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M Bouyon,
N Collongues,
H Zéphir,
L Ballonzoli,
L Jeanjean,
C Lebrun,
Jb Chanson,
F Blanc,
M Fleury,
O Outteryck,
S Defoort,
P Labauge, P Vermersch,
C Speeg,
J De Seze
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ABSTRACT: BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an interesting method for the evaluation of disease severity; however, to date there are no studies with a longitudinal follow-up of visual function in NMO. The aim of this study was to assess the ability of OCT to evaluate the progression of visual dysfunction in NMO. PATIENTS AND METHODS: A group of 30 NMO patients (thus, 60 eyes), comprised of 20 women and 10 men with a mean age of 43.7 +/- 12.3 years, were prospectively evaluated clinically and by a whole neuro-ophthalmological work-up, including: visual acuity (VA), fundoscopy, visual evoked potential (VEP), visual field (VF) and optical coherence tomography (OCT). All patients were tested at baseline (after a mean disease duration of 6.1 years) and after a mean time of follow-up of 18 months (range: 12-36 months). RESULTS: Mean VA was similar at the two evaluation times (0.77 +/- 0.36 versus 0.77 +/- 0.35). The mean VF defect decreased slightly, but the difference was not significant (-5.9 +/- 1.3 dB versus -5.3 +/- 1.3 dB). In contrast, the mean retinal thickness seen on OCT decreased from 87.4 +/- 23.3 µm to 79.7 +/- 22.4 µm (p = 0.006). These modifications were only observed in eyes with a past or a recent history of optic neuritis (-15.1 µm; p < 0.001) and not in eyes without any history of optic neuritis (-2.4 µm; not significant). Also, they occurred independently of the occurrence of relapses (n = 13) and especially optic neuritis episodes; however, the number of optic neuritis episodes was low (n = 5). CONCLUSION: OCT seems to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis. Our results suggest that this easily performed technique should be used in the follow-up of NMO, but complementary studies are warranted to confirm its interest at an individual level.
Multiple Sclerosis 02/2013; · 4.26 Impact Factor
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J Hodel,
P Besson,
O Outteryck,
H Zéphir,
D Ducreux,
A Monnet,
D Chéchin,
M Zins,
M Rodallec,
J P Pruvo, P Vermersch,
X Leclerc
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ABSTRACT: BACKGROUND AND PURPOSE:DTI is a promising technique for imaging of the spinal cord, but the technique has susceptibility-induced artifacts. We evaluated a pulse-triggered DTI sequence with an rFOV technique and coronal acquisition for the assessment of the cervical spinal cord in patients with myelitis at 3T.MATERIALS AND METHODS:A rFOV acquisition was established by a noncoplanar application of the excitation and the refocusing pulse in conjunction with outer volume suppression. The DTI sequence was performed in the coronal plane in 12 healthy volunteers and 40 consecutive patients with myelitis. Probabilistic tractography of the posterior and lateral funiculi was performed from the C1 to C7 levels. FA, MD, aD, rD, and ratios of aD and rD were measured.RESULTS:In healthy volunteers, mean DTI indices within the whole-fiber pathways were the following: FA = 0.61, MD = 1.17 × 10(-3) mm(2)/s, aD = 1.96 × 10(-3) mm(2)/s, rD = 0.77 × 10(-3) mm(2)/s, and ratios of aD and rD = 2.5. Comparison of healthy controls and patients with myelitis identified statistically significant differences for all DTI parameters. Different patterns of myelitis, including spinal cord atrophy and active inflammatory lesions, were recognized. There was a significant correlation between clinical severity and DTI parameters.CONCLUSIONS:The present work introduces a new approach for DTI of the cervical spinal cord at 3T, enabling a quantitative follow-up of patients with myelitis.
American Journal of Neuroradiology 08/2012; · 2.93 Impact Factor
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ABSTRACT: The impact of autoimmunity on malaria-infection evolution reported by various works has led us to compare reactive patterns of self-dependent systemic IgG from 54 patients aged less than 15 years old to those from 46 subjects older than 15 years. These subjects were divided into 34 Plasmodium falciparum asymptomatic carriers (ACs), 30 cases of uncomplicated malaria (UM), and 36 patients suffering from cerebral malaria (CM) living in the same endemic area. The reactivity of the plasma antibodies against human brain tissue extract was assessed by western blotting. Comparative analysis of reactive bands (linear discriminant analysis, LDA) revealed the existence of patterns that distinguish, among the more susceptible subjects aged less than 15 years old, the different clinical forms. In contrast, in less susceptible subjects aged more than 15 years old, the patterns are homogenous and do not allow the separation of these clinical forms. This self-reactive repertoire might be witnessed as an imprint of the clinical tolerance acquired during the years of living in endemic areas. The singularity of this profile under the age of 15 years might have a prognostic value.
