TY - JOUR T1 - Early intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with onconeural antibodies JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry DO - 10.1136/jnnp-2017-316904 SP - jnnp-2017-316904 AU - Giulia Berzero AU - Evgenia Karantoni AU - Caroline Dehais AU - François Ducray AU - Laure Thomas AU - Géraldine Picard AU - Véronique Rogemond AU - Gaëlle Candelier AU - Jean-Philippe Camdessanché AU - Jean-Christophe Antoine AU - Jérôme De Seze AU - Amélie Liou-Schischmanoff AU - Jérôme Honnorat AU - Jean-Yves Delattre AU - Dimitri Psimaras Y1 - 2018/02/10 UR - http://jnnp.bmj.com/content/early/2018/02/10/jnnp-2017-316904.abstract N2 - Paraneoplastic neurological syndromes (PNS) are immune-mediated complications of cancer, characterised by relentless progression. The mainstay of PNS treatment is the achievement of tumour remission,1 while immunotherapy provides only little additional benefit.2 3 However, in historical series, immunotherapy was initiated over 6 months after neurological onset and, at that stage, neuronal loss is already extensive and irreversible.Among available immunotherapies, intravenous immunoglobulin (IVIg) has been used in single cases4 and in one retrospective series,3 showing some efficacy when administered timely.4 Based on these findings, we designed a prospective study to assess the efficacy and safety of early IVIg treatment in patients with PNS.Study designThis prospective, multicentre, non-comparative, phase II clinical study was performed by the ‘Centre de Reference Français des Syndromes Neurologiques Paranéoplasiques’. Written informed consent was obtained from all participants. This trial is registered at ClinicalTrials.gov (NCT02343211). ParticipantsInclusion criteria were: (1) diagnosis of ‘definite’ PNS5; (2) anti-Hu, anti-Yo, or anti-CV2/CRMP5 antibodies in the serum and/or in the cerebrospinal fluid; (3) neurological symptom onset within 6 months; (4) modified Rankin Score (mRS) 2 or 3; (5) neurological deterioration over the last 3 weeks. Exclusion criteria were: (1) other concomitant immunotherapy; (2) absolute contraindications to IVIg (hypersensitivity to IVIg, selective IgA deficiency); (3) thrombophilia; (4) renal insufficiency (creatinine clearance <30 mL/min).InterventionsEnrolled patients received three cycles of IVIg (Privigen, 2 g/kg, every 4 weeks), followed by an interim evaluation. If the patient was stable or improved according to the primary outcome measure, three additional IVIg cycles were administered. If the patient deteriorated, IVIg was discontinued. Final evaluation was performed at 6 months.The primary endpoint was improvement on the mRS at 3 months … ER -