Contributions
Abstract: P293
Type: Poster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Background: Neuromyelitis optica spectrum disorders (NMOSD) mainly involve spinal cord and optic nerve in association with highly specific serum antibodies against the aquaporin-4 water channel (AQP4-IgG). Few series of NMOSD AQP4-IgG positive patients have been reported in relation with the presence of tumors. Nevertheless, these series have included benign tumors, don't have applied established paraneoplastic neurological syndrome (PNS) criteria and have included limited clinical or follow-up data.
Objective: To define the clinical and paraclinical characteristics of AQP4-IgG positive patients with paraneoplastic NMOSD diagnosed according established PNS criteria and to compare with those of a cohort of idiopathic AQP4-IgG NMOSD patients.
Methods: We performed a retrospective, multicenter study. Data were collected from 2006 through 2016. We identified 186 AQP4-IgG positive NMOSD patients. Patients with a diagnosis of neoplasm within 2 years of the NMOSD clinical onset were classified as paraneoplastic. Samples were tested for AQP4-IgG and onconeuronal antibodies using immunohistochemistry and cell-based assays as described.
Results: Five (2.7%) AQP4-IgG positive NMOSD patients fulfilled PNS criteria. Three patients had non-small cell lung cancer, 1 breast cancer, and 1 oral epidermoid carcinoma. None of these patients had onconeuronal antibodies. Compared with idiopathic NMOSD, paraneoplastic NMOSD patients were more frequently male (60% vs 9%, p< 0.01) and older (mean age at onset 62 vs 40 years, p< 0.01). The clinical presentation was longitudinal extensive transverse myelitis (LETM) in 3 cases, and encephalitis/brainstem followed by sequential LETM (less than 1 month from first episode). None of the 5 patients presented episodes of optic neuritis. Paraneoplastic NMOSD patients exhibited, longer spinal cord lesions (mean vertebral segments affected: 13 vs 6, p=0.02), worse disability at onset and at last follow-up (p< 0.05) and higher mortality (60% vs 5%, p< 0.01).
Conclusions: Paraneoplastic NMOSD are more likely in older, male patients who present with LETM. Patients with this profile should be evaluated for the presence of an underlying cancer.
Disclosure:
Funding:This work was funded by a grant from La Marató de TV3 (20141830).
MS received speaker honoraria from Genzyme and Novartis and receives funding from the Generalitat de Catalunya (SLT002/16/00354).
NS-V receives funding from the Spanish Government (Instituto de Salud Carlos III, Spain and Fondo Europeo de Desarrollo Regional (FEDER, FI16/00251), and a Predoctoral Grant for Health Research.
SL received speaker honoraria from Biogen Idec, Novartis, Teva, Genzyme and Merck, and research support from a Juan Rodes grant from the Instituto de Salud Carlos III (JR14/00016) and the Spanish Government (PI15/00587).
YB received speaking honoraria from Biogen, Novartis and Genzyme.
AS received compensation for consulting services and speaker honoraria from Bayer-Schering, Merck-Serono, Biogen-Idec, Sanofi-Aventis, TEVA and Novartis, and receives funding from the Spanish Government (PI15/00587, RD16/0015/0002, RD16/0015/0003, RD12/0032/0002, RD12/0060/01-02)
DE and FG report no disclosures.
Abstract: P293
Type: Poster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Background: Neuromyelitis optica spectrum disorders (NMOSD) mainly involve spinal cord and optic nerve in association with highly specific serum antibodies against the aquaporin-4 water channel (AQP4-IgG). Few series of NMOSD AQP4-IgG positive patients have been reported in relation with the presence of tumors. Nevertheless, these series have included benign tumors, don't have applied established paraneoplastic neurological syndrome (PNS) criteria and have included limited clinical or follow-up data.
Objective: To define the clinical and paraclinical characteristics of AQP4-IgG positive patients with paraneoplastic NMOSD diagnosed according established PNS criteria and to compare with those of a cohort of idiopathic AQP4-IgG NMOSD patients.
Methods: We performed a retrospective, multicenter study. Data were collected from 2006 through 2016. We identified 186 AQP4-IgG positive NMOSD patients. Patients with a diagnosis of neoplasm within 2 years of the NMOSD clinical onset were classified as paraneoplastic. Samples were tested for AQP4-IgG and onconeuronal antibodies using immunohistochemistry and cell-based assays as described.
Results: Five (2.7%) AQP4-IgG positive NMOSD patients fulfilled PNS criteria. Three patients had non-small cell lung cancer, 1 breast cancer, and 1 oral epidermoid carcinoma. None of these patients had onconeuronal antibodies. Compared with idiopathic NMOSD, paraneoplastic NMOSD patients were more frequently male (60% vs 9%, p< 0.01) and older (mean age at onset 62 vs 40 years, p< 0.01). The clinical presentation was longitudinal extensive transverse myelitis (LETM) in 3 cases, and encephalitis/brainstem followed by sequential LETM (less than 1 month from first episode). None of the 5 patients presented episodes of optic neuritis. Paraneoplastic NMOSD patients exhibited, longer spinal cord lesions (mean vertebral segments affected: 13 vs 6, p=0.02), worse disability at onset and at last follow-up (p< 0.05) and higher mortality (60% vs 5%, p< 0.01).
Conclusions: Paraneoplastic NMOSD are more likely in older, male patients who present with LETM. Patients with this profile should be evaluated for the presence of an underlying cancer.
Disclosure:
Funding:This work was funded by a grant from La Marató de TV3 (20141830).
MS received speaker honoraria from Genzyme and Novartis and receives funding from the Generalitat de Catalunya (SLT002/16/00354).
NS-V receives funding from the Spanish Government (Instituto de Salud Carlos III, Spain and Fondo Europeo de Desarrollo Regional (FEDER, FI16/00251), and a Predoctoral Grant for Health Research.
SL received speaker honoraria from Biogen Idec, Novartis, Teva, Genzyme and Merck, and research support from a Juan Rodes grant from the Instituto de Salud Carlos III (JR14/00016) and the Spanish Government (PI15/00587).
YB received speaking honoraria from Biogen, Novartis and Genzyme.
AS received compensation for consulting services and speaker honoraria from Bayer-Schering, Merck-Serono, Biogen-Idec, Sanofi-Aventis, TEVA and Novartis, and receives funding from the Spanish Government (PI15/00587, RD16/0015/0002, RD16/0015/0003, RD12/0032/0002, RD12/0060/01-02)
DE and FG report no disclosures.