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Your search keyword '"Lennon, Vanda A."' showing total 694 results
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694 results on '"Lennon, Vanda A."'

2. Neurological autoimmunity in patients with non‐pulmonary neuroendocrine neoplasms: clinical manifestations and neural autoantibody profiles.

Author

Mangioris, Georgios, Halfdanarson, Thorvardur R., Lennon, Vanda A., Chang, Bryce K., Dubey, Divyanshu, Dyck, P. James B., Flanagan, Eoin P., McKeon, Andrew, Mills, John R., Pittock, Sean J., and Zekeridou, Anastasia

Subjects
NEUROENDOCRINE tumors, SYMPTOMS, AUTOIMMUNE diseases, AUTOANTIBODIES, NEUROMUSCULAR diseases, AUTOIMMUNITY
Abstract

Background and purpose: Paraneoplastic neurological autoimmunity is well described with small‐cell lung cancer, but information is limited for other neuroendocrine neoplasms (NENs). Methods: Adult patients with histopathologically confirmed non‐pulmonary NENs, neurological autoimmunity within 5 years of NEN diagnosis, and neural antibody testing performed at the Mayo Clinic Neuroimmunology Laboratory (January 2008 to March 2023) were retrospectively identified. Control sera were available from patients with NENs without neurological autoimmunity (116). Results: Thirty‐four patients were identified (median age 68 years, range 31–87). The most common primary tumor sites were pancreas (nine), skin (Merkel cell, eight), small bowel/duodenum (seven), and unknown (seven). Five patients received immune checkpoint inhibitor (ICI) therapy before symptom onset; symptoms preceded cancer diagnosis in 62.1% of non‐ICI‐treated patients. The most frequent neurological phenotypes (non‐ICI‐treated) were movement disorders (12; cerebellar ataxia in 10), dysautonomia (six), peripheral neuropathy (eight), encephalitis (four), and neuromuscular junction disorders (four). Neural antibodies were detected in 55.9% of patients studied (most common specificities: P/Q‐type voltage‐gated calcium channel [seven], muscle‐type acetylcholine receptor [three], anti‐neuronal nuclear antibody type 1 [three], and neuronal intermediate filaments [two]), but in only 6.9% of controls. Amongst patients receiving cancer or immunosuppressive therapy, 51.6% had partial or complete recovery. Outcomes were unfavorable in 48.3% (non‐ICI‐treated) and neural autoantibody positivity was associated with poor neurological outcome. Discussion: Neurological autoimmunity associated with non‐pulmonary NENs is often multifocal and can be treatment responsive, underscoring the importance of rapid recognition and early treatment. [ABSTRACT FROM AUTHOR]

Published
2024
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3. Independent CHRNE mutations at serine 503 in English Springer Spaniels and a Smooth Fox Terrier having congenital myasthenic syndrome.

Author

Peterson, Erin, Rudolph, Tori E., Starr‐Moss, Alison, Anderson, Kendall, Lennon, Vanda A., Shelton, G. Diane, and Clark, Leigh Anne

Subjects
CONGENITAL myasthenic syndromes, ANIMAL genetics, NEUROMUSCULAR diseases, GENETIC mutation, GOLDEN retriever
Abstract

This article discusses the discovery of independent CHRNE mutations in English Springer Spaniels (ESSs) and a Smooth Fox Terrier (SFT) with congenital myasthenic syndrome (CMS), which are inherited disorders affecting neuromuscular transmission. The affected dogs exhibited muscle weakness and fatigue, and skeletal muscle biopsies revealed a deficiency of acetylcholine receptors (AChRs). Through genetic sequencing, unique point mutations were identified in the CHRNE gene in both breeds. The study also highlights other gene mutations associated with CMS in different dog breeds. The authors conclude that these findings contribute to a better understanding of CMS in dogs. [Extracted from the article]

Published
2024
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4. Astrocyte-microglia interaction drives evolving neuromyelitis optica lesion

Author

Chen, Tingjun, Lennon, Vanda A., Liu, Yong U., Bosco, Dale B., Li, Yujiao, Yi, Min-Hee, Zhu, Jia, Wei, Shihui, and Wu, Long-Jun

