Les traitements de la sclérose en plaques (SEP), en particulier des biothérapies, sont complexes à organiser et nécessitent une rigueur dans la planification et le suivi.
Créer un outil informatique ...d’aide à l’organisation et la planification en vue d’améliorer la qualité et la sécurité des biothérapies de la sclérose en plaques.
Création de l’outil informatique sur la base des résumés des caractéristiques des produits et des recommandations des sociétés savantes pour le natalizumab, l’ocrelizumab et le rituximab : bilans préthérapeutiques, modalités du suivi intercure et précure, recommandations de suivi clinique, biologique, radiologique, formule de risque de LEMP. Développement d’une base de données : choix des tables, des champs, création de requêtes, de formulaires, d’états, de nomenclature. Choix de l’outil. Tests puis validation.
ClickSEP permet la création de fiche patients incluant choix du médicament, date de début, adaptation des dates de rendez-vous et de cures, dénomination des médecins, planification automatique ou personnalisée des délais entre examens/consultations/IRM ; création d’alertes et de seuils d’alertes ; calcul automatique des risques de LEMP et infectieux selon le taux d’immunoglobulines ; automatisme des rappels par emails, courriers types. Paramétrage complet générique et par patient. Adaptable à tout nouveau traitement.
Les rappels de ClickSEP au prescripteur et au patient des examens recommandés limitent les oublis ; les alertes spécifiques (LEMP) améliorent la sécurité de prescription. Ils permettent d’assurer la qualité et la sécurité des traitements avec une actualisation aisée par un référent pour le non-spécialiste. Outil de coordination, ClickSEP ne se substitue pas au dossier médical ni au planning hospitalier.
ClickSEP est le premier outil de coordination du parcours patient traité par biothérapie au cours de la sclérose en plaques visant la qualité et la sécurité du suivi.
Domains encompassing emotional disorders in relapsing-remitting MS (RRMS) patients are still unclear.
We performed a 24-month, multicenter, single-arm, prospective study. RRMS patients started IFN-β ...treatment at baseline. The primary endpoint was lack of emotional control, measured using the “Echelle d'HumeurDépressive” (EHD) scale three times at baseline and at 10 post-treatment visits. Secondary endpoints were emotional blunting, irritability, fatigue, depression and anxiety. A linear mixed covariance model assessed change from baseline on an intention-to-treat basis, under the assumption of no mood disorder effect (one-sided 97.5% level), in which autoregressive type of autocorrelation was tested.
Out of 79 recruited patients, 70 were analyzed: 80% female; mean (SD) age, 37.0 (11.5) years. Mean (SD) lack of emotional control score at baseline and Month 24 was 12.7 (4.4) and 12.6 (5.5), respectively, versus 10.1 (3.2) in a healthy control population matched for age and sex. Stepwise analysis identified younger age, male sex and antidepressant use as significant predictors of higher lack of emotional control values.
Based on 24 months of prospective follow-up, the results of this study highlights a broad spectrum of emotional disorders in the MS population at the time of disease modifying drugs initiation but no major IFN-β-related emotional disorders (mood dyscontrol, anxiety, depression) were observed. However, sporadic occurrences of severe mood disorders and suicidality cannot be excluded.
•70 MS patients including 16 evaluations•This 24-month prospective study identifies emotional disorders in MS patients.•On an intent-to-treat basis no significant change under interferon-β treatment.•Sporadic emotional disorders under interferon-β treatment cannot be excluded.
•Ulnar ratio definition: SNAP amplitude of palmar cutaneous over that of dorsal branch of ulnar nerve.•Ulnar ratio ≥ 0.78 can rule out diagnosis of Acute Inflammatory Demyelinating Polyneuropathy ...(AIDP), with high specificity (100%) and sensitivity (87%).•Incorporating specific sensory abnormalities in AIDP criteria may enhance their reliability.
To explore the value of a novel sensory criterion, the ulnar ratio – defined as the SNAP amplitude of the palmar cutaneous (pUN) over that of the dorsal branch (dUN) of the ulnar nerve – as a predictor of Acute Inflammatory Demyelinating Polyneuropathy (AIDP).
We prospectively included 22 patients with AIDP, 20 patients with diabetic peripheral neuropathy (DPN), and 18 controls. Eligible subjects underwent nerve conduction studies including, among others, the dUN, pUN, and sural nerve.
