Publications by authors named "Jean-Philippe Camdessanche"
Objectives: To capture patient perceptions about living with myasthenia gravis (MG) with respect to aspirations and ways to improve treatment.
Design: Online patient survey.
Setting: Patients recruited by MG patient associations or at MG reference treatment centres.
Background: We hypothesized that differences in access to disease-modifying treatments (DMTs) could explain the association between socioeconomic status and disability progression in multiple sclerosis (MS).
Objective: This study aimed to analyze the association between education level and DMT use in France.
Methods: All patients from OFSEP network with MS onset over 1996-2014 and aged ⩾ 25 years at onset were included.
Background: Studies have reported an association between socioeconomic status and disability progression in multiple sclerosis (MS), but findings using the pre-MS individual socioeconomic status are missing.
Objective: The objective was to investigate the association between education level and disability progression.
Methods: All Observatoire Français de la Sclérose en Plaques (OFSEP) patients with MS clinical onset over 1960-2014, and aged ⩾25 years at MS onset were included.
Article Synopsis
- * Researchers analyzed data from 275 CMTX1 patients across 13 centers in France, finding that those with mutations in transmembrane domains had more severe symptoms and earlier onset than those with mutations in intracellular or extracellular domains.
- * The findings suggest that the type of genetic mutation not only helps diagnose CMTX1 but also predicts disease severity, emphasizing the need to consider these correlations in upcoming clinical research.
Background And Purpose: Few data are available on the course of myasthenia gravis (MG) regarding disease severity and stability over time in real-world settings. This study used the French National Health Insurance Database (SNDS) to assess markers of disease severity in patients with MG longitudinally.
Methods: All patients with MG-related claims in the SNDS between 2013 and 2020 were identified.
Article Synopsis
- The study aimed to compare disability progression between primary progressive multiple sclerosis (PPMS) patients treated with anti-CD20 therapies (rituximab and ocrelizumab) and a control group that was untreated.
- Data was gathered retrospectively from the French MS registry, including factors like time to confirmed disability progression (CDP), relapse rates, and MRI activity in patients from 2016 to 2021.
- Results showed no significant difference in CDP or MRI activity between treated and untreated groups, although a trend suggested treated patients might experience fewer relapses, warranting further investigation.
Article Synopsis
- - The diagnosis of amyotrophic lateral sclerosis (ALS) involves identifying degeneration of upper and lower motor neurons across various regions, accompanied by progressive motor weakness, but can be complicated due to diverse clinical presentations.
- - Symptoms of ALS can vary significantly, affecting different areas like limbs, bulbar region, or respiratory muscles, and the disease duration can range from months to decades.
- - Diagnosing ALS relies on a combination of clinical assessments and tests, as there's no single specific test, necessitating careful evaluation to avoid delaying treatment while ruling out similar conditions.
Objective: There is currently scarce data on the electroclinical characteristics of epilepsy associated with synapsin 1 (SYN1) pathogenic variations. We examined clinical and electro-encephalographic (EEG) features in patients with epilepsy and SYN1 variants, with the aim of identifying a distinctive electroclinical pattern.
Methods: In this retrospective multicenter study, we collected and reviewed demographic, genetic, and epilepsy data of 19 male patients with SYN1 variants.
Article Synopsis
- * Conducted over eight years (2015-2023) using data from a French MS registry, researchers categorized relapses based on MRI results to better understand their impact.
- * Findings indicate that certain factors, like treatment type and fatigue, increase the likelihood of clinically defined relapses without MRI evidence, suggesting a need for revised monitoring and treatment strategies for MS patients.
Benefits of Individualized Training in Fatigued Patients with Multiple Sclerosis.
Med Sci Sports Exerc
September 2024
Introduction: Chronic fatigue is the most common and debilitating symptom in people with multiple sclerosis (PwMS). Recently, exercise has been proven to alleviate chronic fatigue and improve physical functions. Tailoring the training intervention to the potential fatigue causes could optimize the beneficial effects of training on fatigue.
View Article and Find Full Text PDFArticle Synopsis
- The study analyzed 166 patients with acute neurological symptoms linked to anti-GQ1b antibodies, revealing frequent symptoms like areflexia, sensory issues, and muscle weakness.
- The majority of patients were treated with intravenous immunoglobulins, leading to complete neurological recovery for 69% at the one-year mark, although 15% experienced relapses.
- Key predictors for incomplete recovery included age over 70, initial ICU admission, and absence of anti-GQ1b antibodies; no predictors for relapse were identified.
Article Synopsis
- Congenital myasthenic syndromes (CMS) are genetic disorders that impact neuromuscular transmission, primarily identified in childhood but often diagnosed in adulthood, leading to challenges in management.
- A study of 235 adult CMS patients in France revealed diverse genetic mutations and highlighted the need for ongoing care, as the prognosis and long-term outcomes remain unclear.
- The research categorized patients based on the initial symptoms and found varied disease progression patterns, with certain genotypes showing higher rates of ICU admission and the stability of phenotypical features across a patient's life.
Article Synopsis
- A randomized clinical trial suggests that stopping medium-efficacy therapy for older patients with nonactive multiple sclerosis (MS) may be safe, but data is lacking for high-efficacy therapy (HET).