Bulletin de la Société de pathologie exotique 08/2012; 105(4):276-83.
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G Lefèvre,
H Zéphir,
F Warembourg,
E Michelin,
J-P Pruvo,
E Hachulla,
F Semah,
S Dubucquoi,
P Lenfant, P Vermersch,
P-Y Hatron,
L Prin,
D Launay
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ABSTRACT: Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with "neuropsychiatric systemic lupus erythematosus" (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation.
La Revue de Médecine Interne 05/2012; · 0.61 Impact Factor
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G Lefèvre,
H Zéphir,
E Michelin,
F Semah,
F Warembourg,
J-P Pruvo,
E Hachulla,
P Lenfant,
S Dubucquoi, P Vermersch,
P-Y Hatron,
L Prin,
D Launay
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ABSTRACT: Neurological and psychiatric manifestations of systemic lupus erythematosus are a heterogenous set of clinical manifestations grouped under the term of "neuropsychiatric systemic lupus erythematosus". The classification of these manifestations published in 1999 has harmonized the definitions cases used in the studies but did not help the clinician to positively identify a specific manifestation of lupus or a neurological or psychiatric event occurred independently of the disease. Published cases series help us to identify neurological or psychiatric manifestations of lupus but modern diagnosis tools contribution have to be evaluated in order to optimize diagnosis management of such manifestations and to distinguish specific events related to lupus and independent manifestations. In this second part of our literature review about neuropsychiatric lupus, we propose to identify arguments, which could be in favor of lupus responsibility in front of a neurological or psychiatric event, and immunosuppressive treatments which are recommended.
La Revue de Médecine Interne 05/2012; · 0.61 Impact Factor
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B Bourre,
R Marignier,
H Zéphir,
C Papeix,
D Brassat,
G Castelnovo,
N Collongues,
S Vukusic,
P Labauge,
O Outteryck,
B Fontaine, P Vermersch,
C Confavreux,
J de Seze
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ABSTRACT: The purpose of our study was to assess the influence of pregnancy on the course of neuromyelitis optica (NMO) and the impact of epidural analgesia and breastfeeding on its activity in the postpartum period.
We performed a retrospective study of patients with NMO diagnosed according to Wingerchuk criteria. We noted the number of relapses during the year before pregnancy (BP), during pregnancy (first trimester, second trimester, third trimester), and the year after (Y + 1: first trimester, second trimester [PP2], and third and fourth trimesters postpartum). Epidural analgesia and breastfeeding were recorded. Disability was evaluated with the Kurtzke Expanded Disability Status Scale (EDSS). The annualized relapse rate (ARR) was calculated.
We identified 124 patients (85 female) in the French NOMADMUS cohort on November 1, 2010. A total of 20 women (including 25 pregnancies) were informative with complete files. Comparisons between the ARR of each period and BP (1.0 ± 0.09) only showed an increased tendency for PP2 (0.8 ± 0.06, p = 0.07). Epidural analgesia and breastfeeding had no influence on the course of NMO. The EDSS score increased from 1.5 ± 1.7 BP to 2.6 ± 1.9 Y + 1 (p = 0.027).
This study shows that pregnancy influences the activity of NMO, a finding that justifies close medical monitoring. We found no evidence to suggest that either epidural analgesia or breastfeeding has an aggravating effect on NMO.
