Publications by authors named "J-M Mignot"
Background And Objectives: While patients with paraneoplastic autoimmune encephalitis (AE) with gamma-aminobutyric-acid B receptor antibodies (GABAR-AE) have poor functional outcomes and high mortality, the prognosis of nonparaneoplastic cases has not been well studied.
Methods: Patients with GABAR-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples.
Background And Objectives: REM sleep behavior disorder (RBD) is a parasomnia characterized by dream enactment. The International RBD Study Group developed the RBD Symptom Severity Scale (RBDSSS) to assess symptom severity for clinical or research use. We assessed the psychometric and clinimetric properties of the RBDSSS in participants enrolled in the North American Prodromal Synucleinopathy (NAPS) Consortium for RBD.
View Article and Find Full Text PDFCharacterization of T cell receptors reactive to HCRT, pHA, and NP in control and narcolepsy patients.
Proc Natl Acad Sci U S A
August 2022
Narcolepsy type 1 (NT1), a disorder caused by hypocretin/orexin (HCRT) cell loss, is associated with human leukocyte antigen (HLA)-DQ0602 (98%) and T cell receptor (TCR) polymorphisms. Increased CD4 T cell reactivity to HCRT, especially DQ0602-presented amidated C-terminal HCRT (HCRT), has been reported, and homology with pHA flu antigens from pandemic 2009 H1N1, an established trigger of the disease, suggests molecular mimicry. In this work, we extended DQ0602 tetramer and dextramer data to 77 cases and 44 controls, replicating our prior finding and testing 709 TCRs in Jurkat 76 T cells for functional activation.
View Article and Find Full Text PDFArticle Synopsis
- The study investigates the association of certain human leukocyte antigen (HLA) alleles with anti-LG1 encephalitis in a diverse group of 269 patients compared to 1,359 controls.
- The findings highlight a strong link between the DRB1*07:01 allele and the disease, indicating a significant increase in risk, especially in homozygous individuals, while also noting demographic differences among those without the allele.
- Additionally, another allele, DRB1*04:02, shows independent associations with both the disease and younger onset age, suggesting distinct mechanisms of pathogenesis linked to these alleles.