title: Systemic sclerosis in aquaporin-4 antibody-positive longitudinally extensive transverse myelitis.
abstract: We report on the first patient with a relapsing, anti-aquaporin-4 (AQP-4) antibody-positive, longitudinally extensive transverse myelitis (LETM) who developed systemic sclerosis (SSc). A 62-year-old woman, who presented with bilateral, distal lower limb and perineal numbness, developed clinical manifestations and paraclinical features of SSc. Spinal cord imaging revealed lesions that were consistent with LETM. Patient's serum was positive for neuromyelitis optica (NMO)-IgG/AQP-4 antibodies. High-dose intravenous corticosteroids improved the neurological symptoms. The present case expands the list of autoimmune systemic diseases that occur in neuromyelitis optica spectrum disorders associated with NMO-IgG/AQP-4 antibodies.
authors.affiliation: Laboratory of Neuroimmunology, IRCCS, National Neurological Institute C. Mondino, Italy. diego.franciotta@mondino.it
authors.forename: Diego
authors.initials: D
authors.lastname: Franciotta
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Zardini
authors.forename: Roberto
authors.initials: R
authors.lastname: Caporali
authors.forename: Laura
authors.initials: L
authors.lastname: Piccolo
authors.forename: Elisa
authors.initials: E
authors.lastname: Alberici
authors.forename: Alfredo
authors.initials: A
authors.lastname: Romani
authors.forename: Roberto
authors.initials: R
authors.lastname: Bergamaschi
authors.forename: Enrico
authors.initials: E
authors.lastname: Marchioni
authors.forename: Mauro
authors.initials: M
authors.lastname: Ceroni
authors.forename: Giovanni
authors.initials: G
authors.lastname: Piccolo
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000032:analysis*; D001921:Brain / Q000473:pathology; D005155:Facial Nerve Diseases / Q000150:complications / Q000473:pathology; D005260:Female; D006801:Humans; D007275:Injections, Intravenous; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D018908:Muscle Weakness / Q000150:complications; D009188:Myelitis, Transverse / Q000150:complications* / Q000276:immunology*; D009471:Neuromyelitis Optica / Q000150:complications / Q000276:immunology; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000209:etiology*; D013116:Spinal Cord / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D051401:Aquaporin 4; D001323:Autoantibodies; D011239:Prednisolone; D008775:Methylprednisolone