Alberto Vogrig1,2,3,4, Bastien Joubert1,2,3, Aurélien Maureille1,2,3,5, Laure Thomas1,2,3, Emilien Bernard6, Nathalie Streichenberger7, Francois Cotton8,9, Francois Ducray1,2,3, Jérome Honnorat10,11,12,13. 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. French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France. jerome.honnorat@chu-lyon.fr. 11. SynatAc Team, NeuroMyoGene Institute, INSERM U1217/CNRS UMR5310, Lyon, France. jerome.honnorat@chu-lyon.fr. 12. University Claude Bernard Lyon 1, Université de Lyon, Lyon, France. jerome.honnorat@chu-lyon.fr. 13. Centre de Référence National pour les Syndromes Neurologiques Paranéoplasique, Hôpital Neurologique, 59 Boulevard Pinel, 69677, Bron Cedex, France. jerome.honnorat@chu-lyon.fr.
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 definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the literature. RESULTS: Among 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identified 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 myeloradiculopathy. The most common MNS signs/symptoms were: hyperreflexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyramidal 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 first-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identified 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 differs from amyotrophic lateral sclerosis.
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 definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the literature. RESULTS: Among 33 Ma2-Abpatients referred between 2002 and 2016, we retrospectively identified 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 myeloradiculopathy. The most common MNS signs/symptoms were: hyperreflexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyramidal 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 first-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identified 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 differs from amyotrophic lateral sclerosis.
Authors: F Graus; J Y Delattre; J C Antoine; J Dalmau; B Giometto; W Grisold; J Honnorat; P Sillevis Smitt; Ch Vedeler; J J G M Verschuuren; A Vincent; R Voltz Journal: J Neurol Neurosurg Psychiatry Date: 2004-08 Impact factor: 10.154
Authors: Alberto Vogrig; Gian Luigi Gigli; Samantha Segatti; Elisa Corazza; Alessandro Marini; Andrea Bernardini; Francesca Valent; Martina Fabris; Francesco Curcio; Francesco Brigo; Donatella Iacono; Paolo Passadore; Michele Rana; Jérôme Honnorat; Mariarosaria Valente Journal: J Neurol Date: 2019-09-24 Impact factor: 4.849