Neurology 03/2012; 78(12):875-9. · 8.31 Impact Factor
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A Melin,
O Outteryck,
N Collongues,
H Zéphir,
M C Fleury,
F Blanc,
A Lacour,
J C Ongagna,
A S Berteloot, P Vermersch,
J de Sèze
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ABSTRACT: "Disease activity free" in relapsing-remitting multiple sclerosis (RRMS) is a new concept introduced by the results of the AFFIRM study. Our objective was to analyze the clinical and radiological efficacy of natalizumab treatment in actual clinical practice and compare it with the post hoc analysis of the AFFIRM study. All patients with RRMS who began treatment with natalizumab at our two French MS centres between April 2007 and May 2008 were included and followed-up for at least 2 years. No measurable disease activity ("disease activity free") was defined as no activity on clinical measures (no relapses and no sustained disability progression) and radiological measures (no gadolinium-enhancing lesions and no new T2-hyperintense lesions on cerebral MRI). A total of 193 patients were included. Natalizumab was discontinued in 25.9% of cases before the completion of 2 years of treatment. In our cohort, we observed patients with more severe disease than in the AFFIRM study. The proportion of patients remaining free of clinical activity during 2 years of treatment was lower than in the AFFIRM study (37.8% vs. 64.3%). The proportion of patients remaining free of radiological activity during 2 years of treatment was higher than in the AFFIRM study (68.9% vs. 57.7%), while the proportion of patients remaining free of disease activity during 2 years of treatment was comparable to the AFFIRM study (33.3% vs. 36.7%). Natalizumab seems to be as effective in a real-life setting as in pivotal and post hoc studies. The confirmation of such benefits is important because of the progressive multifocal leukoencephalopathy risk.
Journal of Neurology 12/2011; 259(6):1215-21. · 3.47 Impact Factor
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ABSTRACT: Cyclophosphamide is still used in progressive forms of multiple sclerosis (MS) in view of its suggested efficacy and safety in the short term. No data exist on its long-term safety in MS, particularly on the risk of malignancy.
The objective of this study was to evaluate cancer incidence in MS after cyclophosphamide treatment.
We performed a historical prospective study in a cohort of MS patients treated with cyclophosphamide. We collected demographic data and medical history from medical databases and patient interviews. Reported cancers were histologically confirmed. Cancer incidence was compared with the incidence in the general population by estimating standardized incidence ratios (SIRs).
We included 354 patients, with a median follow-up of 5 years (range 2-15) after cyclophosphamide treatment. Fifteen patients developed a solid cancer, which occurred at a median of 3 years (range 0.5-14) after cyclophosphamide introduction. The cumulative incidence of cancer after cyclophosphamide was 3.1% at 5 years and 5.9% at 8 years. We found no increase in cancer incidence after cyclophosphamide treatment in men (SIR = 0.83, 95% confidence interval [CI] 0.30-1.82), women (SIR = 0.99, 95% CI 0.43-1.95), or men and women combined (SIR = 0.92, 95% CI 0.50-1.54).
We found no evidence of an increased risk of cancer associated with cyclophosphamide treatment in MS patients.
Multiple Sclerosis 08/2011; 18(1):55-63. · 4.26 Impact Factor
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I De Vidi,
G Boursier,
N Delouche,
P Portalès,
E Cadars,
M Bouthier,
C Mettling,
Y L Lin,
E Thouvenot,
B Carlander,
W Camu,
J P Antel,
A Bar-Or,
H Zephir, P Vermersch,
J De Seze,
P Corbeau,
J F Eliaou,
T Vincent
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ABSTRACT: NMO-IgG is a specific biomarker of neuromyelitis optica (NMO) that targets the aquaporin-4 (AQP4) water channel protein. The current gold standard for NMO-IgG identification is indirect immunofluorescence (IIF). Our aim in this study was to develop a new quantitative cell-based assay (CBA) and to propose a rational strategy for anti-AQP4 Ab identification and quantification. We observed an excellent correlation between the CBA and IIF for NMO-IgG/anti-AQP4 detection. The CBA appeared more sensitive than IIF but on the other hand, IIF allows the simultaneous detection of various auto-Abs, underlining the complementarity between both methods. In conclusion, we propose to use IIF for the screening of patients at diagnosis in order to identify auto-Abs targeting the central nervous system. A highly sensitive, AQP4 specific and quantitative assay such as our CBA could be used thereafter to specifically identify the target of the Ab and to monitor its serum concentration under treatment.
