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1.
Eur Radiol ; 34(3): 1453-1460, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37668695

RESUMEN

OBJECTIVES: Optic nerve head edema (ONHE) detected by fundoscopy is observed in one-third of patients presenting optic neuritis (ON). While ONHE is an important semiological feature, the correlation between ONHE and optic nerve head MRI abnormalities (ONHMA), sometimes called "optic nerve head swelling," remains unknown. Our study aimed to assess the diagnostic accuracy of T2 fluid-attenuated inversion recovery (FLAIR) MRI sequence in detecting ONHE in patients with acute ON. METHODS: In the present single-center study, data were extracted from two prospective cohort studies that consecutively included adults with a first episode of acute ON treated between 2015 and 2020. Two experienced readers blinded to study data independently analyzed imaging. A senior neuroradiologist resolved any discrepancies. The primary judgment criterion of ONHMA was assessed as optic nerve head high signal intensity on gadolinium-enhanced T2FLAIR MRI sequence. Its diagnostic accuracy was evaluated with both the gold standard of ONHE on fundus photography (FP) and peripapillary retinal nerve fiber layer thickening on optic coherence tomography (OCT). RESULTS: A total of 102 patients were included, providing 110 affected and 94 unaffected optic nerves. Agreement was high between the different modalities: 92% between MRI and FP (k = 0.77, 95% CI: 0.67-0.88) and 93% between MRI and OCT (k = 0.77, 95% CI: 0.67-0.87). MRI sensitivity was 0.84 (95% CI: 0.70-0.93) and specificity was 0.94 (95% CI: 0.89-0.97) when compared with the FP. CONCLUSION: Optic nerve head high T2FLAIR signal intensity corresponds indeed to the optic nerve head edema diagnosed by the ophthalmologists. MRI is a sensitive tool for detecting ONHE in patients presenting acute ON. CLINICAL RELEVANCE STATEMENT: In patients with optic neuritis the high T2FLAIR (fluid-attenuated inversion recovery) signal intensity of the optic nerve head corresponds indeed to optic nerve head edema, which is a useful feature in optic neuritis etiological evaluation and treatment. KEY POINTS: Optic nerve head edema is a prominent clinical feature of acute optic neuritis and is usually diagnosed during dilated or non-dilated eye fundus examination. Agreement was high between magnetic resonance imaging, fundus photography, and optical coherence tomography. Optic nerve head high T2 fluid attenuation inversion recovery signal intensity is a promising detection tool for optic nerve head edema in patients presenting acute optic neuritis.


Asunto(s)
Disco Óptico , Neuritis Óptica , Adulto , Humanos , Disco Óptico/patología , Estudios Prospectivos , Neuritis Óptica/complicaciones , Neuritis Óptica/diagnóstico por imagen , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Tomografía de Coherencia Óptica/métodos , Edema/diagnóstico por imagen , Edema/patología
2.
J Neurol Neurosurg Psychiatry ; 94(4): 309-313, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36600666

RESUMEN

BACKGROUND: To characterise the response to treatment of inaugural optic neuritis (ON) in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). METHODS: We searched the French MOGAD database for adults with inaugural ON with a detailed report of acute treatment modalities and measures of high-contrast best-corrected visual acuity (BCVA) at nadir and after 3 months. Predictors of visual outcomes were assessed by multivariable analysis. RESULTS: Among 245 patients with at least one episode of ON, 82 fulfilled all criteria, and data on the peripapillary retinal nerve fibre layer (pRNFL) were available for 44. All patients received methylprednisolone (MP), combined with plasma exchange in 18. After 3 months, 75 of 82 (91%) patients retained full BCVA recovery, and median (range) pRNFL of the affected eye was 72 µm (40-102). Failure to regain 0.0 logarithmic minimum angle of resolution vision (Snellen 20/20) at 3 months was associated with time to first MP treatment ≥10 days (OR 16, 95% CI 1.14 to 213, p=0.01). pRNFL thickness after 3 months was related to better BCVA at nadir and time to first MP treatment <10 days (r2=19%, p=0.004 and r2=11%, p=0.03, respectively). CONCLUSIONS: Time to MP affects functional but also structural visual outcomes of ON in MOGAD.


