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. 2018 Nov;24(13):1753-1759.
doi: 10.1177/1352458517731914. Epub 2017 Sep 18.

Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies

Maria Sepúlveda  1 , Nuria Sola-Valls  1 , Domingo Escudero  1 , Bojan Rojc  2 , Manuel Barón  3 , Luis Hernández-Echebarría  4 , Begoña Gómez  5 , Josep Dalmau  6 , Albert Saiz  1 , Francesc Graus  1
Affiliations

Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies

Maria Sepúlveda et al. Mult Scler. 2018 Nov.

Abstract

Background: 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.

Objective: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases.

Methods: 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.

Results: 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 < 0.001). The frequency of longitudinal extensive transverse myelitis (LETM) as heralding symptom was similar in both groups, but patients with paraneoplastic NMOSD were older than those with non-paraneoplastic NMOSD (median age: 63 (range: 48-73) vs 43 (range: 14-74) years; p = 0.001).

Conclusion: 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.

Keywords: AQP4 antibodies; Neuromyelitis optica spectrum disorders; cancer; paraneoplastic.

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Conflict of interest statement

Declaration of Conflicting Interests

The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr Sepulveda received speaker honoraria from Genzyme and Novartis. Dr Dalmau receives royalties from Athena Diagnostics for the use of Ma2 as an autoantibody test and from Euroimmun for the use of NMDA, GABAB receptor, GABAA receptor, DPPX, and IgLON5 as autoantibody tests; he has received an unrestricted research grant from Euroimmun. Dr Graus received a licensing fee from Euroimmun for the use of IgLON5 as an autoantibody test. Dr Sola-Valls, Dr Escudero, Dr Rojc, Dr Barón, Dr Hernández-Echebarría, Dr Gómez, and Dr Saiz report no disclosures.

Figures

Figure 1
Figure 1
(a) Paraffin section of a lung adenocarcinoma from a patient with LETM and AQP4 antibodies (hematoxylin eosin) and (b and c) AQP4 reactivity detected with a commercial polyclonal rabbit antibody against AQP4.
See this image and copyright information in PMC

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