A sural sparing pattern was found in 72% of AIDP cases. The ulnar ratio was significantly lower in patients with AIDP compared to those with DPN or controls. The ROC curve area to discriminate AIDP (versus controls and diabetics together) was higher with the ulnar ratio and pUN compared to dUN. An ulnar ratio ≥ 0.78 seems to be the best threshold to rule out the diagnosis of AIDP, with a specificity of 100% and a sensitivity of 87%. The ulnar ratio was equally reliable in the subgroup of patients presenting within a week of symptoms onset.
The ulnar ratio is a highly sensitive and specific marker of AIDP and can help confirm the diagnosis when direct signs of demyelination are lacking.
Incorporating specific sensory abnormalities, such as the ulnar ratio, in the electrodiagnostic criteria of AIDP could enhance their reliability.
We conducted the current study to analyze the clinical, immunologic, and neurophysiologic features of primary Sjögren syndrome (pSS)-associated sensory small fiber neuropathies (SFNs). Forty ...consecutive pSS patients with SFN were included. SFN was defined by the presence of suggestive sensory painful symptoms with normal nerve conduction studies and abnormal neurophysiologic tests for small nerve fibers or a low intraepidermal nerve fiber density at skin biopsy. Included patients were compared to 100 pSS patients without peripheral neuropathy.SFN patients were mainly female (92.5%). Age at pSS diagnosis was 55.3 ± 13.1 years, and at SFN diagnosis, 58.9 ± 11.8 years, with a median time to SFN diagnosis after symptom onset of 3.4 years. Clinical symptoms included burning pains (90%), numbness (87.5%), tingling (82.5%), pins and needles (72.5%), electric discharges (70%), and allodynia (55%). Dysautonomia included vasomotor symptoms (66%) and hyperhidrosis (47%). Abnormal neurophysiologic tests included laser evoked potentials (97.5%), thermal quantitative sensory testing (67.5%), and sympathetic skin reflex (40%). A skin biopsy revealed low intraepidermal nerve fiber density in 76% of the 17 tested patients.Compared to the 100 pSS patients without peripheral neuropathy, the 40 pSS-SFN patients were older at pSS diagnosis (55.3 ± 13.1 vs. 49.5 ± 14.9 yr; p = 0.03), and more often had xerostomia (97.5% vs. 81%; p = 0.01) and arthralgia (82.5% vs. 65.0%; p = 0.04). Immunologically, they were characterized by a lower prevalence of serum B-cell activation markers, that is, antinuclear antibodies (65% vs. 85%; p = 0.01), anti-SSA (42.5% vs. 71%; p = 0.002), and anti-SSB (17.5% vs. 39%; p = 0.017); rheumatoid factor (32.5% vs. 66%; p = 0.0005); and hypergammaglobulinemia (35% vs. 62%; p = 0.005).In conclusion, we report the main features of SFN in patients with pSS, the first such study to our knowledge. Our results show that patients with pSS-associated SFN are characterized by an older age at pSS diagnosis and a distinctive immunologic profile hallmarked by a lower frequency of serum B-cell activation markers.
The specialized nurse in MS is a new profession practiced in MS clinics and outpatients specialized coordination structures. She is part of a specialized multi-professional team. The nurse must ...master a broad knowledge of the disease and the treatments. Have skills in therapeutic education, communication and coordination. She has a detailed knowledge of the resources of the care offer. The MS nurse thus participates in the assessment of the patient's needs for the implementation of a personalized care plan combining response to medical, paramedical, psychological and social needs.
Background and purpose
Disease‐modifying therapies (DMTs) have an impact on relapses and disease progression. Nonetheless, many patients with multiple sclerosis (MS) remain untreated. The objectives ...of the present study were to determine the proportion of untreated patients with MS followed in expert centers in France and to determine the predictive factors of nontreatment.
Methods
We conducted a retrospective cohort study. Data were extracted from the 38 centers participating in the European Database for Multiple Sclerosis (EDMUS) on December 15, 2018, and patients with MS seen at least once during the study period (from June 15, 2016 to June 14, 2017) were included.