Asunto(s)
Neuritis Óptica , Humanos , Retina , Metilprednisolona/uso terapéutico , Agudeza Visual , Tomografía de Coherencia Óptica , Glicoproteína Mielina-Oligodendrócito , Autoanticuerpos
3.
Mult Scler ; 29(2): 270-276, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36453174

RESUMEN

BACKGROUND AND OBJECTIVE: Myelin-oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) frequently initiates during childbearing years. This study investigated the impact of pregnancy and post-partum on MOGAD activity. METHODS: Retrospective analysis of clinical and demographic data from a multicenter French cohort of adult patients with MOGAD. All adult female patients who had a pregnancy after disease onset or in the year before disease onset were included. The annualized relapse rate was evaluated in patients who had a pregnancy after disease onset, to evaluate the impact of pregnancy and post-partum on MOGAD course. RESULTS: Twenty-five informative pregnancies after disease onset were identified. No relapse was recorded during these pregnancies and only three relapses occurred during the first 3 months post-partum. The annualized relapse rate decreased from 0.67 (95% confidence interval: 0.40-1.10) during the pre-pregnancy period to 0 (95% confidence interval: 0-0.21) during pregnancy and to 0.22 (95% confidence interval: 0.09-0.53) during the first year post-partum. Among 144 female patients in their childbearing age recorded in the database, 18 (12.5%) reported their first symptoms during pregnancy or in the 12 months post-partum. DISCUSSION: Our study suggests a marked reduction of MOGAD relapse rate during pregnancy and the post-partum period. Prospective studies on the role of pregnancy and delivery in MOGAD course are needed.


Asunto(s)
Autoanticuerpos , Periodo Posparto , Embarazo , Humanos , Femenino , Glicoproteína Mielina-Oligodendrócito , Estudios Retrospectivos , Estudios Prospectivos , Recurrencia
4.
Mult Scler ; 29(1): 11-36, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-36317497

RESUMEN

OBJECTIVE: The objective of this study was to develop evidence-based recommendations on pregnancy management for persons with multiple sclerosis (MS). BACKGROUND: MS typically affects young women in their childbearing years. Increasing evidence is available to inform questions raised by MS patients and health professionals about pregnancy issues. METHODS: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and university databases (January 1975 through June 2021). The RAND/UCLA appropriateness method was developed to synthesise the scientific literature and expert opinions on healthcare topics; it was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. RESULTS: A strong agreement was reached for all 104 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, locoregional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses and disease-modifying treatments. CONCLUSION: The 2022 recommendations of the French MS society should be helpful to harmonise counselling and treatment practice for pregnancy in persons with MS, allowing for better and individualised choices.


Asunto(s)
Esclerosis Múltiple , Complicaciones del Embarazo , Embarazo , Humanos , Femenino , Esclerosis Múltiple/terapia , Periodo Posparto , Vacunación , Complicaciones del Embarazo/terapia , Recurrencia
5.
Mult Scler ; 29(1): 37-51, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-36345839

RESUMEN

BACKGROUND: In 2020, the French Multiple Sclerosis (MS) Society (SFSEP) decided to develop a national evidence-based consensus on pregnancy in MS. As neuromyelitis optica spectrum disorders (NMOSD) shares a series of commonalities with MS, but also some significant differences, specific recommendations had to be developed. OBJECTIVES: To establish recommendations on pregnancy in women with NMOSD. METHODS: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and universities databases (January 1975 through June 2021). The RAND/UCLA appropriateness method, which was developed to synthesise the scientific literature and expert opinions on health care topics, was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A sub-group of nine NMOSD experts was dedicated to analysing available data on NMOSD. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. RESULTS: A strong agreement was reached for all 66 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, loco-regional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses, and disease-modifying treatments. CONCLUSION: Physicians and patients should be aware of the new and specific evidence-based recommendations of the French MS Society for pregnancy in women with NMOSD. They should help harmonise counselling and treatment practise, allowing for better individualised choices.


Asunto(s)
Esclerosis Múltiple , Neuromielitis Óptica , Embarazo , Humanos , Femenino , Neuromielitis Óptica/diagnóstico , Neuromielitis Óptica/terapia , Esclerosis Múltiple/diagnóstico , Esclerosis Múltiple/terapia , Vacunación , Periodo Posparto , Recurrencia
6.
Eur J Neurol ; 30(9): 2865-2869, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37243935

