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Title: Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies.
Authors: Sepúlveda, Maria
Sola-Valls, Nuria
Escudero, Domingo
Rojc, Bojan
Barón, Manuel
Hernández-Echebarría, Luis
Gómez, Begoña
Dalmau, Josep
Saiz, Albert
Graus, Francesc
Keywords: AQP4 antibodies;Neuromyelitis optica spectrum disorders;cancer;paraneoplastic
metadata.dc.subject.mesh: Adenocarcinoma
Aquaporin 4
Magnetic Resonance Imaging
Middle Aged
Myelitis, Transverse
Neuromyelitis Optica
Retrospective Studies
Issue Date: 18-Sep-2017
Abstract: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG. Paraneoplastic patients were defined as those with cancer identified within 2 years of the diagnosis of NMOSD. Five (3.2%) of 156 patients had paraneoplastic NMOSD, and 12 previously reported patients were identified. The most common tumors were adenocarcinoma of the lung (five patients) and breast (five). Compared with the 151 non-paraneoplastic NMOSD patients, the 17 (5 current cases and 12 previously reported) were older at symptom onset (median age = 55 (range: 17-87) vs 40 (range: 10-77) years; p = 0.006), more frequently male (29.4% vs 6.6%; p = 0.009), and presented with severe nausea and vomiting (41.2% vs 6.6%; p Patients, predominantly male, with NMOSD and AQP4-IgG should be investigated for an underlying cancer if they present with nausea and vomiting, or LETM after 45 years of age.
metadata.dc.identifier.doi: 10.1177/1352458517731914
Appears in Collections:Producción 2020

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