Results
Of the 21,189 patients with MS (age 47.1 ± 13.1 years; Expanded Disability Status Scale (EDSS) score 3.4 ± 2.4), 6,631 (31.3%; 95% confidence interval CI 30.7–31.9) were not receiving any DMT. Although patients with a relapsing‐remitting course (n = 11,693) were the most likely to receive DMT, 14.8% (95% CI 14.2–15.4) were still untreated (6.8% never treated). After multivariate analysis among patients with relapsing‐remitting MS, the main factors explaining never having been treated were: not having ≥9 lesions on brain magnetic resonance imaging (odds ratio OR 0.52 95% CI 0.44–0.61) and lower EDSS score (OR 0.78 95% CI 0.74–0.82). Most patients with progressive MS (50.4% for secondary and 64.2% for primary progressive MS) did not receive any DMT during the study period, while 11.6% of patients with secondary and 34.0% of patients with primary progressive MS had never received any DMT.
Conclusion
A significant proportion of patients with MS did not receive any DMT, even though such treatments are reimbursed by the healthcare system for French patients. This result highlights the unmet need for current DMTs for a large subgroup of patients with MS.
The objectives were to determine the proportion of untreated patients with multiple sclerosis (MS) followed in expert centers in France and to determine the predictive factors of non‐treatment. Among the 21,189 patients with MS, 31.3% were not receiving any disease‐modifying treatment and the most important predictive factor for not being treated was the disease course: 14.8% of patients with a relapsing‐remitting course were untreated, while 50.4% of those with secondary and 64.2% of those with primary progressive MS were untreated. After multivariate analysis among patients with relapsing‐remitting MS, the main factors explaining never having been treated were not having ≥9 lesions on brain magnetic resonance imaging and lower Expanded Disability Status Scale score.
Phosphorus magnetic resonance spectroscopy (31P-MRS) has previously shown abnormal changes in energy metabolites in the brain of multiple sclerosis (MS) patients. However, the relationship between ...these energy metabolites - particularly adenosine triphosphate (ATP) - and the disease severity remains unclear. The objective of this study was to determine whether measuring ATP metabolites can help to predict disease severity in MS patients.
31P-MRS at 3 tesla was performed in 9 relapsing remitting (RRMS), 9 secondary progressive MS patients (SPMS), and 10 age-matched healthy controls. ATP metabolites (expressed as %) in normally appearing white matter of the centrum semiovale were compared between patients and healthy controls. The relationship between Expanded Disability Status Scale (EDSS) and ATP metabolites was evaluated.
RRMS and SPMS patients had higher phosphocreatine (PCr) and lower phosphodiesters than healthy controls. In addition, RRMS patients had higher β-ATP% than SPMS patients. β-ATP% was negatively correlated with EDSS in all patients.
Our findings suggest a defective PCr metabolism in both patient groups, and a higher state of energy production in RRMS that might reflect a compensatory mechanism in face of the increased needs. The correlation of β-ATP with EDSS makes it a candidate biomarker for assessing MS disease severity.
Abstract Background Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of ...neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration. Methods Eighty-six patients with acute/subacute inflammatory SNN (paraneoplastic, associated with dysimmune diseases, or idiopathic) were retrospectively studied. The monthly SNAP reduction was determined and normalized with the lower limit of normal. Disability progression was expressed by the modified Rankin score and correlated with SNAP reduction. Results The monthly SNAP reduction was similar in the four limbs although the median nerve was less severely affected. The monthly SNAP reduction was very severe within the first two months of evolution, began to slow down after seven months, and stabilized after ten months. It was tightly correlated with disability progression. Kaplan–Meier analysis showed that the median time until matching the diagnostic criteria of SNN was 8.5 months. Within this period, 42% of nerves remained excitable. Conclusions Developing treatment aiming at the stabilization of SNN is possible within the first 8 months of evolution. An improvement of the disease is possible if patients are treated within two months, which needs an early referral to an expert center and ENMG testing of the radial and ulnar nerves, which are most sensitive to changes.
Highlights • In 74 patients, quantitative sensory testing was performed to characterize the changes induced by peripheral neuropathy, especially associated with pain. • The presence or absence of ...pain was not associated with differences in sensory thresholds, regarding either detection or pain thresholds for mechanical and thermal sensations. • However, the intensity of pain, when present, increased with thermal and pain threshold alteration, showing complex relationship between pain and small fiber dysfunction.
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