RESUMEN

BACKGROUND: The kappa free light chains index (κ-index) is increasing in importance as a fast, easy, cost-effective, and quantitative biomarker in multiple sclerosis (MS), which can replace cerebrospinal fluid (CSF)-restricted oligoclonal bands (OCB) detection. In previous studies, controls often included mixed patients with several inflammatory central nervous system disorders. The aim of the present study was to assess the κ-index in patients with serum aquaporin-4 (AQP4)-IgG or myelin-oligodendrocyte-glycoprotein (MOG)-IgG. METHODS: We analyzed CSF/serum samples of patients with AQP4-IgG or MOG-Ig and evaluated distinct κ-index cut-offs. We described clinical and magnetic resonance imaging (MRI) features of patients with the highest κ-index values. RESULTS: In 11 patients with AQP4-IgG, median κ-index was 16.8 (range 0.2; 63) and 6/11 (54.5%) had κ-index >12. Among 42 patients with MOG-IgG, 2 had low positive MOG-IgG titers, were ultimately diagnosed with MS, and had a markedly increased κ-index (54.1 and 102.5 respectively). For the remaining 40 MOG-IgG-positive patients the median κ-index was 0.3 (range 0.1; 15.5). Some 6/40 (15%) and 1/40 (2.5%) patients had a κ-index >6 and >12, respectively. None fulfilled MRI dissemination in space and dissemination in time (DIS/DIT) criteria and the final diagnosis was MOG-IgG-associated disease (MOGAD) for these 40 patients. Four of the 40 (10%) MOG-IgG-positive patients had OCB. CONCLUSION: While a marked increase in κ-index could discriminate MS from MOGAD, a low κ-index threshold could lead to confusion between MS and MOGAD or AQP4 antibody-positive neuromyelitis optica spectrum disorder.


Asunto(s)
Esclerosis Múltiple , Neuromielitis Óptica , Humanos , Glicoproteína Mielina-Oligodendrócito , Vaina de Mielina , Acuaporina 4 , Neuromielitis Óptica/diagnóstico por imagen , Esclerosis Múltiple/diagnóstico por imagen , Autoanticuerpos , Inmunoglobulina G
7.
Ann Neurol ; 89(1): 30-41, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32959427

RESUMEN

OBJECTIVE: The main objective was to compare clinical features, disease course, and myelin oligodendrocyte glycoprotein (MOG) antibody (Ab) dynamics between children and adults with MOG-Ab-associated disease (MOGAD). METHODS: This retrospective multicentric, national study included 98 children and 268 adults with MOGAD between January 2014 and September 2019. Cox regression model for recurrent time-to-event data and Kaplan-Meier curves for time to antibody negativity were performed for the objectives. RESULTS: Isolated optic neuritis was the most frequent clinical presentation in both children (40.8%) and adults (55.9%, p = 0.013), and acute disseminated encephalomyelitis syndrome was more frequent in children (36.7% vs 5.6%, p < 0.001). Compared to adults, children displayed better recovery (Expanded Disability Status Scale ≥ 3.0 at last follow-up reached only by 10 of 97 [10.3%] vs 66/247 [26.7%], p < 0.001). In the multivariate analysis, adults were at higher risk of relapse than children (hazard ratio = 1.41, 95% confidence interval [CI] = 1.12-1.78, p = 0.003). At 2 years, 64.2% (95% CI = 40.9-86.5) of nonrelapsing children became MOG-Ab negative compared to 14.1% (95% CI = 4.7-38.3) of relapsing children (log-rank p < 0.001), with no differences observed in adults (log-rank p = 0.280). INTERPRETATION: MOGAD patients differ in the clinical presentation at onset, showing an age-related shift in the clinical features across age groups. Compared to children, adults have a higher risk of relapse and worse functional recovery. Finally, children with monophasic disease become MOG-Ab negative earlier than relapsing children, but this is not true in adults. Considering these differences, management and treatment guidelines should be considered independently in children and adults. ANN NEUROL 2021;89:30-41.


Asunto(s)
Acuaporina 4/inmunología , Autoanticuerpos/inmunología , Glicoproteína Mielina-Oligodendrócito/metabolismo , Neuritis Óptica/diagnóstico , Adolescente , Adulto , Factores de Edad , Enfermedad Crónica , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Neuritis Óptica/inmunología , Neuritis Óptica/terapia , Factores de Riesgo , Adulto Joven
8.
Eur J Neurol ; 29(9): 2781-2791, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35617154

