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34 citation(s) found for "Shen, Xin".

Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients
Xie, Nairong , Liu, Qing , Wang, Yaye , Di, Li , Zhu, Wenjia , Wen, Xinmei , Shen, Xin‐Ming , Da, Yuwei , Da, Yuwei , Wen, Qi, Liu, Haoran , Jiang, Yuting , Lü, Yan , Di, Li, Wang, Min , Zhang, Xuxiang

Mayo Clinic

Therapeutic Advances in Neurological Disorders | January 8, 2025 | Volume: 18 | Issue: No issue available | Pages: No page available

10.1177/17562864241309793 | PMID 39803329 Get It

Background: Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes. Objectives: To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years. Design: This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years). Methods: A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis. Results: Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months ( p = 0.635) or 24 months ( p = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, p = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis ( p = 0.007 and p = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors ( p = 0.025, p = 0.004, p = 0.025, and p < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups. Conclusion: This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.
Undiagnosed Congenital Myasthenic Syndromes in Adult Neuromuscular Practice: a Long Road to Diagnosis and Treatment. (P2.033)
Kao, Justin, Milone, Margherita , Selcen, Duygu , Shen, Xin‐Ming , Engel, Andrew, Liewluck, Teerin

Mayo Clinic

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.p2.033 Get It

Congenital Myasthenic Syndromes (CMS) Due to Impaired Principal Coupling Pathway in the ɛ Subunit of Muscle Acetylcholine Receptor (AChR) (P2.031)
Shen, Xin‐Ming , Brengman, Joan M., Shen, Shelley , Durmuş, Hacer , Deymeer, Feza , Sine, Steven M. , Engel, Andrew, Preethish‐Kumar, Veeramani , Yüceyar, Nur , Vengalil, Seena , Nalini, Atchayaram

Mayo Clinic

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.p2.031 Get It

Determinants of the Repetitive-CMAP Occurrence and Therapy Efficacy in Slow-Channel Myasthenia (1253)
Di, Li , Chen, Hai , Selcen, Duygu , Engel, Andrew, Da, Yuwei , Shen, Xin‐Ming , Di, Li, Lü, Yan

Mayo Clinic

Neurology | December 7, 2023 | Volume: 96 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.96.15_supplement.1253 Get It

A Spontaneously Resolving Pyridostigmine Responsive Congenital Myasthenic Syndrome Caused by Isolated PREPL Deficiency (S45.004)
Selcen, Duygu , Shen, Xin , Meulemans, Sandra, Creemers, John W.M. , Engel, Andrew, Régal, Luc

Mayo Clinic

Neurology | December 7, 2023 | Volume: 80 | Issue: 7_supplement | Pages: No page available

10.1212/wnl.80.7_supplement.s45.004 Get It

Investigation of Congenital Myasthenic Syndrome (CMS) Reveals Asymmetric Contribution of Invariant Cys-Loop Aspartate Residues Critical for AChR Channel Opening (S45.002)
Shen, Xin , Brengman, Joan M., Neubauer, David , Engel, Andrew G.

Mayo Clinic

Neurology | December 7, 2023 | Volume: 80 | Issue: 7_supplement | Pages: No page available

10.1212/wnl.80.7_supplement.s45.002 Get It

Investigation of 11 Patients with GFPT1-Myasthenia Reveals Clinical, Structural, and Electrophysiologic Heterogeneity (S45.001)
Selcen, Duygu , Shen, Xin , Milone, Margherita , Brengman, Joan M., McQuillen, Michael, Deymeer, Feza , Rowin, Julie, Ohno, Kinji , McQuillen, Michael, Finkel, Richard S. , Engel, Andrew G.

Mayo Clinic

Neurology | December 7, 2023 | Volume: 80 | Issue: 7_supplement | Pages: No page available

10.1212/wnl.80.7_supplement.s45.001 Get It

A Spontaneously Resolving Pyridostigmine Responsive Congenital Myasthenic Syndrome Caused by Isolated PREPL Deficiency (IN1-1.001)
Selcen, Duygu , Shen, Xin , Meulemans, Sandra, Creemers, John W.M. , Engel, Andrew, Régal, Luc

Mayo Clinic

Neurology | December 7, 2023 | Volume: 80 | Issue: 7_supplement | Pages: No page available

10.1212/wnl.80.7_supplement.in1-1.001 Get It

Mutant SNAP25B Causes Novel Congenital Myasthenic Syndrome (CMS), Cortical Hyperexcitability, Ataxia, and Intellectual Disability (S34.003)
Shen, Xin , Selcen, Duygu , Brengman, Joan M., Engel, Andrew

Mayo Clinic

Neurology | December 6, 2023 | Volume: 84 | Issue: 14_supplement | Pages: No page available

10.1212/wnl.84.14_supplement.s34.003 Get It

Congenital myasthenic syndrome (CMS) caused by novel mutation in LRP4. Phenotypic heterogeneity and defects in neuromuscular transmission (NMT) identified in a second kinship (P2.021)
Selcen, Duygu , Shen, Xin‐Ming , McEvoy, Kathleen M., Engel, Andrew, Ohkawara, Bisei , Ohno, Kinji

Mayo Clinic

Neurology | December 6, 2023 | Volume: 84 | Issue: 14_supplement | Pages: No page available

10.1212/wnl.84.14_supplement.p2.021 Get It