Vol.:(0123456789) 1 3 Journal of Neurology https://doi.org/10.1007/s00415-018-9143-x ORIGINAL COMMUNICATION Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome Alberto Vogrig 1,2,3,4  · Bastien Joubert 1,2,3  · Aurélien Maureille 1,2,3,5  · Laure Thomas 1,2,3  · Emilien Bernard 6  · Nathalie Streichenberger 7  · Francois Cotton 8,9  · Francois Ducray 1,2,3  · Jérome Honnorat 1,2,3,10 Received: 1 October 2018 / Revised: 12 November 2018 / Accepted: 26 November 2018 © Springer-Verlag GmbH Germany, part of Springer Nature 2018 Abstract Objective To present clinical, radiological, and pathological features of a cohort of patients with motor neuron involvement in association with anti-Ma2 antibodies (Ma2-Ab). Methods Retrospective case-series of patients with defnite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identifed from a review of the literature. Results Among 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identifed three patients (9.1%) with a motor neuron syndrome (MNS). Seven additional cases were retrieved among the 75 Ma2-patients reported in the literature (9.3%). A total of ten patients are, therefore, described herein. MNS was evident as combined upper and lower MNS in four patients, isolated upper MNS in two, and isolated lower MNS in one; three patients were diagnosed with myeloradiculopa- thy. The most common MNS signs/symptoms were: hyperrefexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyrami- dal tract T2-weighted/FLAIR hyperintensities (three patients). Spine MRI found bilateral, symmetric, T2-weighted signal abnormalities in the anterior horn in two patients. CSF examination was abnormal in nine patients. Cancer was found in seven patients (four testicular, two lung, and one mesothelioma). Eight patients underwent frst-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identifed in the literature. Motor improvement was observed in 33% of our patients, and 20% in the literature series. Conclusions Motor neuron involvement could complicate Ma2-Ab-associated PNS in almost 10% of patients and must be carefully studied to adapt treatment. This disorder difers from amyotrophic lateral sclerosis. Keywords Paraneoplastic syndromes · Motor neuron syndrome · Amyotrophic lateral sclerosis · Anti-Ma · Myelopathy · Radiculopathy * Jérome Honnorat jerome.honnorat@chu-lyon.fr 1 French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France 2 SynatAc Team, NeuroMyoGene Institute, INSERM U1217/CNRS UMR5310, Lyon, France 3 University Claude Bernard Lyon 1, Université de Lyon, Lyon, France 4 Department of Neurosciences, Santa Maria della Misericordia University Hospital, Udine, Italy 5 Department of Neurology, Lille University Hospital, Lille, France 6 Department of Electroneuromyography and Neuromuscular Diseases, Hospices Civils de Lyon, Lyon, France 7 Centre de Neuropathologie Est, Hospices Civils de Lyon, Université Claude Bernard Lyon1, Institut NeuroMyogène, CNRS UMR 5310, INSERM U1217, Lyon, France 8 Service de Radiologie, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France 9 CREATIS-CNRS UMR 5220 & INSERM U1044, University Claude Bernard Lyon 1, Lyon, France 10 Centre de Référence National pour les Syndromes Neurologiques Paranéoplasique, Hôpital Neurologique, 59 Boulevard Pinel, 69677 Bron Cedex, France