title: Contribution of spinal cord biopsy to diagnosis of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder.
abstract: Longitudinally extensive transverse myelitis is characteristic but not pathognomonic for neuromyelitis optica spectrum disorders (NMOSDs) and may mimic local tumors. In this retrospective study based on a cohort of 175 NMOSD patients we identified seven patients who initially presented with a longitudinally extensive spinal cord lesion and underwent spinal cord biopsy due to magnetic resonance imaging (MRI)-suspected malignancies. Remarkably, routine neuropathology was inconclusive and did not guide the diagnostic process to anti-aquaporin-4 (AQP4)-seropositive NMOSD. Serious postoperative complications occurred in 5/7 patients and persisted during follow-up in 2/7 patients (29%). Considering these sequelae, AQP4-antibody testing should be mandatory in patients with inconclusive longitudinally extensive spinal cord lesions prior to biopsy.
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Ringelstein
authors.affiliation: Department of Neuropathology, University Hospital Göttingen, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Metz
authors.affiliation: Department of Neurology, Charité-Universitätsmedizin Berlin, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Ruprecht
authors.affiliation: Department of Neuropathology, Charité-Universitätsmedizin Berlin, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Koch
authors.affiliation: Department of Neurology, Landesklinikum Thermenregion Hochegg, Austria.
authors.forename: J
authors.initials: J
authors.lastname: Rappold
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Ingwersen
authors.affiliation: Department of Diagnostic and Interventional Radiology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Mathys
authors.affiliation: Division of Molecular Neuroimmunology, Department of Neurology, University Hospital Heidelberg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Jarius
authors.affiliation: Department of Neuropathology, University Hospital Göttingen, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Brück
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany.
authors.forename: H-P
authors.initials: HP
authors.lastname: Hartung
authors.affiliation: NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Paul
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University Düsseldorf, Germany orhan.aktas@uni-duesseldorf.de.
authors.forename: O
authors.initials: O
authors.lastname: Aktas
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012698:Serologic Tests; D013116:Spinal Cord / Q000473:pathology*; D013997:Time Factors; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D015415:Biomarkers
keywords: Spinal cord tumor; aquaporin-4 antibody; longitudinally extensive spinal cord lesion; longitudinally extensive transverse myelitis; neuromyelitis optica; spinal biopsy