Maria Sepúlveda1, Nuria Sola-Valls1, Domingo Escudero1, Bojan Rojc2, Manuel Barón3, Luis Hernández-Echebarría4, Begoña Gómez5, Josep Dalmau6, Albert Saiz1, Francesc Graus1. 1. Service of Neurology, Hospital Clinic, University of Barcelona, Barcelona, Spain Neuroimmunology Program, Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain. 2. Service of Neurology, General Hospital Izola, Izola, Slovenia. 3. Service of Neurology, Hospital Universitario Fundación Alcorcón, Madrid, Spain. 4. Service of Neurology, Hospital de León, León, Spain. 5. Service of Neurology, Hospital Universitario Puerto Real, Cádiz, Spain. 6. Neuroimmunology Program, Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain/Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain/Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
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.
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. Paraneoplasticpatients 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 NMOSDpatients, 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.
Authors: Metha Apiwattanakul; Bogdan F Popescu; Marcelo Matiello; Brian G Weinshenker; Claudia F Lucchinetti; Vanda A Lennon; Andrew McKeon; Adam F Carpenter; Gary M Miller; Sean J Pittock Journal: Ann Neurol Date: 2010-11 Impact factor: 10.422
Authors: F Graus; J Y Delattre; J C Antoine; J Dalmau; B Giometto; W Grisold; J Honnorat; P Sillevis Smitt; Ch Vedeler; J J G M Verschuuren; A Vincent; R Voltz Journal: J Neurol Neurosurg Psychiatry Date: 2004-08 Impact factor: 10.154
Authors: Dean M Wingerchuk; Brenda Banwell; Jeffrey L Bennett; Philippe Cabre; William Carroll; Tanuja Chitnis; Jérôme de Seze; Kazuo Fujihara; Benjamin Greenberg; Anu Jacob; Sven Jarius; Marco Lana-Peixoto; Michael Levy; Jack H Simon; Silvia Tenembaum; Anthony L Traboulsee; Patrick Waters; Kay E Wellik; Brian G Weinshenker Journal: Neurology Date: 2015-06-19 Impact factor: 9.910
Authors: Maria Sepúlveda; Thaís Armangué; Nuria Sola-Valls; Georgina Arrambide; José E Meca-Lallana; Celia Oreja-Guevara; Mar Mendibe; Amaya Alvarez de Arcaya; Yolanda Aladro; Bonaventura Casanova; Javier Olascoaga; Adolfo Jiménez-Huete; Mireya Fernández-Fournier; Lluis Ramió-Torrentà; Alvaro Cobo-Calvo; Montserrat Viñals; Clara de Andrés; Virginia Meca-Lallana; Angeles Cervelló; Carmen Calles; Manuel Barón Rubio; Cristina Ramo-Tello; Ana Caminero; Elvira Munteis; Alfredo R Antigüedad; Yolanda Blanco; Pablo Villoslada; Xavier Montalban; Francesc Graus; Albert Saiz Journal: Neurol Neuroimmunol Neuroinflamm Date: 2016-04-14
Authors: Pilar Carrillo; Teresa Gorría; Daniel Santana; Maria Sepulveda; Iban Aldecoa; Blanca Gonzalez-Farré; Esther Sanfeliu; Eduard Mension; Isaac Cebrecos; Olga Martínez-Saez; Imma Alonso; Albert Saiz Journal: Biomed Hub Date: 2022-01-31