- This observational cohort study from the French MS registry examined 1857 older patients with relapsing-remitting MS on HET and aimed to find out if stopping HET increased relapse risks.
- The study included 1620 matched patients, with results indicating that both groups (continuing vs. discontinuing HET) were closely monitored over an average of 5.1 years to determine the time to first relapse.
Recent studies in adults suggested that extended-interval dosing of rituximab/ocrelizumab (RTX/OCR) larger than 12 months was safe and could improve safety. This was an observational cohort study of very active pediatric-onset multiple sclerosis (PoMS) (median (range) age, 16 (12-17) years) treated with RTX/OCR with 6 month standard-interval dosing ( = 9) or early extended-interval dosing ( = 12, median (range) interval 18 months (12-25)). Within a median (range) follow-up of 31 (12-63) months after RTX/OCR onset, one patient (standard-interval) experienced relapse and no patient showed disability worsening or new T2-weighted lesions.
View Article and Find Full Text PDFArticle Synopsis
- Hereditary transthyretin amyloidosis (ATTRv) is a genetic disease that typically starts in adults and leads to progressive nervous system issues, but there are effective treatments available.
- A study conducted in France over five years analyzed 553 patients over 50 with progressive nerve problems, finding that 2.7% had pathogenic gene variants linked to ATTRv, primarily the Val30Met variant.
- Patients with ATTRv were more likely to experience symptoms like severe weight loss and orthostatic hypotension, and the diagnosis allowed for specific treatments and identified additional cases in family members, emphasizing the importance of early detection.
Article Synopsis
- This study compares two methods for estimating death probabilities in multiple sclerosis (MS) patients: the Cause-Specific Framework (CSF) which requires known causes of death, and the Excess Mortality Framework (EMF) which does not.
- Using data from a large MS registry and a subset with detailed death records, the researchers found that EMF generally estimated higher death probabilities than CSF across different age groups.
- Overall, the analysis revealed varying mortality probabilities for MS patients based on the initial disease type, sex, and age; with significant differences particularly noted between relapsing-onset MS and primary progressive patients over a 30-year follow-up.
Background: X-Linked Charcot-Marie-Tooth disease type 1 (CMTX1) is characterized by gender differences in clinical severity. Women are usually clinically affected later and less severely than men. However, their clinical presentation appears to be heterogenous.
View Article and Find Full Text PDFIntroduction: Fatigue is the most common and disabling symptom in multiple sclerosis (MS), being reported by 55% to 78% of patients with MS (PwMS). Etiology of MS-related fatigue remains poorly understood, but an increased neuromuscular fatigability (i.e.
View Article and Find Full Text PDFAnti-AGO1 Antibodies Identify a Subset of Autoimmune Sensory Neuronopathy.
Neurol Neuroimmunol Neuroinflamm
May 2023
Background And Objectives: Autoantibodies (Abs) improve diagnosis and treatment decisions of idiopathic neurologic disorders. Recently, we identified Abs against Argonaute (AGO) proteins as potential autoimmunity biomarkers in neurologic disorders. In this study, we aim to reveal (1) the frequency of AGO1 Abs in sensory neuronopathy (SNN), (2) titers and IgG subclasses, and (3) their clinical pattern including response to treatment.
View Article and Find Full Text PDFArticle Synopsis
- * A 16-year-old girl with a history of severe bilateral frontotemporal headaches was found to have right hippocampal sclerosis through MRI, and her headache was confirmed to be IEH using video-electroencephalographic monitoring.
- * After initially managing her condition with medication, she underwent surgery (anterior temporal lobectomy) due to worsening seizures, resulting in a 10-year period without seizures or headaches.
Background And Objectives: The question of the long-term safety of pregnancy is a major concern in patients with multiple sclerosis (MS), but its study is biased by reverse causation (women with higher disability are less likely to experience pregnancy). Using a causal inference approach, we aimed to estimate the unbiased long-term effects of pregnancy on disability and relapse risk in patients with MS and secondarily the short-term effects (during the perpartum and postpartum years) and delayed effects (occurring beyond 1 year after delivery).
Methods: We conducted an observational cohort study with data from patients with MS followed in the Observatoire Français de la Sclérose en Plaques registry between 1990 and 2020.
Background: In relapsing-remitting multiple sclerosis (RRMS), early identification of suboptimal responders can prevent disability progression.
Objective: We aimed to develop and validate a dynamic score to guide the early decision to switch from first- to second-line therapy.
Methods: Using time-dependent propensity scores (PS) from a French cohort of 12,823 patients with RRMS, we constructed one training and two validation PS-matched cohorts to compare the switched patients to second-line treatment and the maintained patients.
Background: The effects of socio-economic status on mortality in patients with multiple sclerosis is not well known. The objective was to examine mortality due to multiple sclerosis according to socio-economic status.
Methods: A retrospective observational cohort design was used with recruitment from 18 French multiple sclerosis expert centers participating in the All patients lived in metropolitan France and had a definite or probable diagnosis of multiple sclerosis according to either Poser or McDonald criteria with an onset of disease between 1960 and 2015.