RESUMEN

BACKGROUND AND PURPOSE: This study was undertaken to determine the role of optical coherence tomography (OCT) in predicting the final visual and structural outcome, and to evaluate the correlation between functional eye outcome and retinal changes, in patients with a first episode of optic neuritis (ON). METHODS: In this prospective study, consecutive adult patients with acute ON underwent ophthalmological evaluation at baseline and at 1 and 12 months, including OCT measurements of peripapillary retinal nerve fiber layer (pRNFL), macular ganglion cell and inner plexiform layer, and inner nuclear layer thicknesses; high- and low-contrast visual acuity; visual field assessment; and baseline brain magnetic resonance imaging. Univariate and multivariate linear regressions were used to assess predictive factors of outcome. Correlations between 12-month visual function and retinal structure were estimated by Spearman coefficients. Two groups of patients were analyzed, with or without multiple sclerosis (MS). RESULTS: Among 116 patients, 79 (68.1%) had MS, and 37 (31.9%) had ON not related to MS (including 19 idiopathic [i.e., isolated] ON, and 13 and five with myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies, respectively). We found no independent predictive factor of visual and retinal outcome. Analysis of the relationship between the visual field test (mean deviation) and pRNFL thickness demonstrated a threshold of 75.4 µm and 66.4 µm, below which the mean deviation was worse, for patients with MS (p = 0.007) and without MS (p < 0.001), respectively. CONCLUSIONS: We found that inner retinal layer measurements during the first month are not predictive of final outcome. The critical threshold of axonal integrity, below which visual function is damaged, is different between patients with and without MS.


Asunto(s)
Esclerosis Múltiple , Neuritis Óptica , Humanos , Estudios Longitudinales , Pronóstico , Estudios Prospectivos , Tomografía de Coherencia Óptica/métodos , Trastornos de la Visión
9.
Mult Scler ; 27(6): 855-863, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-32633601

RESUMEN

BACKGROUND: A paradoxical discrepancy between severe peripapillary retinal nerve fiber layer (pRNFL) atrophy and good visual outcome had been reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated optic neuritis (ON). However, only visual acuity (VA) was assessed. OBJECTIVES: To study visual field (VF) outcomes of patients with MOG-IgG-associated ON and evaluate the correlation between functional eye outcome and retinal structural changes assessed by optical coherence tomography. METHODS: The records of 32 patients with MOG-IgG-associated ON who underwent ophthalmological examination at least 12 months after ON onset were reviewed. Degree of VF disability was determined by mean deviation (MD). RESULTS: At final assessment (median, 35 months), 4.2% of 48 affected eyes (AE) had VA ⩽ 0.1, 40% had abnormal MD, and among AE with final VA ⩾ 1.0, 31% had mild to moderate damage. Thinning of the inner retinal layers was significantly correlated with MD impairment. Analysis demonstrated a threshold of pRNFL thickness (50 µm), below which MD was significantly worse (mean, -2.27 dB vs -17.72 dB; p = 0.0003). ON relapse was significantly associated with poor visual outcome assessed by MD. CONCLUSION: Functional impairment measured with VF is not rare, and MD assessment better reflects actual structural damage.


Asunto(s)
Neuritis Óptica , Campos Visuales , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudios Retrospectivos , Relación Estructura-Actividad
10.
Eur J Neurol ; 28(5): 1659-1664, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33528851

RESUMEN

BACKGROUND AND OBJECTIVE: The prognosis in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a matter of debate. Our aim was to assess the long-term outcomes of patients with MOGAD. METHODS: We retrospectively analysed the clinical and paraclinical data of patients from the French nationwide observatory study NOMADMUS who tested positive for MOG antibodies (MOG-IgG) and who had clinical follow-up of at least 8 years from their first episode. RESULTS: Sixty-one patients (median [range] age at onset 27 [3-69] years), with a median (mean; range) follow-up of 177 (212.8; 98-657) months, were included. Among 58 patients with a relapsing course, 26.3% relapsed in the first year after onset. Of the 61 patients, 90.2% experienced at least one episode of optic neuritis. At last visit, the median (mean; range) Expanded Disability Status Scale (EDSS) score was 1 (2.12; 0-7.5), 12.5% had an EDSS score ≥6 and 37.5% had an EDSS score ≥3. Of 51 patients with final visual acuity (VA) data available, 15.7% had VA ≤0.1 in at least one eye and 25.5% had VA ≤0.5 in at least one eye. Bilateral blindness (VA ≤0.1) was present in 5.9% of patients. Finally, 12.5% of patients presented bladder dysfunction requiring long-term urinary catheterization. No factor associated significantly with a final EDSS score ≥3 or with final VA ≤0.1 was found. CONCLUSION: Overall long-term favourable outcomes were achieved in a majority of our patients, but severe impairment, in particular visual damage, was not uncommon.


