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Myasthenia Gravis (MG) is a neuromuscular disease characterized by fatigue and weakness that worsens with exercise and improves with rest. Etymologically, the word myasthenia comes from the Greek “mys” (muscle) and “astheneia” (weakness), while “gravis” (severe) derives from Latin.
MG is classified as a rare disease, meaning it is uncommon. Its estimated prevalence is 50 to 200 cases per million people. It has a bimodal presentation: in women, it typically begins before the age of 40, while in men it usually appears around 50 years of age. It is rare in children. Recent epidemiological studies have described an increase in the incidence of late-onset cases (after age 60) without a clear sex predominance.
The main initial symptom of myasthenia gravis (MG) is muscle weakness and fatigue, which vary throughout the day. Muscle weakness worsens progressively during periods of physical activity and improves after rest. Many patients also report fluctuations related to seasons, work or psychological stress, menstruation, or intercurrent infections. In general, weakness and fatigue worsen toward the end of the day. These fluctuations and the worsening with physical activity are key signs to differentiate MG from other conditions, such as mental fatigue or chronic fatigue syndrome, where tiredness appears in the morning or at rest.
MG usually begins with ocular symptoms, such as double vision (diplopia) and/or drooping eyelids (ptosis), which can be unilateral or bilateral. Ocular symptoms often worsen when watching TV, reading, driving, or in bright light. Symptoms from bulbar muscle involvement are also common, including difficulty swallowing, chewing, or speaking. Approximately 16% of patients present initially with one of these bulbar symptoms. Other possible manifestations include difficulty holding the head upright, inability to keep the mouth closed, shortness of breath, early fatigue when walking, turning in bed, raising arms, or opening bottles or jars.
Approximately 20% of patients may experience a myasthenic crisis during their lifetime, causing serious respiratory or swallowing problems that may require hospitalization. Crises can be triggered by stress, infections, medications, surgery, or even emotional stress.
Diagnosis of MG is based on symptoms, physical examination, and paraclinical tests. The most important tests include electromyography (EMG and SF-EMG) and the edrophonium test (Tensilon®). In most patients, antibodies (AChR and MuSK) can be detected in serum. A small percentage of patients are seronegative. A CT scan is recommended to rule out the presence of a thymoma.
Treatment usually involves acetylcholinesterase inhibitors combined with immunosuppressive medications. Most of these drugs have dose-dependent side effects that must be considered. Treatment decisions should be made jointly with the patient, taking into account disease guidelines, physician experience, and the clinical form of MG. Care of an MG patient typically involves lifelong follow-up with a neurologist.
Thymectomy (surgical removal of the thymus) can improve symptoms, prevent certain complications, and reduce the dose of immunosuppressive drugs. It is recommended for generalized MG in patients under 55 years of age and in all patients with thymoma, regardless of age.
Plasmapheresis and intravenous immunoglobulins (IVIG) can be used during exacerbations. In myasthenic crises, mechanical ventilation may be required.
With proper treatment, most patients lead relatively normal lives and have a normal life expectancy.
Pregnancy: Physiological changes during pregnancy (nausea, vomiting, weight gain, increased blood volume, changes in kidney function, and altered gastrointestinal absorption) can affect MG and may require medication dose adjustments. Pregnant women with MG are at higher risk of complications and adverse effects. About one-third of pregnancies experience clinical worsening of MG, mainly in the first and third trimesters and postpartum. Some medications used in MG can cause fetal malformations or increase the risk of miscarriage or low birth weight.
Newborns of MG mothers may present transient neonatal myasthenia due to maternal antibody transfer.
Because complications during pregnancy and delivery are unpredictable, specialized multidisciplinary care is recommended, involving at least a neurologist, obstetrician, anesthesiologist, and neonatologist.
Surgery, particularly thymectomy, can precipitate a myasthenic crisis. High disease functional scores, low spirometric values, and severe comorbidities are major risk factors. Risk assessment should involve pulmonologists, surgeons, and anesthesiologists.
Procedures under local, regional, or epidural anesthesia generally do not carry a risk of myasthenic crisis.
Some medications may worsen myasthenic symptoms, so it is important to inform healthcare providers about MG before prescribing new drugs. Certain vaccines, foods, and beverages may also be contraindicated. A list of safe medications is available at: https://www.neuromuscularbarcelona.org/miastenia/
Many MG patients also have other autoimmune disorders. The most common is hypothyroidism.
Daily gentle exercise with rest periods is recommended, considering the fluctuating nature of symptoms. Respiratory exercises at home can complement training. Maintaining a healthy weight is very important.
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