Abstract: P296
Type: Poster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Background: Longitudinally extensive transverse myelitis (LETM) is frequently associated to neuromyelitis optica (NMO) spectrum disorders. However some patients, despite a large work-up, remains negative for any diagnosis, including AQP4 and MOG auto-antibodies (Ab). For this double seronegative LETM patients, NMO criteria are not fulfilled, and data about natural history and treatments recommendation are lacking.
Objectives: To describe clinical, biological and radiological course of patients who experienced a first episode of double seronegative LETM.
Methods: We included patients for whom, despite a comprehensive work-up including MOG-Ab and AQP4-Ab, the final diagnosis was double seronegative LETM with brain MRI at admission not suggestive of multiple sclerosis. The minimum clinical follow-up required was 1 year. Clinical and radiological outcomes were assessed by EDSS, brain and spinal cord MRI at 6, 12, 18, 24 months, when available, and at last visit. The initial work-up including CSF analysis was collected.
Results: 17 patients fulfilled inclusion criteria: 11 women and 6 men. Mean age at episode was 38.6 years (range 16-80). Mean EDSS at nadir was 5.3 (range 1-8). The LETM localisation was as follows: 9 thoracic (53%), 4 whole spinal cord (23%), 2 cervical (12%) and 2 cervico-thoracic (12%). Mean number of white cells in the CSF was 62 (range 1 - 500). Intrathecal synthesis of IgG was positive for only 3/16 patients (19%). All patients received high dose intravenous steroids within a mean of 17 days. Nine patients received second line therapy: plasmapheresis (n=6), additional pulse of steroids (n=2) or IV immunoglobulins (n=1). Mean follow-up was 4.7 years (range 1-11). Improvement was reported for 12 patients (70.5%), with however a mean EDSS at 6 and 12 months at 3.79 and 3.53, respectively. 11 patients (65%) received an immunosuppressive treatment. Among them, only 6 patients were treated at first episode. Six patients (35.3%) (without immunosuppressive treatment) experienced a second relapse during the following year (mean interval: 7.9 months), and one patient at 17 months.
Conclusions: In this cohort, the majority of patients experienced incomplete recovery after a first episode of LETM. In addition, the high rate of relapse in the first year, suggests a more frequently chronic relapsing course than expected. Thus, immunosuppressive treatments should be considered after a first episode of double seronegative LETM.
Disclosure:
Dr Maillart has received funding for travel and honoraria from Biogen, Genzyme, Merck, Novartis, Roche and Teva, with no relation to the submitted work.
Dr Durand-Dubief has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva, with no relation to this study.
Dr Collongues has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva, with no relation to this study.
Dr Kerschen: nothing to disclose
Dr Deschamps: nothing to disclose
Dr Marignier serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva.
Abstract: P296
Type: Poster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Background: Longitudinally extensive transverse myelitis (LETM) is frequently associated to neuromyelitis optica (NMO) spectrum disorders. However some patients, despite a large work-up, remains negative for any diagnosis, including AQP4 and MOG auto-antibodies (Ab). For this double seronegative LETM patients, NMO criteria are not fulfilled, and data about natural history and treatments recommendation are lacking.
Objectives: To describe clinical, biological and radiological course of patients who experienced a first episode of double seronegative LETM.
Methods: We included patients for whom, despite a comprehensive work-up including MOG-Ab and AQP4-Ab, the final diagnosis was double seronegative LETM with brain MRI at admission not suggestive of multiple sclerosis. The minimum clinical follow-up required was 1 year. Clinical and radiological outcomes were assessed by EDSS, brain and spinal cord MRI at 6, 12, 18, 24 months, when available, and at last visit. The initial work-up including CSF analysis was collected.
Results: 17 patients fulfilled inclusion criteria: 11 women and 6 men. Mean age at episode was 38.6 years (range 16-80). Mean EDSS at nadir was 5.3 (range 1-8). The LETM localisation was as follows: 9 thoracic (53%), 4 whole spinal cord (23%), 2 cervical (12%) and 2 cervico-thoracic (12%). Mean number of white cells in the CSF was 62 (range 1 - 500). Intrathecal synthesis of IgG was positive for only 3/16 patients (19%). All patients received high dose intravenous steroids within a mean of 17 days. Nine patients received second line therapy: plasmapheresis (n=6), additional pulse of steroids (n=2) or IV immunoglobulins (n=1). Mean follow-up was 4.7 years (range 1-11). Improvement was reported for 12 patients (70.5%), with however a mean EDSS at 6 and 12 months at 3.79 and 3.53, respectively. 11 patients (65%) received an immunosuppressive treatment. Among them, only 6 patients were treated at first episode. Six patients (35.3%) (without immunosuppressive treatment) experienced a second relapse during the following year (mean interval: 7.9 months), and one patient at 17 months.
Conclusions: In this cohort, the majority of patients experienced incomplete recovery after a first episode of LETM. In addition, the high rate of relapse in the first year, suggests a more frequently chronic relapsing course than expected. Thus, immunosuppressive treatments should be considered after a first episode of double seronegative LETM.
Disclosure:
Dr Maillart has received funding for travel and honoraria from Biogen, Genzyme, Merck, Novartis, Roche and Teva, with no relation to the submitted work.
Dr Durand-Dubief has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva, with no relation to this study.
Dr Collongues has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva, with no relation to this study.
Dr Kerschen: nothing to disclose
Dr Deschamps: nothing to disclose
Dr Marignier serves on scientific advisory board for MedImmune and has received funding for travel and honoraria from Biogen Idec, Merck Serono, Novartis, Sanofi- Genzyme, Roche and Teva.