Long-term follow-up of acute partial transverse myelitis.

BACKGROUND: Acute partial transverse myelitis (APTM) may be the first clinical symptom of multiple sclerosis (MS) or may remain a monophasic event. OBJECTIVES: To evaluate the risk of conversion to MS and long-term disability, and to determine prognosis factors for disability. DESIGN: We identified patients with no previous history of neurological disease who experienced APTM between January 1998 and December 2005 and were followed up at 3 university hospitals in France. Data on the patients' demographics and clinical states during follow-up, as well as data on cerebrospinal fluid (CSF) analysis, brain and spinal cord magnetic resonance imaging (MRI), and visual evoked potentials, were analyzed. SETTING: Neurology departments of 3 university hospitals in Lille, Strasbourg, and Rouen, France, respectively. PATIENTS: A total of 85 patients with no previous history of neurological disease who experienced APTM. RESULTS: The mean (SD) follow-up period was 104.8 (29.8) months. There were 57 women (67%) and 28 men (33%), with a mean (SD) age at onset of 36.7 (11.7) years. At the end of follow-up, 53 patients (62%) were classified as having MS with a mean (SD) Expanded Disability Status Scale score of 2.6 (1.8), 1 patient (1%) was classified as having postinfectious myelitis, 1 (1%) as having neuromyelitis optica, 1 (1%) as having Sjögren syndrome, and 29 (34%) still had APTM of undetermined etiology. Oligoclonal bands in CSF were more frequent in patients with MS (92%) than in patients with APTM of undetermined etiology (38%). Brain MRI results were abnormal in 87% of patients with MS and 27% of patients with APTM of undetermined etiology; visual evoked potentials were abnormal in 43% of patients with MS and 4% of patients with APTM of undetermined etiology. Oligoclonal bands in CSF (odds ratio, 15.76 [95% CI, 2.95-84.24]) and at least 1 MRI-detected brain lesion (odds ratio, 7.74 [95% CI, 2.42-24.74]) were independent predictive factors for conversion to MS. CONCLUSION: Our study confirms that abnormal brain MRI results and the presence of oligoclonal bands in CSF are 2 independent predictive factors for conversion to MS. No clinical, biological, or MRI factor at onset was predictive of long-term disability.journal article2012 MarimportedErratum in : Arch Neurol. 2012 Jun;69(6):789. Outerryck, Olivier [corrected to Outteryck, Olivier]

Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients

BACKGROUND: The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity. OBJECTIVE: To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus. METHODS: Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%). RESULTS: Seropositive patients were found to be predominantly female (p 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome. CONCLUSION: This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients

Pediatric multiple sclerosis: update on diagnostic criteria, imaging, histopathology and treatment choices

Pediatric multiple sclerosis (MS) represents less than 5% of the MS population, but patients with pediatric-onset disease reach permanent disability at a younger age than adult onset patients. Accurate diagnosis at presentation and optimal long-term treatment is vital to mitigate ongoing neuroinflammation and irreversible neurodegeneration. However, it may be difficult to early differentiate pediatric MS from acute disseminated encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD) as they often have atypical presentation that differs from that of adult-onset MS. The purpose of this review is to summarize the updated views on diagnostic criteria, imaging, histopathology and treatment choices

Interleukin-6 receptor blockade in treatment-refractory MOG-IgG-associated disease and neuromyelitis optica spectrum disorders

BACKGROUND AND OBJECTIVES: To evaluate the long-term safety and efficacy of tocilizumab (TCZ), a humanized anti-interleukin-6 receptor antibody in myelin oligodendrocyte glycoprotein-IgG-associated disease (MOGAD) and neuromyelitis optica spectrum disorders (NMOSD). METHODS: Annualized relapse rate (ARR), Expanded Disability Status Scale score, MRI, autoantibody titers, pain, and adverse events were retrospectively evaluated in 57 patients with MOGAD (n = 14), aquaporin-4 (AQP4)-IgG seropositive (n = 36), and seronegative NMOSD (n = 7; 12%), switched to TCZ from previous immunotherapies, particularly rituximab. RESULTS: Patients received TCZ for 23.8 months (median; interquartile range 13.0-51.1 months), with an IV dose of 8.0 mg/kg (median; range 6-12 mg/kg) every 31.6 days (mean; range 26-44 days). For MOGAD, the median ARR decreased from 1.75 (range 0.5-5) to 0 (range 0-0.9; p = 0.0011) under TCZ. A similar effect was seen for AQP4-IgG+ (ARR reduction from 1.5 [range 0-5] to 0 [range 0-4.2]; p < 0.001) and for seronegative NMOSD (from 3.0 [range 1.0-3.0] to 0.2 [range 0-2.0]; p = 0.031). During TCZ, 60% of all patients were relapse free (79% for MOGAD, 56% for AQP4-IgG+, and 43% for seronegative NMOSD). Disability follow-up indicated stabilization. MRI inflammatory activity decreased in MOGAD (p = 0.04; for the brain) and in AQP4-IgG+ NMOSD (p < 0.001; for the spinal cord). Chronic pain was unchanged. Regarding only patients treated with TCZ for at least 12 months (n = 44), ARR reductions were confirmed, including the subgroups of MOGAD (n = 11) and AQP4-IgG+ patients (n = 28). Similarly, in the group of patients treated with TCZ for at least 12 months, 59% of them were relapse free, with 73% for MOGAD, 57% for AQP4-IgG+, and 40% for patients with seronegative NMOSD. No severe or unexpected safety signals were observed. Add-on therapy showed no advantage compared with TCZ monotherapy. DISCUSSION: This study provides Class III evidence that long-term TCZ therapy is safe and reduces relapse probability in MOGAD and AQP4-IgG+ NMOSD

Cadre nosologique et stratégie diagnostique de la myélite aiguë transverse longitudinalement étendue

International audienc

Recommendations for the use of Rituximab in neuromyelitis optica spectrum disorders

International audienc

Efficacy of rituximab in refractory neuromyelitis optica

International audienceBACKGROUND:Despite a growing use of rituximab (RTX) in neuromyelitis optica (NMO), data are lacking in patients with refractory NMO (RNMO), defined as cases with at least one relapse during immunosuppressive therapy.OBJECTIVE:The purpose of this study was to assess RTX as a maintenance therapy in RNMO.METHODS:Out of a total of 305 NMO cases from a population-based cohort, 21 RNMO patients received RTX during a mean follow-up period of 31 months.RESULTS:After RTX, 11 patients (52.3%) were relapse free, meaning that 47.7% were refractory to RTX. The mean annualized relapse rate decreased from 1.3 to 0.4 (p<0.001) and median EDSS from 5 to 3 (p=0.02). Body mass index (BMI) was predictive of EDSS worsening.CONCLUSIONS:RTX is an effective and well-tolerated treatment in RNMO. BMI could be a predictive factor for efficacy

MRI characteristics of MOG-Ab associated disease in adults: An update

International audienceOur knowledge of the radiological spectrum of myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is growing rapidly. An update on the radiological features of the disease, and its evolution is thus necessary. Magnetic resonance imaging (MRI) has an increasingly important role in the differential diagnosis of MOGAD particularly from aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD), and multiple sclerosis (MS). Differentiating these conditions is of prime importance because the management is different between the three inflammatory diseases, and thus could prevent further attack-related disability. Therefore, identifying the MRI features suggestive of MOGAD has diagnostic and prognostic implications. We herein review optic nerve, spinal cord and the brain MRI findings from MOGAD adult patients, and compare them to AQP4-NMOSD and MS