title: [Contribution of spinal cord biopsy to the differential diagnosis of longitudinal extensive transverse myelitis].
abstract: BACKGROUND
  Neuromyelitis optica spectrum disorders (NMOSD) are characterized by recurrent optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM) as well as the serological detection of antibodies to aquaporin-4 (AQP4-ab). However, longitudinal extensive spinal cord lesions are not pathognomonic for NMOSD as they can also occur in systemic autoimmune diseases or mimic spinal cord tumors.
  
  
  OBJECTIVE
  We report a female patient who initially presented with a subacute spinal syndrome and a longitudinal spinal cord lesion on magnetic resonance imaging (MRI). As the brain MRI showed only unspecific white matter lesions and the cerebrospinal fluid was normal, a spinal cord biopsy was performed to exclude malignancies and revealed inflammatory demyelinating changes. In addition, after several deep vein thromboses and the detection of antiphospholipid antibodies, an antiphospholipid syndrome (APS) was diagnosed. Many years after the spinal cord biopsy, AQP4-ab were tested and found to be positive. We discuss the important differential diagnoses of LETM, give an overview of previously reported NMOSD cases in which a spinal cord biopsy was performed and highlight the crucial role of AQP4-ab testing for the differential diagnosis of longitudinal spinal cord lesions.
  
  
  CONCLUSIONS
  Considering possible serious sequelae of spinal biopsy procedures, testing for AQP4-ab is mandatory in patients with unclear longitudinally extensive spinal cord lesions and should be performed preoperatively in all cases. In light of the heterogeneity of available assays, different detection methods should be used in doubtful cases. The relationship between NMO and APS needs further clarification; however, AQP4 IgG testing is recommended in patients presenting with APS and myelitis, optic neuritis or brainstem encephalitis.
authors.affiliation: Neurologische Klinik, Medizinische Fakultät, Heinrich-Heine-Universität, Universitätsklinikum Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Ringelstein
authors.forename: O
authors.initials: O
authors.lastname: Aktas
authors.forename: J
authors.initials: J
authors.lastname: Harmel
authors.forename: D
authors.initials: D
authors.lastname: Prayer
authors.forename: S
authors.initials: S
authors.lastname: Jarius
authors.forename: B
authors.initials: B
authors.lastname: Wildemann
authors.forename: H-P
authors.initials: HP
authors.lastname: Hartung
authors.forename: S
authors.initials: S
authors.lastname: Salhofer-Polanyi
authors.forename: F
authors.initials: F
authors.lastname: Leutmezer
authors.forename: P S
authors.initials: PS
authors.lastname: Rommer
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009188:Myelitis, Transverse / Q000473:pathology*; D009471:Neuromyelitis Optica / Q000473:pathology*; D009902:Optic Neuritis / Q000473:pathology*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D013116:Spinal Cord / Q000473:pathology*