Subjects
Neuromyelitis optica, Autoimmunity, Autoantibodies, Immunoglobulin G, Aquaporins, Health care industry
Abstract

Neuromyelitis optica (NMO) is a severe inflammatory autoimmune CNS disorder triggered by binding of an IgG autoantibody to the aquaporin 4 (AQP4) water channel on astrocytes. Activation of cytolytic complement has been implicated as the major effector of tissue destruction that secondarily involves myelin. We investigated early precytolytic events in the evolving pathophysiology of NMO in mice by continuously infusing IgG (NMO patient serum-derived or AQP4-specific mouse monoclonal), without exogenous complement, into the spinal subarachnoid space. Motor impairment and sublytic NMO-compatible immunopathology were IgG dose dependent, AQP4 dependent, and, unexpectedly, microglia dependent. In vivo spinal cord imaging revealed a striking physical interaction between microglia and astrocytes that required signaling from astrocytes by the C3a fragment of their upregulated complement C3 protein. Astrocytes remained viable but lost AQP4. Previously unappreciated crosstalk between astrocytes and microglia involving early-activated CNS-intrinsic complement components and microglial C3a receptor signaling appears to be a critical driver of the precytolytic phase in the evolving NMO lesion, including initial motor impairment. Our results indicate that microglia merit consideration as a potential target for NMO therapeutic intervention., Introduction Neuromyelitis optica (NMO) is an inflammatory autoimmune disorder of the central nervous system (CNS) with secondary myelin loss. It disproportionately affects women and non-White populations, typically presents as optic [...]

Published
2020
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9. Alterations in Aquaporin‐4‐IgG Serostatus in 986 Patients: A Laboratory‐Based Longitudinal Analysis.

Author

Majed, Masoud, Valencia Sanchez, Cristina, Bennett, Jeffrey L., Fryer, James, Mulligan, Martin D., Redenbaugh, Vyankya, McKeon, Andrew, Mills, John R., Wingerchuk, Dean M., Lennon, Vanda A., Weinshenker, Brian, Chen, John J., Flanagan, Eoin P., Pittock, Sean J., and Kunchok, Amy

Subjects
LOGISTIC regression analysis, ODDS ratio
Abstract

Objective: This study was undertaken to investigate factors associated with aquaporin‐4 (AQP4)‐IgG serostatus change using a large serological database. Methods: This retrospective study utilizes Mayo Clinic Neuroimmunology Laboratory data from 2007 to 2021. We included all patients with ≥2 AQP4‐IgG tests (by cell‐based assay). The frequency and clinical factors associated with serostatus change were evaluated. Multivariable logistic regression analysis examined whether age, sex, or initial titer was associated with serostatus change. Results: There were 933 patients who had ≥2 AQP4‐IgG tests with an initial positive result. Of those, 830 (89%) remained seropositive and 103 (11%) seroreverted to negative. Median interval to seroreversion was 1.2 years (interquartile range [IQR] = 0.4–3.5). Of those with sustained seropositivity, titers were stable in 92%. Seroreversion was associated with age ≤ 20 years (odds ratio [OR] = 2.25; 95% confidence interval [CI] = 1.09–4.63; p = 0.028) and low initial titer of ≤1:100 (OR = 11.44, 95% CI = 3.17–41.26, p < 0.001), and 5 had clinical attacks despite seroreversion. Among 62 retested after seroreversion, 50% returned to seropositive (median = 224 days, IQR = 160–371). An initial negative AQP4‐IgG test occurred in 9,308 patients. Of those, 99% remained seronegative and 53 (0.3%) seroconverted at a median interval of 0.76 years (IQR = 0.37–1.68). Interpretation: AQP4‐IgG seropositivity usually persists over time with little change in titer. Seroreversion to negative is uncommon (11%) and associated with lower titers and younger age. Seroreversion was often transient, and attacks occasionally occurred despite prior seroreversion, suggesting it may not reliably reflect disease activity. Seroconversion to positive is rare (<1%), limiting the utility of repeat testing in seronegative patients unless clinical suspicion is high. ANN NEUROL 2023;94:727–735 [ABSTRACT FROM AUTHOR]

