• K Chevalier, N Noel, F Benoudiba, P Chrétien, S Hacein-Bey-Abina, and O Lambotte.
• Department of Internal Medicine and Clinical Immunology, Université Paris-Saclay, AP-HP, Le Kremlin-Bicêtre, France.
• Rev Med Interne. 2021 Aug 1; 42 (8): 575-578.

IntroductionOnconeuronal antibodies directed against intracellular antigens are strongly associated with paraneoplastic syndromes and their detection in the absence of cancer is unusual. We herein report a case of anti-Ma2 encephalitis associated with Sjogren's syndrome (SS).Case ReportAn 81-year-old woman followed for a cutaneous lupus with vasculitis associated with SS presented a flare of her disease with neurological worsening including walking difficulty, hypersialorrhea and dysphagia. A paraneoplastic origin of the symptoms was suspected and anti-Ma2 antibodies were positive in serum. The search for an underlying neoplasia was negative. The diagnosis of anti-Ma2 encephalitis secondary to a SS was made. In the literature, the association of anti-Ma2 encephalitis and SS has been previously reported twice. Cases of patients with other onconeuronal antibodies associated with SS have been also reported. Anti-Ma2 encephalitis is a rare condition with a wide spectrum of symptoms associated with a cancer in more than 90% of the cases. Anti-Ma2 encephalitis has also been described after the use of immune check points inhibitors underscoring the role of autoimmunity in its pathogenesis.ConclusionAnti-Ma2 encephalitis is essentially associated with neoplasia but can occur in Sjogren's syndrome.Copyright © 2021 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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