Clinical Immunology 03/2011; 138(3):239-46. · 4.05 Impact Factor
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N Collongues,
R Marignier,
H Zéphir,
F Blanc,
S Vukusic,
O Outteryck,
M Fleury,
A Ruet,
F Borgel,
E Thouvenot, [......],
B Audoin,
M Debouverie,
P Labauge,
O Gout,
W Camu,
D Brassat,
B Brochet, P Vermersch,
C Confavreux,
J de Seze
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ABSTRACT: Neuromyelitis optica (NMO) frequently begins with a monofocal episode of optic neuritis or myelitis. A concept named high-risk syndrome (HRS) for NMO has been proposed for patients with monofocal episodes and NMO-IgG antibodies.
To describe HRS patients and compare them with NMO patients.
We identified 30 patients with HRS: 18 with extensive myelitis (HRM) and 12 with optic neuritis (HRON), in a database pooling patients from 25 centres in France. Clinical, laboratory/magnetic resonance imaging (MRI) data and outcome were analysed and compared with a national cohort of 125 NMO patients extracted from the same database.
Mean follow-up was 4.8 years. Mean age at onset was 42.8 years (range: 12.4-70) with a female:male ratio of 0.9. Asymptomatic lesions were report on visual evoked potentials in 4/8 tested HRM patients and on spinal cord MRI in 2/7 HRON patients. Three patients died, two owing to a cervical lesion. HRS and NMO patients had similar clinical/paraclinical data, except for a predominance of men in the HRS group and a later mean age at onset in the HRM subgroup.
The description of HRS patients is compatible with a monofocal form of NMO. Asymptomatic lesions could be included in a new set of NMO diagnostic criteria.
Multiple Sclerosis 01/2011; 17(6):720-4. · 4.26 Impact Factor
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ABSTRACT: Clinical reports of cerebrovascular system involvement in sarcoidosis are extremely rare though pathological studies frequently describe granulomatous cerebral arterial and venous lesions. We report the case of a 47-year-old man with a history of pulmonary sarcoidosis at age 32 and abducens palsy at age 40, who presented cerebral pseudotumoral histologically proven sarcoidosis. He was admitted for acute left hemiplegia. Brain CT scan and MRI demonstrated a right posterior parietal haematoma associated with a superior sagittal sinus occlusion. He received intravenous corticosteroids and anticoagulant therapy. Six months later, he presented a right motor status epilepticus. MRI revealed new parenchymal haematomas. Cerebral angiography demonstrated cerebral vasculitis.
Acta neurologica Belgica 12/2010; 110(4):349-52. · 0.54 Impact Factor
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ABSTRACT: The burden of multiple sclerosis (MS) includes fatigue, depression and worsening of health-related quality of life (HRQOL). These changes have not been yet measured in neuromyelitis optica (NMO). Our aim was to assess the HRQOL, fatigue and depression in NMO.
We administered French validated self-questionnaires on HRQOL (SEP-59), fatigue (EMIF-SEP) and depression (EHD) to 40 patients followed up in two centres. We assessed the relationship of these parameters with gender, age, disability, disease duration, visual acuity and NMO-antibody status and also compared our results with equivalent data in MS and normal subjects derived from previous studies.
Health-related quality of life scores were lower (P < 0.01) in patients with NMO when compared to normal subjects. No significant difference was noted between patients with NMO and MS for most scores, the exceptions being HRQOL related to cognitive function (better in NMO than in MS), HRQOL related to sphincter dysfunction (worse in NMO than in MS) and the psychological dimension of fatigue (milder in NMO than in MS). Disability was the main predictive factor of an unfavourable evolution.
This study reveals the strong impact of NMO on HRQOL, fatigue and depression and the importance of screening patients, especially the more disabled, so as to initiate suitable treatment.
European Journal of Neurology 11/2010; 18(6):836-41. · 3.69 Impact Factor
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N Collongues,
R Marignier,
H Zéphir,
C Papeix,
B Fontaine,
F Blanc,
D Rodriguez,
M Fleury,
S Vukusic,
J Pelletier,
B Audoin,
E Thouvenot,
W Camu,
B Barroso,
A Ruet,
B Brochet, P Vermersch,
C Confavreux,
J de Seze
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ABSTRACT: Neuromyelitis optica (NMO) is a rare inflammatory disease. Average age at onset is 35 years. Few data exist on patients with pediatric-onset NMO (p-NMO), with disease onset before age 18 years. We report the clinical and paraclinical features and long-term outcome of patients with p-NMO and compare them with a large adult-onset NMO (a-NMO) cohort.