Asunto(s)
Acuaporina 4 , Neuritis Óptica , Autoanticuerpos , Estudios de Cohortes , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudios Retrospectivos
11.
J Neuroinflammation ; 17(1): 128, 2020 Apr 23.
Artículo en Inglés | MEDLINE | ID: mdl-32326965

RESUMEN

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. METHODS: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. RESULTS: Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). CONCLUSIONS: A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.


Asunto(s)
Mielitis Transversa/inmunología , Mielitis Transversa/patología , Recuperación de la Función , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Acuaporina 4/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Estudios de Cohortes , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Glicoproteína Mielina-Oligodendrócito/inmunología , Mielitis Transversa/terapia , Plasmaféresis/métodos , Pronóstico , Recurrencia , Factores de Riesgo , Adulto Joven
12.
Mult Scler ; 26(11): 1441-1443, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32539531

RESUMEN

An 87-year-old man presented with a 2-day history of painful bilateral visual loss. On examination, exophthalmos, lid edema, chemosis, and optic disc edema, on the left side only, were found. Visual acuity was 4/10 OD and no light perception OS. Magnetic resonance imaging (MRI) revealed bilateral optic neuritis and a diffuse and severe infiltration of the intra- and extraconal fat on the left. Laboratory testing was negative except for serum myelin oligodendrocyte glycoprotein (MOG) antibodies. This presentation adds a new variant to the MOG-associated disease spectrum. Testing for MOG antibodies should be considered in patients presenting with diffuse orbital inflammation and optic neuritis.


Asunto(s)
Autoanticuerpos , Neuritis Óptica , Anciano de 80 o más Años , Humanos , Inflamación , Masculino , Glicoproteína Mielina-Oligodendrócito , Neuritis Óptica/diagnóstico por imagen , Agudeza Visual
13.
Mult Scler ; 26(8): 936-944, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-31148523

RESUMEN

OBJECTIVES: We aim to (1) determine the frequency and distinctive features of short myelitis (SM) and longitudinally extensive transverse myelitis (LETM) in a cohort of adults with myelin oligodendrocyte glycoprotein (MOG)-antibody (Ab)-associated myelitis and (2) determine baseline prognostic factors among MOG-Ab-positive patients whose disease started with myelitis. MATERIAL AND METHODS: We retrospectively analyzed clinical and paraclinical variables from a multicentric French cohort of adults with MOG-Ab-associated myelitis. At last follow-up, patients were classified into two groups according to the severity of the Expanded Disability Status Scale (EDSS) as ⩽2.5 or ⩾3.0. RESULTS: Seventy-three patients with at least one episode of myelitis over disease course were included; among them, 28 (38.4%) presented with SM at the time of the first myelitis. Motor and sphincter involvement was less frequently observed in SM (51.9% and 48.2%, respectively) than in LETM patients (83.3% and 78.6%, respectively), p = 0.007 and p = 0.017; 61% of LETM patients displayed brain lesions compared to 28.6% in the SM group, p = 0.008, and the thoracic segment was more frequently involved in the LETM (82.2%) than in the SM group (39.3%), p < 0.001. EDSS at last follow-up was higher in LETM (median 3.0 (interquartile range: 2.0-4.0)) compared to SM patients (2.0, (1.0-3.0)), p = 0.042. Finally, a higher EDSS at onset was identified as the only independent risk factor for EDSS ⩾3.0 (odds ratio, 1.40, 95% confidence interval (CI): 1.01-1.95, p = 0.046). CONCLUSION: SM in MOG-Ab-associated disease is not rare. The severity at onset was the only independent factor related to the final prognosis in MOG-Ab-associated myelitis.


