Biogen Idec. Dr. Krecke has nothing to disclose. Dr. Lennon stands to receive royalty payments for commercial assays to detect of Aquaporin 4-specific Autoantibody. Dr. O9Toole has nothing to disclose. Dr. Weinshenker has received personal compensation for activities with Novartis, Biogen Idec, and Mitsubishi Pharmaceuticals as consultant on data safety monitoring boards; and with Elan Corporation, Ono Pharmaceutical, GlaxoSmithKline Inc., Alexion and Chugai Pharmaceuticals, and Asahi Kasei Medical Company as a consultant. Dr. Weinshenker has received royalty payments from Mayo Foundation. Dr. Boyd has nothing to disclose. Dr. Krieger has received personal compensation for activities with Acorda Therapeutics, Bayer Healthcare, Biogen Idec, Genzyme Corporation, Questcor, and Teva Neuroscience. Dr. Krieger has received research support from Bayer Healthcare. Dr. McGraw has received personal compensation for activities with Biogen Idec as a speaker. Dr. Guo has nothing to disclose. Dr. Pittock9s institution has received compensation for activities with Alexion Pharmaceuticals, MedImmune, and Chugai Pharma. Dr. Pittock stands to receive royalty payments from the technology entitled Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia. Dr. Pittock has received research support from Alexion Pharmaceuticals, Inc." @default.
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• W1935202080 date "2014-04-08" @default.
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• W1935202080 title "Hydrocephalus in Neuromyelitis Optica (P2.270)" @default.
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