We have tested clinical and electrophysiological parameters as well as sera for specific anti-titin-antibodies in 40 myasthenic patients with thymoma, 10 with late-onset and 40 with early-onset without thymoma. We have revealed the prevalence of clinical symptoms: chew and bulbar disturbances, neck and deltoideus muscle weakness in the patients with thymoma and late-onset myasthenia gravis without thymoma. These clinical peculiarities were associated with the absence of changes in duration of minimal motor unit potential after correction for synaptic lesions by proserine injections and increase of titin-antibodies level. The clinical data, along with the results of electrophysiological and immunological studies, suggest a presence of "myasthenic myopathy" in patients with myasthenia, comorbid with thymoma, and patients with late-onset of myasthenia without thymoma.
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Front Immunol
January 2025
Department of Ophthalmology, National University Hospital, National University Health System, Singapore, Singapore.
Autoimmune retinopathy (AIR) is a rare, potentially blinding retinal disease that remains a challenging condition to manage when resistant to conventional immune-modulatory approaches. We report clinical and electrophysiological improvement in a 49-year-old patient who underwent an autologous hematopoietic stem cell transplant (aHSCT) for thymoma-associated AIR after experiencing progressive disease despite receiving periocular and systemic steroids, mycophenolate mofetil, baricitinib, tacrolimus, bortezomib, rituximab, plasmapheresis, and intravenous immunoglobulin. The aHSCT had two stages: (i) peripheral blood stem cell harvest following mobilization with cyclophosphamide and granulocyte colony-stimulating factor, and (ii) conditioning regimen with plasmapheresis, rituximab, cyclophosphamide, and anti-thymocyte globulin high-dose therapy, followed by autologous hematopoietic cell infusion of 5.
View Article and Find Full Text PDFJ Community Hosp Intern Med Perspect
November 2024
Department of Internal Medicine, HCA Healthcare, MountainView Hospital, 2880 N Tenaya Way, 89128, Las Vegas, NV, USA.
Paraneoplastic limbic encephalitis (PLE) is a poorly understood condition, thought to be caused by the cross-reacting of tumor antibodies with neurons in the brain, resulting in neuropsychiatric sequelae, such as personality and behavioral changes, psychosis, memory loss, and seizures. Anti-contactin-associated protein-like 2 (CASPR2) antibodies can cause PLE in patients with particular tumors, which in most cases can be identified as thymoma, lung cancer, or endometrial cancer. Some case reports show rare instances with other tumors, such as throat or sigmoid carcinoma.
View Article and Find Full Text PDFCureus
December 2024
Graduate Medical Education, Eisenhower Health, Rancho Mirage, USA.
Autoimmune enteropathy (AIE) is a rare cause of chronic diarrhea associated with autoantibodies and susceptibility to other autoimmune diseases, such as rheumatoid arthritis, diabetes, autoimmune hemolytic anemia, and atopic dermatitis. While it is more common in children, the prevalence of AIE in adults is increasing. Due to the nonspecific nature of its presenting symptoms and the lack of consistent findings, AIE can be challenging to diagnose.
View Article and Find Full Text PDFBMC Pulm Med
January 2025
Department of Thoracic Surgery, Beijing Chest Hospital, Capital Medical University, Beijing Tuberculosis and Thoracic Tumor Research Institute, No 9, Bei guan Street, Tong Zhou District, Beijing, 101149, P. R. China.
Objectives: Complete removal of the tumor and surrounding tissue is the most important prognostic factor such as survival after surgery. When the tumor invades the phrenic nerve, the impact of intraoperative phrenic nerve sacrifice on the short- and long-term prognosis of patients is not clear. This study aims to explore the differences in prognosis between patients with malignant thymoma with and without phrenic nerve sacrifice during surgery, as well as analyze related factors.
View Article and Find Full Text PDFGland Surg
December 2024
Department of Thoracic Surgery and Oncology, State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, the First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Background: Thymomas and thymic carcinomas are rare and aggressive thymic tumors that are usually detected in advanced stages. Surgery is the mainstay of treatment; however, the role of surgery in advanced disease is controversial due to factors such as myasthenia gravis; thus, decisions about whether to perform surgical interventions are complex. Further studies need to be conducted to explore the potential benefits of surgery in the treatment of advanced thymic tumors.
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