Analysis of prognostic factors associated with longitudinally extensive transverse myelitis

作者：Sepulveda Maria; Blanco Yolanda; Rovira Alex; Rio Jordi; Mendibe Mar; Llufriu Sara; Gabilondo Inigo; Villoslada Pablo; Castillo Joaquin; Corral Juan; Ayuso Teresa; Iniguez Cristina; Santos Sonia; Guijarro Cristina; Ramio Torrenta Lluis; Sempere Angel P; Olascoaga Javier; Graus Francesc; Montalban Xavier; Saiz Albert^*

来源：Multiple Sclerosis, 2013, 19(6): 742-748.

DOI：10.1177/1352458512461968

• Transverse myelitis
• multiple sclerosis
• neuromyelitis optica
• MRI
• HLA

Objective: The aim of this study is to report the clinical profile and outcome of longitudinally extensive transverse myelitis (LETM). %26lt;br%26gt;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). %26lt;br%26gt;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) %26lt;= 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. %26lt;br%26gt;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.

• 出版日期2013-5