Published
2023
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10. Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder

Author

Chen, John J., Flanagan, Eoin P., Bhatti, M. Tariq, Jitprapaikulsan, Jiraporn, Dubey, Divyanshu, Lopez Chiriboga, Alfonso (Sebastian) S., Fryer, James P., Weinshenker, Brian G., McKeon, Andrew, Tillema, Jan-Mendelt, Lennon, Vanda A., Lucchinetti, Claudia F., Kunchok, Amy, McClelland, Collin M., Lee, Michael S., Bennett, Jeffrey L., Pelak, Victoria S., Van Stavern, Gregory, Adesina, Ore-Ofe O., Eggenberger, Eric R., Acierno, Marie D., Wingerchuk, Dean M., Lam, Byron L., Moss, Heather, Beres, Shannon, Gilbert, Aubrey L., Shah, Veeral, Armstrong, Grayson, Heidary, Gena, Cestari, Dean M., Stiebel-Kalish, Hadas, and Pittock, Sean J.

Published
2020
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13. GABAA receptor autoimmunity: A multicenter experience

Author

OʼConnor, Kevin, Waters, Patrick, Komorowski, Lars, Zekeridou, Anastasia, Guo, Chu-Yueh, Mgbachi, Victor C., Probst, Christian, Mindorf, Swantje, Teegen, Bianca, Gelfand, Jeffrey M., Geschwind, Michael D., Lennon, Vanda, Pittock, Sean J., and McKeon, Andrew

Published
2019
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16. Autoimmune septin-5 cerebellar ataxia

Author

Honorat, Josephe A., Lopez-Chiriboga, A. Sebastian, Kryzer, Thomas J., Fryer, James P., Devine, Michelle, Flores, Angela, Lennon, Vanda A., Pittock, Sean J., and McKeon, Andrew

Published
2018
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37. Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum

Author

Flanagan, Eoin P., Cabre, Philippe, Weinshenker, Brian G., Sauver, Jennifer St, Jacobson, Debra J., Majed, Masoud, Lennon, Vanda A., Lucchinetti, Claudia F., McKeon, Andrew, Matiello, Marcelo, Kale, Nilifur, Wingerchuk, Dean M., Mandrekar, Jay, Sagen, Jessica A., Fryer, James P., Robinson, Angala Borders, and Pittock, Sean J.

Published
2016
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41. distinctive IgG-mediated pathogenesis for primary progressive multiple sclerosis?

Author

Guo, Yong and Lennon, Vanda A

Subjects
*MULTIPLE sclerosis, *MYELIN sheath diseases, *RUBELLA, *NEUROMYELITIS optica
Abstract

This scientific commentary refers to 'Cerebrospinal fluid immunoglobulins in primary progressive multiple sclerosis are pathogenic' by Wong I et al. i (https://doi.org/10.1093/brain/awad031). CSF, CSF-IgG and recombinant IgG derived from B cells isolated from the CSF of patients with PPMS had variable pathogenic effects when injected into the C4-5 subarachnoid space of mice. The findings thus suggest the existence of a pathogenic IgG that is exclusive to PPMS-CSF and is, in this subset of patients, a major driver of multiple sclerosis pathology. [Extracted from the article]

Published
2023
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46. Identification of Caveolae-Associated Protein 4 Autoantibodies as a Biomarker of Immune-Mediated Rippling Muscle Disease in Adults.

Author

Dubey, Divyanshu, Beecher, Grayson, Hammami, M. Bakri, Knight, Andrew M., Liewluck, Teerin, Triplett, James, Datta, Abhigyan, Dasari, Surendra, Zhang, Youwen, Roforth, Matthew M., Jerde, Calvin R., Murphy, Stephen J., Litchy, William J., Amato, Anthony, Lennon, Vanda A., McKeon, Andrew, Mills, John R., Pittock, Sean J., and Milone, Margherita

Published
2022
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