title: Analysis of prognostic factors associated with longitudinally extensive transverse myelitis.
abstract: OBJECTIVE
  The aim of this study is to report the clinical profile and outcome of longitudinally extensive transverse myelitis (LETM).
  
  
  METHODS
  We prospectively studied adult patients who presented with LETM from January 2008 to December 2011. Information on demographic, clinical course, magnetic resonance imaging (MRI) and outcome was collected. HLA-DRB1 genotype was compared with those of 225 normal controls and patients with MS (228) and neuromyelitis optica (NMO) (22).
  
  
  RESULTS
  In total, 23 patients (16 female) with a median age of 44.5 years (range: 20-77 years) were included. Most (74%) had moderate-severe disability at nadir (48% non-ambulatory), normal/non-multiple sclerosis (MS) brain MRI (96%) and a median MRI cord lesion of 5 vertebral segments (range: 3-19). Laboratory analysis showed cerebrospinal fluid pleocytosis (45%), NMO-IgG (9%), antinuclear antibodies (70%), and genotype HLA-DRB1*13 (57%). The frequency of DRB1*13 genotype was higher compared with controls (p=0.002), MS (p=0.001) and NMO (p=0.003) patients. After a median follow-up of 32 months, one patient converted to MS, two had relapsing LETM with NMO-IgG, and 20 remained as idiopathic with recurrences in four (20%). Twelve (52%) patients recovered with minimal disability (Expanded Disability Status Scale (EDSS) ≤2.5) and three (13%) remained wheelchair dependent. Disability at nadir was associated with the final outcome and extension of the spinal cord lesion with risk of recurrence. Recurrence was not associated with worse outcome.
  
  
  CONCLUSIONS
  Inflammatory LETM is mostly idiopathic with a good outcome. It includes a relatively homogenous group of patients with an overrepresentation of the HLA-DRB1*13 genotype. EDSS at nadir is a predictor of the final outcome and extension of the myelitis of the recurrence risk.
authors.affiliation: Center for Neuroimmunology, Service of Neurology, Hospital Clinic and Institut d'Investigació Biomèdica August Pi i Sunyer, Universitat de Barcelona, Barcelona, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Sepúlveda
authors.forename: Yolanda
authors.initials: Y
authors.lastname: Blanco
authors.forename: Alex
authors.initials: A
authors.lastname: Rovira
authors.forename: Jordi
authors.initials: J
authors.lastname: Rio
authors.forename: Mar
authors.initials: M
authors.lastname: Mendibe
authors.forename: Sara
authors.initials: S
authors.lastname: Llufriu
authors.forename: Iñigo
authors.initials: I
authors.lastname: Gabilondo
authors.forename: Pablo
authors.initials: P
authors.lastname: Villoslada
authors.forename: Joaquin
authors.initials: J
authors.lastname: Castilló
authors.forename: Juan
authors.initials: J
authors.lastname: Corral
authors.forename: Teresa
authors.initials: T
authors.lastname: Ayuso
authors.forename: Cristina
authors.initials: C
authors.lastname: Iñiguez
authors.forename: Sonia
authors.initials: S
authors.lastname: Santos
authors.forename: Cristina
authors.initials: C
authors.lastname: Guijarro
authors.forename: Lluis
authors.initials: L
authors.lastname: Ramió-Torrentà
authors.forename: Angel P
authors.initials: AP
authors.lastname: Sempere
authors.forename: Javier
authors.initials: J
authors.lastname: Olascoaga
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.forename: Xavier
authors.initials: X
authors.lastname: Montalban
authors.forename: Albert
authors.initials: A
authors.lastname: Saiz
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000134:cerebrospinal fluid*; D015415:Biomarkers / Q000134:cerebrospinal fluid; D001921:Brain / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D016022:Case-Control Studies; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D005787:Gene Frequency; D005838:Genotype; D059811:HLA-DRB1 Chains / Q000235:genetics*; D006801:Humans; D007964:Leukocytosis / Q000134:cerebrospinal fluid / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D010641:Phenotype; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012008:Recurrence; D013030:Spain; D013116:Spinal Cord / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; D059811:HLA-DRB1 Chains; C506473:HLA-DRB1*13 antigen