Occurrence of area postrema syndrome during follow-up: phenotype and influence over NMOSD activity in LATAM in real-world settings

• Published in: Journal of neurology Vol. 271; no. 7; pp. 4292 - 4299
• Main Authors: Pestchanker, Claudia, Bertado Cortez, Brenda, Lana Peixoto, Marco A., Gortari, José Ignacio, Castro Suarez, Sheila, Caparo Zamalloa, Cesar, Galiana, Graciana, Peñalver, Francisco, Daccach Marques, Vanesa, Messias, Katharina, Galleguillos, Lorna, García, Fernando, Rojas, Juan I., Patrucco, Liliana, Cristiano, Edgardo, Tkachuk, Verónica, Liwacki, Susana, Correale, Jorge, Marrodan, Mariano, Ysraelit, María C., Vrech, Carlos, Deri, Norma, Leguizamon, Felisa, Tavolini, Darío, Mainella, Carolina, Zanga, Gisela, Alonso Serena, Marina, Ciampi, Ethel, Neto, Herval Ribeiro Soares, Lopez, Pablo, Carnero Contentti, Edgar
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-07-2024; Springer Nature B.V
• Subjects: Adult; Area Postrema; Female; Follow-Up Studies; Humans; Immunotherapy; Male; Medicine; Medicine & Public Health; Middle Aged; Neurology; Neuromyelitis; Neuromyelitis Optica - epidemiology; Neuromyelitis Optica - physiopathology; Neuromyelitis Optica - therapy; Neuroradiology; Neurosciences; Original Communication; Phenotype; Phenotypes; Plasmapheresis; Regression analysis; Retrospective Studies; Severity of Illness Index; Steroid hormones; Phenotype; Severity; Area postrema syndrome; NMOSD
• ISSN: 0340-5354; 1432-1459; 1432-1459
• DOI: 10.1007/s00415-024-12371-8

Introduction
We aimed to assess the frequency, duration, and severity of area postrema syndrome (APS) during follow-up in neuromyelitis optica spectrum disorder (NMOSD) patients, as well as its association with inflammatory activity and prognostic factors of APS severity in a real-world setting.
Methods
We conducted a retrospective study on a cohort of Latin American (LATAM) NMOSD patients who had experienced APS during their follow-up. Patients from Mexico, Peru, Brazil, Colombia, Panama, Chile and Argentina patients who met 2015 NMOSD criteria were included. We evaluated data on symptom type (nausea, vomiting and/or hiccups), frequency, duration, severity (measured by APS severity scale), association with other NMOSD core relapses, and acute treatments (symptomatic and immunotherapy or plasmapheresis). Logistic regression was conducted to evaluate factors associated with APS severity (vs. mild-moderate).
Results
Out of 631 NMOSD patients, 116 (18.3%) developed APS during their follow-up. The most common APS phenotype was severe. Inflammatory activity (i.e., relapses) significantly decreased after the onset of APS. Half of the patients experienced isolated APS with a median duration of 10 days, and the most frequently used acute treatment was IV steroids. All three symptoms were present in 44.6% of the patients. APS symptoms resolved following immunotherapy. Logistic regression did not identify independent factors associated with the severity of APS.
Conclusions
Our findings indicate that 18.3% of NMOSD patients developed APS during the follow-up period, with most patients fulfilling criteria for severe APS. The inflammatory activity decreased after the onset of APS compared to the previous year.