Asunto(s)
Autoanticuerpos , Progresión de la Enfermedad , Glicoproteína Mielina-Oligodendrócito/inmunología , Mielitis , Sistema de Registros , Índice de Severidad de la Enfermedad , Adulto , Anciano , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Mielitis/diagnóstico , Mielitis/inmunología , Mielitis/patología , Mielitis/fisiopatología , Pronóstico , Estudios Retrospectivos , Adulto Joven
14.
J Neuroinflammation ; 16(1): 134, 2019 Jul 02.
Artículo en Inglés | MEDLINE | ID: mdl-31266527

RESUMEN

BACKGROUND: Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are related to several acquired demyelinating syndromes in adults, but the therapeutic approach is currently unclear. We aimed to describe the response to different therapeutic strategies in adult patients with relapsing MOG-Ab-associated disease. METHODS: This is a retrospective study conducted in France and Spain including 125 relapsing MOG-Ab patients aged ≥ 18 years. First, we performed a survival analysis to investigate the relapse risk between treated and non-treated patients, performing a propensity score method based on the inverse probability of treatment weighting. Second, we assessed the annualised relapse rates (ARR), Expanded Disability Status Scale (EDSS) and visual acuity pre-treatment and on/end-treatment. RESULTS: Median age at onset was 34.1 years (range 18.0-67.1), the female to male ratio was 1.2:1, and 96% were Caucasian. At 5 years, 84% (95% confidence interval [CI], 77.1-89.8) patients relapsed. At the last follow-up, 66 (52.8%) received maintenance therapy. Patients initiating immunosuppressants (azathioprine, mycophenolate mophetil [MMF], rituximab) were at lower risk of new relapse in comparison to non-treated patients (HR, 0.41; 95CI%, 0.20-0.82; p = 0.011). Mean ARR (standard deviation) was reduced from 1.05(1.20) to 0.43(0.79) with azathioprine (n = 11; p = 0.041), from 1.20(1.11) to 0.23(0.60) with MMF (n = 11; p = 0.033), and from 1.08(0.98) to 0.43(0.89) with rituximab (n = 26; p = 0.012). Other immunosuppressants (methotrexate/mitoxantrone/cyclophosphamide; n = 5), or multiple sclerosis disease-modifying drugs (MS-DMD; n = 9), were not associated with significantly reduced ARR. Higher rates of freedom of EDSS progression were observed with azathioprine, MMF or rituximab. CONCLUSION: In adults with relapsing MOG-Ab-associated disease, immunosuppressant therapy (azathioprine, MMF and rituximab) is associated with reduced risk of relapse and better disability outcomes. Such an effect was not found in the few patients treated with MS-DMD.


Asunto(s)
Autoanticuerpos/sangre , Inmunosupresores/uso terapéutico , Glicoproteína Mielina-Oligodendrócito/sangre , Neuromielitis Óptica/sangre , Neuromielitis Óptica/tratamiento farmacológico , Adolescente , Adulto , Anciano , Estudios de Cohortes , Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple Recurrente-Remitente/sangre , Esclerosis Múltiple Recurrente-Remitente/diagnóstico , Esclerosis Múltiple Recurrente-Remitente/tratamiento farmacológico , Neuromielitis Óptica/diagnóstico , Estudios Retrospectivos , Rituximab/uso terapéutico , Resultado del Tratamiento , Adulto Joven
16.
J Neuroradiol ; 46(5): 312-318, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31228536

RESUMEN

The spectrum of Myelin Oligodendrocytes Glycoprotein (MOG) antibody disease constitutes a recently described challenging entity, referring to a relatively new spectrum of autoimmune disorders with antibodies against MOG predominantly involving the optic nerve and spinal cord. The purpose of this article is to describe MRI features of MOG-AD involvement in the optic nerves, spinal cord and the brain of adults.


Asunto(s)
Enfermedades Desmielinizantes/diagnóstico por imagen , Enfermedades Desmielinizantes/patología , Imagen por Resonancia Magnética , Adolescente , Adulto , Autoanticuerpos , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Enfermedades Desmielinizantes/inmunología , Encefalitis/diagnóstico por imagen , Encefalitis/inmunología , Encefalitis/patología , Femenino , Humanos , Masculino , Glicoproteína Mielina-Oligodendrócito/inmunología , Mielitis/diagnóstico por imagen , Mielitis/inmunología , Mielitis/patología , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Neuritis Óptica/diagnóstico por imagen , Neuritis Óptica/inmunología , Neuritis Óptica/patología , Médula Espinal/diagnóstico por imagen , Médula Espinal/patología , Adulto Joven
17.
Pract Neurol ; 18(2): 159-161, 2018 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-29208730

RESUMEN

IgG4-related disease is now recognised as an important cause of intracranial and spinal hypertrophic pachymeningitis. Treatment with corticosteroids generally leads to significant clinical improvement. We present two cases of IgG4 pachymeningitis unresponsive to corticosteroids who improved with rituximab.


