JS1.2 Increased frequency of paraneoplastic encephalitis with anti-Ma2 antibodies in the cancer immunotherapy era. (6th September 2019)

• Record Type: Journal Article
• Authors: Vogrig, A; Fouret, M; Joubert, B; Picard, G; Rogemond, V; Pinto, A; Muñiz Castrillo, S; Roger, M; Raimbourg, J; Dayen, C; Grignou, L; Pallix-Guyot, M; Lannoy, J; Ducray, F; Desestret, V; Psimaras, D; Honnorat, J
• Is Part Of: Neuro-oncology. Volume 21(2019)Supplement 3
• Page Start: iii4
• Page End: iii5
• Publication Date: 2019-09-06
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noz126.011
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: BACKGROUND: Given the mechanism of action of immune checkpoint inhibitors (ICIs), a possible association with the development of paraneoplastic neurological syndromes (PNS) has been hypothesized. We sought to determine if ICI treatment might trigger anti-Ma2 antibody-associated paraneoplastic neurological syndromes (Ma2-PNS). MATERIAL AND METHODS: We performed a retrospective nationwide study of all patients with Ma2-PNS developed during ICI treatment between 2017–2018. The frequency of neuronal-antibody detection before and after ICI implementation was also analyzed. RESULTS: Our series of patients included 5 men and 1 woman (median age, 63 years). The patients were receiving either nivolumab (n = 3), pembrolizumab (n = 2) or a combination of nivolumab and ipilimumab (n=1), for treatment of neoplasms that included lung (n=4) and kidney (n=1) cancers, and pleural mesothelioma (n=1). Median delay between ICI introduction and onset of the neurological syndrome was 4 months. Clinical syndromes included a combination of limbic encephalitis and diencephalitis (n=3), isolated limbic encephalitis (n=2) and a syndrome characterized by opthalmoplegia and head-drop (n=1). Patients with diencephalitis manifested hyperphagia, weight gain and somnolence. Post-ICI Ma2-PNS accounted for 35% of the total 17 Ma2-PNS diagnosed in our center over the 2017–2018 biennium. Eight cases had been detected in the preceding biennium 2015–2016, corresponding to a 112% increase of Ma2-PNS frequency since the implementation of ICIs in France. No other neuronal-antibody demonstrated a similar increment. CONCLUSION: We show a clear association between ICI use and increased frequency of Ma2-PNS. Physicians need to be aware that ICIs can trigger Ma2-PNS since clinical presentation can be challenging.