We performed a retrospective, multicenter study of patients with p-NMO in pediatric and adult medical centers. We identified 125 patients with NMO (12 p-NMO; 113 a-NMO) fulfilling the 2006 criteria. Data were collected using hospital files and standardized assessment forms for NMO.
Patients with p-NMO were followed up during a mean 19.3 years. Median age at onset was 14.5 years (4.1-17.9) with a female:male ratio of 3:1. Three patients (25%) fulfilled Paty criteria for multiple sclerosis on first brain MRI, including one patient with acute disseminated encephalomyelitis. Median interval between onset and residual Expanded Disability Status Scale (EDSS) score 4 was 20.7 years, score 6 was 26 years, and score 7 was 28.7 years. Median interval between onset and residual visual loss ≤1/10 was 1.3 years. Compared with a-NMO, p-NMO showed a longer time to EDSS scores 4 and 6, largely explained by the severity of the first myelitis in the a-NMO group. Time to first treatment was longer in the p-NMO group (13.1 vs 3.4 years).
Patients with p-NMO can present a diffuse inflammatory process on first brain MRI and have a longer time to disability than patients with a-NMO.
Neurology 09/2010; 75(12):1084-8. · 8.31 Impact Factor
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La Revue de Médecine Interne 06/2010; 31 Suppl 1:S8-15. · 0.61 Impact Factor
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N Collongues,
R Marignier,
H Zéphir,
C Papeix,
F Blanc,
C Ritleng,
M Tchikviladzé,
O Outteryck,
S Vukusic,
M Fleury, [......],
D Rodriguez,
S Wiertlewski,
D Laplaud,
F Borgel,
P Tourniaire,
J Grimaud,
B Brochet, P Vermersch,
C Confavreux,
J de Seze
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ABSTRACT: There have been few epidemiologic studies on neuromyelitis optica (NMO) and none used the recent 2006 diagnostic criteria. Here we describe the clinical, laboratory, MRI, and disability course of NMO in a French cohort of 125 patients.
We performed an observational, retrospective, multicenter study. Data were collected from September 2007 through August 2008, corresponding to the endpoint of the study. We identified 125 patients fulfilling the 2006 NMO criteria. Selection was made using hospital files and a specific clinical questionnaire for NMO.
Mean age at onset was 34.5 years (range 4-66) with a mean disease duration of 10 +/- 7.8 years at the endpoint. The patients were mainly (87%) Caucasian, with a female:male ratio of 3:1. In 90% of cases, the association of optic neuritis, longitudinal extensive myelitis, and a Paty-negative initial brain MRI was sufficient to fulfill the supportive criteria. Eighty-eight percent of patients were treated with immunosuppressive therapies. Median delay from onset to Expanded Disability Status Scale (EDSS) score 4 was 7 years; score 6, 10 years; and score 7, 21 years. The first episode of myelitis was immediately followed by an EDSS score > or = 4 in 37.3% of cases, and a severe residual visual loss was observed in 22% of patients after the first episode of optic neuritis. Multivariate analysis did not reveal any predictors of a poor evolution other than a high number of MRI brain lesions at diagnosis, which were predictive of a residual visual acuity < or = 1/10.
Our demographic data provide new data on disability in patients with neuromyelitis optica, most of whom were receiving treatment.
Neurology 03/2010; 74(9):736-42. · 8.31 Impact Factor
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ABSTRACT: Limb-girdle muscular dystrophy 2I (LGMD2I) is caused by mutations in the fukutin-related protein gene FKRP, which is also involved in congenital muscular dystrophy (MDC1C).
To evaluate the clinical, biological, radiological and mutational characteristics of LGMD2I patients with FKRP mutation.
Eleven patients from nine families from the north of France were studied. Demographical data, muscular testing results, cardiac and respiratory examinations, muscle histological features and a genetic analysis of the FKRP gene for each patient are reported. Eight patients underwent brain MRI and seven neuropsychological tests.
The patients included six women and five men. The mean age at onset was 9 years (range 1.5 to 23 years). Five patients remained self-ambulatory, whereas the other six were confined to a wheelchair by a mean age of 19 years, after a mean disease duration of 10 years. Nine patients suffered from restrictive respiratory insufficiency, and two male patients had severe dilated cardiomyopathy. Neuropsychological tests revealed memory impairment in four cases. Brain MRI revealed cerebral abnormalities in four patients (4/8). Ten patients were carriers of the common L276I mutation, which was either homozygous (four patients) or heteroallelic with another mutation (six patients). Among the mutations found, three were novel: L322V, L489R and R275G.
