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Neurological Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Antibodies to the nicotinic acetylcholine receptor cause competitive inhibition of transmission at the neuromuscular junction. Some drugs, including penicillamine, may cause myasthenia gravis and others, including gentamicin and tetracycline, may worsen pre-existing disease. There may be an associated thymoma, particularly in patients over 40 years of age.
Neuromuscular Junction Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Qin Li Jiang
Chest computed tomography (CT) or magnetic resonance imaging (MRI) should be done in every MG patient to evaluate for the presence of thymoma. Intravenous iodine contrast must be used with caution, as it may exacerbate MG symptoms.
Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Thymic carcinomas are very rare, accounting for approximately 20% of thymic epithelial tumours, occurring predominantly in men between 40 and 60 years of age (48). Unlike thymomas, most patients present with non-specific thoracic symptoms including cough, shortness of breath, and chest pain (49). In addition, many patients also complain of systemic features including fatigue, weight loss, and anorexia. Patients may also present with late, locally extensive disease including superior vena cava syndrome and cardiac tamponade (50).
Therapeutic options in thymomas and thymic carcinomas
Published in Expert Review of Anticancer Therapy, 2022
Thymomas progress relatively slowly and rarely metastasize to extrathoracic parts of the body. They are complicated by autoimmune disorders such as myasthenia gravis (MG), pure red cell aplasia (PRCA), hypogammaglobulinemia, and systemic lupus erythematosus (SLE). Pathologically, thymomas are classified into five major subtypes: type A (including atypical subtype), AB, B1, B2, B3, and other rare types (micronodular thymoma with lymphoid stroma, metaplastic thymoma, and lipofibroadenoma of the thymus), based on the WHO classification of 2021. Types A and AB thymomas have spindle-shaped epithelial cells, with few and many immature lymphocytes, respectively. Type B1 and B2 thymomas are both lymphocyte-rich tumors. Type B1 thymoma has a thymus-like architecture and cytology, whereas type B2 thymoma has a higher than a normal number of polygonal neoplastic epithelial cells. Type B3 thymoma is a lymphocyte-poor epithelial-rich tumor. Distinguishing between these types is often difficult. The 10-year survival rate of patients with thymomas of type A, AB, or B1 is more than 90%, whereas the 5-year survival rates of those with type B2 and B3 thymomas are 70% and 75%, respectively [3].
Eculizumab for the treatment of myasthenia gravis
Published in Expert Opinion on Biological Therapy, 2020
Renato Mantegazza, Paola Cavalcante
A consensus-based guidance for MG management has been developed by a panel of 15 international experts, convened by a Task Force of the MGFA, to be a guide for clinicians worldwide [14,15]. A stepwise treatment approach is recommended for gMG, beginning with cholinesterase inhibitors, that prolong acetylcholine availability at the NMJ (first-line treatment), followed by immunosuppressive therapy (IST) with corticosteroids, used chronically in most patients [14–16]. If corticosteroids are not effective or exhibit contraindications, patients can be treated with other IS agents, alone or combined with steroids (second-line treatment). Immune globulin (IVIg) and plasmapheresis (PE) are recommended for MG exacerbation or crises to obtain rapid improvement [14–16]. Finally, thymectomy, mandatory for thymoma patients, is able to improve the disease course in non-thymomatous patients [17,18].
Respiratory insufficiency from myasthenia gravis and polymyositis due to malignant thymoma triggering Takotsubo syndrome
Published in International Journal of Neuroscience, 2018
Josef Finsterer, Claudia Stöllberger, Chen-Yu Ho
Myasthenic crises have been repeatedly reported to trigger TSS (Table 1). The current patient, however, is unique since MG was associated with malignant thymoma and since the response to cholinergic drugs and plasmapheresis was poor. Including the present case, 15 patients have been reported so far in whom TTS was triggered by complications of MG (Table 1) [4–17]. Thirteen patients were female and two were males. Age at onset of MG ranged from 40 to 83 years. In four patients, a thymoma was diagnosed. In three patients, it was not reported whether there was a thymoma or not. The type of TTS was reported in 13 patients, 12 patients presented with the classical type and a single patient had the global type. The presumed trigger of TTS was a myasthenic crisis in 14 patients and unknown in one patient (Table 1). Only seven patients received treatment for TTS. The outcome of TTS was reported in 13 patients. Among these, full recovery was achieved in 10 patients (76.9%). In 3/13 patients (23%), the outcome was fatal.