Asunto(s)
Inmunoglobulina G , Factores Inmunológicos/uso terapéutico , Meningitis/tratamiento farmacológico , Rituximab/uso terapéutico , Corticoesteroides/uso terapéutico , Adulto , Femenino , Humanos , Masculino , Meningitis/inmunología , Persona de Mediana Edad , Recurrencia
18.
J Neurol Neurosurg Psychiatry ; 85(9): 1038-40, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24876183

RESUMEN

OBJECTIVE: To characterise recurrence of multiple sclerosis (MS) inflammatory activity during the year following natalizumab (NTZ) cessation. METHODS: Thirty-two patients with MS were included in a monocentric cohort study. Data were collected prospectively during and after NTZ, with serial clinical and MRI evaluations. The first relapse occurring after interrupting NTZ was the primary outcome measure. The numbers of gadolinium-enhancing lesions before, during and after NTZ treatment, were compared. RESULTS: During the year following NTZ cessation, the cumulative probability of relapses was estimated at 52.9% and an unusually high MRI inflammation was noticed. It was defined by a number of gadolinium-enhancing lesions >5 and exceeding the gadolinium lesions existing before NTZ initiation. Rebound of MS activity after NTZ cessation was characterised by association of relapses and unusual MRI inflammation. Cumulative probability of rebound was estimated at 39% and mostly occurring between 3 months and 9months after interrupting NTZ. Risk of rebound appears related with a higher annualised relapse rate and a lower Expanded Disability Status Scale score before NTZ initiation. Rebound was associated with severe recurring relapses in 9% of the patients. CONCLUSIONS: This study identifies rebound after NTZ cessation as an association of relapses and high MRI activity.


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Inflamación/complicaciones , Inflamación/etiología , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/tratamiento farmacológico , Adulto , Encéfalo/patología , Femenino , Gadolinio , Humanos , Inflamación/patología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/patología , Natalizumab , Neuroimagen , Estudios Prospectivos , Recurrencia
19.
Artículo en Inglés | MEDLINE | ID: mdl-38902008

RESUMEN

BACKGROUND AND PURPOSE: MR imaging is the technique of choice for patients presenting with acute loss of visual acuity with no obvious ophthalmologic cause. The goal of our study was to compare orbits contrast-enhanced 2D coronal T1WI with a whole-brain contrast-enhanced 3D (WBCE-3D) TSE T1WI at 3T for the detection of optic nerve enhancement. MATERIALS AND METHODS: This institutional review board-approved retrospective single-center study included patients presenting with acute loss of vision who underwent 3T MR imaging from November 2014 to February 2020. Two radiologists, blinded to all data, individually assessed the presence of enhancement of the optic nerve on orbits contrast-enhanced 2D T1WI and WBCE-3D T1WI separately and in random order. A McNemar test and a Cohen κ method were used for comparing the 2 MR imaging sequences. RESULTS: One thousand twenty-three patients (638 women and 385 men; mean age, 42 [SD, 18.3] years) were included. There was a strong concordance between WBCE-3D T1WI and orbits contrast-enhanced 2D T1WI when detecting enhancement of the optic nerve: κ = 0.87 (95% CI, 0.84-0.90). WBCE-3D T1WI was significantly more likely to detect canalicular enhancement compared with orbits contrast-enhanced 2D T1WI: 178/1023 (17.4%) versus 138/1023 (13.5%) (P < .001) and 108/1023 (10.6%) versus 90/1023 (8.8%) (P = .04), respectively. The WBCE-3D T1WI sequence detected 27/1023 (3%) instances of optic disc enhancement versus 0/1023 (0%) on orbits contrast-enhanced 2D T1WI. There were significantly fewer severe artifacts on WBCE-3D T1WI compared with orbits contrast-enhanced 2D T1WI: 68/1023 (6.6%) versus 101/1023 (9.8%) (P < .001). The median reader-reported confidence was significantly higher with coronal T1WI compared with 3D TSE T1WI: 5 (95% CI, 4-5) versus 3 (95% CI, 1-4; P < .001). CONCLUSIONS: Our study showed that there was a strong concordance between WBCE-3D T1WI and orbits contrast-enhanced 2D T1WI when detecting enhancement of the optic nerve in patients with acute loss of visual acuity with no obvious ophthalmologic cause. WBCE-3D T1WI demonstrated higher sensitivity and specificity in diagnosing optic neuritis, particularly in cases involving the canalicular segments.

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