This study reveals inter- and intrafamilial phenotypic variability in LGMD2I, with a preponderance of myocardiopathy and restrictive respiratory insufficiency. It also demonstrates central nervous involvement, probably associated with changes in alpha-dystroglycan expression in the brain.
Journal of neurology, neurosurgery, and psychiatry 12/2009; 80(12):1405-8. · 4.87 Impact Factor
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ABSTRACT: Psychotic symptoms are not readily recognized in multiple sclerosis, especially at the beginning of the disease.
We report the cases of four patients who developed psychotic symptoms that led to the diagnosis of multiple sclerosis. We describe the psychiatric and neurological features, MRI findings, clinical outcome and treatment.
Two patients developed persecutory delusions, one presented a manic episode and the fourth melancholia with catatonia. Mean age was 39 years (range 20-49 years). Two patients had a personal history, but none a familial history of psychiatric disease. Examination of the cerebrospinal fluid revealed an oligoclonal pattern in all patients. All patients fulfilled Barkhof's MRI criteria. Three have had brain MRI with injection during psychotic symptoms. In these three cases, a frontal lesion appeared. The patient with catatonia also had a new lesion in the cerebellum and in the brainstem. All patients needed a "psychiatric" treatment, including antipsychotics. The psychiatric event lasted three months for two patients and the two others experienced relapse.
Acute psychiatric symptom may reveal multiple sclerosis at the beginning of the disease. Frontal lobe localization is suggested. We propose that a psychotic event may correspond to a multiple sclerosis event.
Revue Neurologique 10/2009; 166(1):39-48. · 0.49 Impact Factor
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ABSTRACT: Natalizumab is the first selective adhesion molecule inhibitor indicated for treatment of active relapsing-remitting multiple sclerosis (RRMS). Natalizumab has been available in France since April 2007. The aims of this study are to analyze demographic, clinical, and tolerance data from French patients with RRMS treated with natalizumab in actual clinical practice and to draw comparisons with patients in the pivotal AFFIRM study. All patients with RRMS in the Nord-Pas de Calais and Alsace regions of France treated with natalizumab at any time since April 2007 were included. Variables analyzed included previous treatments; disability status [Expanded Disability Status Scale (EDSS) score]; annualized relapse rate (ARR) at baseline and after 12 months of treatment; and adverse events. Data from 384 patients (72% female) were evaluated. Mean baseline EDSS score was 3.53 and mean baseline ARR was 2.19, both significantly greater than in AFFIRM. One hundred twenty-seven patients completed 12 months of treatment; mean EDSS score in this group was 3.02 (14% reduction) and mean ARR was 0.59 (73% reduction). Although these patients had significantly different baseline characteristics and greater disability compared with patients receiving natalizumab in AFFIRM, average disability remained stable and ARR declined by 73%. Tolerability was similar to that observed in AFFIRM.
Journal of Neurology 09/2009; 257(2):207-11. · 3.47 Impact Factor
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ABSTRACT: Near 10 to 20% of patients with myositis have another systemic, sometimes inaugural, disease.
A 48-year-old woman was admitted with progressive hypoesthesia in V2 and V3 areas on both sides, difficulties to chew and swallow and then, proximal and axial muscular deficiency, with weight loss. Brain MRI showed gadolinium-enhanced trigeminal nerves and biological tests revealed anti-SSA and anti-Pm/Scl antibodies and a grade IV in Chisholm scoring system on the labial salivary gland biopsy. Neurophysiological studies revealed a myogenic pattern on tibialis anterior muscles and a muscle biopsy confirmed the diagnosis of polymyositis.
The diagnosis of primitive Sjogren's syndrome was suspected because of the association of bilateral trigeminal neuropathy and anti-SSA and anti-Pm/Scl antibodies.
Revue Neurologique 05/2009; 166(1):96-9. · 0.49 Impact Factor
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Journal of neurology, neurosurgery, and psychiatry 05/2009; 80(4):450-1. · 4.87 Impact Factor
