title: Distinctive clinical and neuroimaging characteristics of longitudinally extensive transverse myelitis associated with aquaporin-4 autoantibodies.
abstract: Longitudinally extensive transverse myelitis (LETM) is a characteristic feature of Neuromyelitis Optica (NMO), but it can also occur in several other inflammatory diseases of the central nervous system (CNS). An IgG autoantibody that binds to aquaporin-4 (AQP4), the predominant water channel of the CNS, is a reliable biomarker of the NMO spectrum disorders, and if detected predicts the recurrence of the myelitis. In this study, we compared the clinical and neuroimaging characteristics of AQP4-IgG+ and AQP4-IgG- LETM patients. Thirty-seven first-ever LETM patients were retrospectively evaluated and divided into two groups according to the presence of AQP4 autoantibodies. AQP4-IgG was detected in the serum and in the cerebrospinal fluid of sixteen patients. The female to male ratio was higher in AQP4-IgG+ patients. Intractable nausea and vomiting and paroxysmal tonic spasms often accompanied the LETM in AQP4-IgG+ patients. T2-weighted spinal cord MRI revealed that inflammatory lesions extending into the brainstem and involving the central grey matter occurred more frequently in AQP4-IgG+ LETM patients. Hypointense lesions on T1-weighted spinal cord MRI were detected more frequently in the seropositive group, and their presence correlated with attack severity. In conclusion, this study provides clinical and spinal cord neuroimaging clues that can help distinguishing AQP4-IgG+ LETM patients.
authors.affiliation: Institute of Neurology, Department of Geriatrics Neurosciences and Orthopedics, Catholic University, L. go Gemelli, 8 00168 Rome, Italy. iorio.raffaele@gmail.com
authors.forename: Raffaele
authors.initials: R
authors.lastname: Iorio
authors.forename: Valentina
authors.initials: V
authors.lastname: Damato
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Mirabella
authors.forename: Amelia
authors.initials: A
authors.lastname: Evoli
authors.forename: Alessandro
authors.initials: A
authors.lastname: Marti
authors.forename: Domenico
authors.initials: D
authors.lastname: Plantone
authors.forename: Giovanni
authors.initials: G
authors.lastname: Frisullo
authors.forename: Anna Paola
authors.initials: AP
authors.lastname: Batocchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000097:blood / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D001921:Brain / Q000473:pathology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000276:immunology* / Q000473:pathology*; D059906:Neuroimaging; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D007074:Immunoglobulin G