A 59-year-old man presents with expressive aphasia and short term memory deficits. Shortly thereafter, he started developing staring spells and intermittent right hand spasms, preliminarily thought to be simple partial seizures. Subsequent MRI brain imaging was highly suggestive of herpes simplex virus (HSV) encephalitis; however, HSV PCR from cerebrospinal fluid was negative. On further testing, the patient was found to have an autoimmune encephalitis thought to be related to an incidentally found thymoma. His clinical presentation, in conjunction with imaging and response to therapy, was strongly suggestive of thymoma associated paraneoplastic encephalitis. Early recognition is the only way to ensure prompt initiation of appropriate treatment. Immunotherapy and cancer directed therapy (including tumour resection, if indicated) have been shown to have favourable outcomes, improved speed of neurological recovery and reduced risk of relapses. Without treatment, progressive neurologic deterioration can occur over months to years, eventually resulting in death.
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http://dx.doi.org/10.1136/bcr-2019-230709 | DOI Listing |
Ann Thorac Surg Short Rep
December 2024
Division of Cardiothoracic Surgery, University of California, San Diego, California.
Thymomas have been associated with the generation of paraneoplastic autoantibodies to neurogenic epitopes, collapsin-response-mediator protein-5 receptor (CRMP-5) and alpha-amino-3-hydroxyl-5methyl-4isoxazolepropionic acid receptor (AMPAR), in patients with acute viral infection. We report a patient with thymoma and myasthenia gravis, with SARS-CoV-2 infection, who became comatose secondary to autoimmune encephalitis. Plasmapheresis, high-dose steroids, pyridostigmine, eculizumab, and rituximab did not restore neurologic function.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of General Thoracic Surgery, Juntendo University School of Medicine, Tokyo, Japan.
Stiff person syndrome (SPS) is a neurologic disorder, some cases of which are associated with malignant disease. Here, we report a case of thymoma-associated SPS that was successfully treated with surgical resection. A 57-year-old man with progressive muscle stiffness and weakness was diagnosed with thymoma-related SPS.
View Article and Find Full Text PDFMediastinum
November 2024
Department of Oncology, Mayo Clinic, Rochester, MN, USA.
Background: Thymoma is a rare mediastinal neoplasm originating from thymic epithelial cells, often associated with paraneoplastic syndromes. These syndromes can manifest as a range of autoimmune disorders, including myasthenia gravis, pure red cell aplasia, and aplastic anemia. Clinical trials involving the use of immune checkpoint inhibitors (ICIs) in thymoma have been complicated by a high incidence of immune-related adverse effects (irAEs).
View Article and Find Full Text PDFMediastinum
October 2024
Department of General Thoracic Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan.
Background And Objective: Thymic epithelial tumors (TETs) are the most common neoplasm of the prevascular mediastinal compartment and are characterized by their rarity and variable clinical presentation. The present study aimed to explore the current management of patients with TET with a special focus on immunotherapy for advanced disease.
Methods: Relevant studies published between 1981 and 2024 were searched in PubMed using search terms "Thymoma", "Thymic cancer", "Myasthenia gravis", "Radiation therapy", "Surgery", and "Immunotherapy".
Mol Diagn Ther
January 2025
Thoracic Surgery and Lung Transplantation, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, 20122, Milan, Italy.
Objectives: To investigate whether 18F-fluorodeoxyglucose positron emission tomography-computed tomography ([F]F-FDG PET/CT) metabolic parameters were associated with histology and to assess their prognostic role in patients with thymic lesions.
Patients And Methods: In total, 116 patients (49/67 M/F; mean age 59.5 years) who underwent preoperative [F]F-FDG PET/CT and thymectomy from 2012 to 2022 were retrospectively analyzed.
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