Glial Fibrillary Acidic Protein Autoimmunity: A French Cohort Study

Background and ObjectivesTo report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies.MethodsWe retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAPα since 2017 from 2 French referral centers.ResultsWe identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score ≤2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies.DiscussionGFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome

“Status trigeminal neuralgia”: Analysis of 39 cases and proposal for diagnostic criteria

International audienceObjective: The aim of this descriptive study was to propose diagnostic criteria for acute exacerbation of trigeminal neuralgia (TN) based on the analysis of retrospective cases.Background: TN is a rare and extremely painful condition whose evolution can be punctuated by major exacerbations, leading to significant functional impairment. Several denominations are used for these exacerbations: "acute exacerbation", "status of trigeminal neuralgia", and "status trigeminus". There is currently no clinical definition of this state. In this manuscript, we used the term "status trigeminal neuralgia" (STN).Methods: We conducted a retrospective study, in a tertiary care specialist headache center, in France. Patients were selected from January 2015 to October 2022, with the French translation of the keyword "STN", in the medical records (outpatients) or the codage for trigeminal neuralgia (inpatients). Additional cases of STN were prospectively recruited from October 2022 to February 2023. We analyzed the clinical and paraclinical data of these patients.Results: Thirty-nine patients presenting with STN were included. There was a preponderance of women (64%) with 24 cases of classic TN (62%) and 15 cases of secondary TN (38%). Concerning STN, 39 episodes were described. Pain was very severe in all patients. Cranial autonomic signs were present in 23% of cases. Pain extended beyond the usual territory in 44% of cases. A continuous pain background was present in 35% of cases. With regard to triggering factors, paroxysms of facial pain were triggered by eating (97% of patients), speaking (90%) or drinking (62% of patients). Repercussions on weight, hydration, or mood disorders were observed in 67%, 56% and 59% of the cases, respectively.Conclusion: STN is a rare clinical presentation of TN. We proposed criteria and a new denomination for this condition

Efficacy of dupilumab in real-life settings: a STROBE study

International audienceBackground: Dupilumab, a monoclonal antibody targeting IL-4 and IL-13, has demonstrated its efficacy in several clinical trials. However, to date, real-life data remains limited.Objective: The aim of our study was to assess the real-life impact of dupilumab on patients with severe and uncontrolled chronic rhinosinusitis with nasal polyps (CRSwNP) quality of life.Materials and methods: This was a retrospective, monocentric, observational, real-life study, conducted in accordance with the STROBE guidelines. The following parameters were collected before treatment and at 1, 4, and 12 months: Sino-Nasal Outcome Test-22 (SNOT-22), nasal polyp score (NPS), Sniffin' Sticks-16 (SST-16), visual analog scale (VAS) for loss of smell, nasal congestion score (NCS), gustatory VAS, asthma control, oral corticosteroid usage, surgery rates, and occurrence of side effects.Results: The study included 47 patients. SNOT-22 scores decreased from 52.4 ± 24.3 to 12.7 ± 10.5 at 12 months (p < 0.001). NPS decreased from 6.15 ± 1.71 to 1.57 ± 1.40 at 12 months (p < 0.001). SST-16 scores increased from 1.6 ± 2.83 to 9.1 ± 5.4 at 12 months (p < 0.001). NCS decreased from 2.45 ± 0.72 to 0.38 ± 0.63 at 12 months (p < 0.001). Prior to treatment, 72.3% were using oral corticosteroids, compared to 17.0% at 12 months (p < 0.01). Two patients required additional surgery, and 17% reported completely uncontrolled asthma, compared to 0% at 12 months (p < 0.01).Conclusion: Our real-life results confirm the efficacy of Dupilumab in the treatment of severe and uncontrolled CRSwNP

Effet des polluants sur la dermatite atopique : Revue de la littérature et méta analyse

International audienc

Effet des polluants sur la dermatite atopique : Revue de la littérature et méta analyse

International audienc