Myasthenia Gravi; It is an autoimmune disease mediated by antibodies against acetylcholine receptors at the muscle-nerve junction.
What May Be the Symptoms of Myasthenia Gravis?
Patients with myasthenia gravis typically complain of clinically fluctuating painless weakness in skeletal muscles. Ocular involvement resulting in droopy eyelid and double vision is the first finding in approximately 40% of cases. Difficulty speaking, difficulty swallowing, weakness in the neck and arms, legs may occur. Loss of facial expression, difficulty in speaking, chewing and swallowing may also occur due to the involvement of the bulbar region in the brain and weakness in the facial muscles. Muscle weakness is usually in the eye and bulbar muscles and in the proximal muscles of our arm and leg muscles close to the body. The most serious symptom of Myasthenia Gravis is breathing difficulties. If airway patency cannot be adequately provided due to the weakness of the inspiratory muscles that allow us to breathe, respiratory support may be needed. p> Myasthenia Gravis can occur in any race and at any age. However, the frequency increases in the 3rd decade in women and in the 6th and 7th decades in men.
How to Diagnose Myasthenia Gravis?
Diagnosis of Myasthenia Gravis; Typical clinical signs are determined by positive response to anticholinesterases, the presence of anti-AChR antibodies, anti-MuSK antibodies, and decrement with electrophysiological low frequency sequential nerve stimulation test or increased jitter on single fiber EMG.
Myasthenia gravis. Examination of the thymus gland has an important place in patients with thymus gland, enlargement of the thymus gland (hyperplasia) can be seen frequently, and a thymus gland tumor (thymoma) can be detected in a group of 10% of patients. In patients diagnosed with myasthenia gravis, the thymus gland should be evaluated by non-contrast thorax computed tomography examination.
Anti-AChR antibodies are detected in 67-93% of myasthenia gravis patients. This group is defined as AChR+ MG. The patient with no acetylcholine receptor antibody detected Anti-MuSK (muscle specific kinase) antibody is positive in 40-70% of patients, this group of patients is defined as MuSK+ MG. The type of disease in which AChR and MuSK antibodies are not detected but showing typical clinical signs and electrophysiological features of myasthenia gravis is seronegative Myasthenia Gravis.
What is Myasthenia Gravis with Eye Findings?
Myasthenia Gravis with eye findings is serologically different from generalized Myasthenia Gravis. AChR antibodies are positive 50% of the time and have a lower titer. In recent years, LRP4 protein (low density lipoprotein receptor related protein 4) has been identified as an agrin receptor. It is reported to activate MuSK and increase AChR aggregation, and anti-LRP4 is thought to be a new diagnostic marker in seronegative MG.
Is There a Treatment for Myasthenia Gravis?
In the treatment of Myasthenia Gravis The first step is to use it orally. Pyridostigmine from this class is used in cases of mild myasthenia.
Corticosteroids are also of great importance in the treatment of myasthenia gravis. In some of the patients, muscle weakness may increase temporarily one week to 10 days after starting steroids. Therefore, it is recommended to start with a slow increase in prednisolone. It is recommended to reduce the steroid dose after recovery.
Immunosuppressive therapy in the treatment of Myasthenia Gravis: Azathioprine can be added to the treatment initially or later. It can be added to the treatment, especially in patients who cannot take steroids, when there is an exacerbation while reducing the steroid dose, or when a rapid reduction of the corticosteroid dose is desired. Mycophenolate mofetil, Methotrexate, Cyclosporine, Cyclophosphamide are other immunosuppressant agents that are alternative to azathioprine in the treatment. It should definitely be done in Myasthenia Gravis patients with thymoma. It is one of the main steps of treatment in patients without thymoma, especially in patients with early-onset generalized AChR+ MG.
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