Patient's question:
Weakness in limbs; Onset time is early; The factor or cause is a genetic diseaseDoctor's answer:
[Edit this section] Myasthenia Gravis Myasthenia gravis is a chronic disease caused by a neuromuscular transmission disorder. The clinical features are that the affected skeletal muscles are easily fatigued and partially recover after rest and treatment with anticholinesterase drugs. The occurrence of this disease is related to genetic factors, and it can affect people of any age, but it is most common in people aged 10 to 35, although it can also occur in middle-aged and older individuals. Clinically, it can affect all skeletal muscles, but the most common is the involvement of extraocular muscles. Specific manifestations include ptosis, diplopia, or the simultaneous involvement of all muscles, which worsen with fatigue and partially recover with rest, with symptoms becoming more severe in the afternoon. The range and extent of affected muscle groups vary greatly. Laboratory tests show that 2/3 of patients have elevated serum immunoglobulins. Most patients have elevated acetylcholine receptor (AchR) antibodies in their serum. Chest X-rays and thymus X-rays often show thymic hyperplasia or thymic tumors. Electromyography shows reduced amplitude of muscle action potentials, and single-fiber electromyography shows delayed or blocked inter-fiber excitation transmission. Western medicine primarily treats myasthenia gravis with anticholinesterase drugs and immunosuppressants. Anticholinesterase drugs include neostigmine, pyridostigmine, and edrophonium (also known as mestinon). The side effects of these drugs include miosis, excessive salivation, sweating, abdominal pain, and diarrhea, which can be counteracted by taking atropine simultaneously. Immunosuppressants mainly include corticosteroids and cyclophosphamide. Surgical therapy is suitable for patients with thymoma. If myasthenia gravis affects the medullary muscles and respiratory muscles, making it impossible for the body to maintain normal ventilation function and entering a dangerous state, it is called a myasthenic crisis. In this case, the most important measure is to maintain airway patency. Tracheostomy or nasal intubation should be performed as soon as possible, along with the placement of a nasogastric tube and assisted ventilation. Additionally, it is important to prevent lung infections and gastrointestinal bleeding. In traditional Chinese medicine, this disease falls under the category of "wasting syndrome."[Edit this section] Clinical Manifestations of Myasthenia Gravis Myasthenia gravis is a long-term and difficult-to-treat disease that not only brings physical and psychological suffering to patients but also imposes a significant burden on families and society. Clinically, it can manifest as ptosis, diplopia, strabismus, weakness in facial and masticatory muscles, and may also cause apathy, difficulty blowing out cheeks, and other symptoms. Weakness in medullary muscles can lead to speech difficulties, poor tongue extension, difficulty eating, and choking during eating. The cause of this disease is systemic, but the muscles affected may vary, leading to different clinical manifestations. Specific clinical manifestations of myasthenia gravis: Myasthenia gravis can occur in all age groups but is most common in people aged 15 to 35, with a male-to-female ratio of approximately 1:2. The onset can be either sudden or gradual, often insidious, primarily characterized by skeletal muscle abnormalities and easy fatigue. Muscles are generally stronger in the morning and worsen in the afternoon or evening. Most patients are affected by extraocular muscles, with the levator palpebrae superioris being the most commonly affected. As the disease progresses, more extraocular muscles can be affected, leading to diplopia, and eventually the eyes may become fixed. Intraocular muscles are generally unaffected. Additionally, muscles controlled by the medulla, neck muscles, scapular muscles, trunk muscles, and upper and lower limb muscles can be affected. Speaking for too long may cause the voice to gradually become hoarse, and speech may become unclear with a nasal tone. Due to weakness in the jaw, soft palate, swallowing muscles, and intercostal muscles, chewing and swallowing functions may be affected, and even breathing difficulties may occur. The temporary relief, remission, recurrence, and worsening of symptoms often alternate, forming an important characteristic of the disease. Based on the range and extent of affected muscles, it is generally classified as ocular type, medullary muscle involvement type, and generalized type. There are also very few fulminant cases with rapid onset, where medullary muscle weakness and breathing difficulties can occur within days to weeks. The different types can coexist or transform into one another. Childhood myasthenia gravis refers to cases that develop from newborns to adolescence, with most being limited to extraocular muscles, except for a few who are generalized.
Lifestyle Management: The main treatments are anticholinesterase drugs and immunosuppressants. 1. Anticholinesterase drugs include neostigmine, pyridostigmine, and edrophonium (also known as mestinon). The side effects of these drugs include miosis, excessive salivation, sweating, abdominal pain, and diarrhea, which can be counteracted by taking atropine simultaneously. 2. Immunosuppressants mainly include corticosteroids and cyclophosphamide. 3. Surgical therapy is suitable for patients with thymoma.