Myasthenia gravis: symptoms and treatment
Myasthenia gravis is a neuromuscular disease with a characteristic chronically recurrent or chronically progressive course. Myasthenia gravis, the symptoms of which are manifested in the rapid pathological fatigue noted in the region of the striated musculature, can progress up to these kinds of conditions whose features make them similar to paralysis.
Myasthenia gravis, which is also defined as asthenic bulbar paralysis, is characterized by pronounced muscle weakness and increased fatigue. The disease implies the defeat of cholinergic receptors in postsynaptic membranes. It is possible to involve any muscle in the process, meanwhile, most often there is a tendency to defeat the muscles of the neck, face, pharynx, tongue and eyes.
Until the end is not determined what exactly causes myasthenia gravis. There is a possibility of family cases, but heredity is not proven in the incidence.
A combination of this disease with hyperplasia or with a tumor of the thymus gland is often noted. In some cases, myasthenic syndromes occur in the case of actual organic diseases that affect the nervous system (in particular, amyotrophic lateral sclerosis, as well as other similar diseases). They also occur with dermatomyositis, with breast, lung, prostate or ovarian cancer. Observed this disease can occur in the case of lethargic encephalitis and thyrotoxicosis. It is noteworthy that most often the myasthenia gravis affects women, than men (2: 1). As for the age category falling under a special risk group, the age is 20 to 40 years old.
Myasthenia gravis: symptoms
The disease is characterized in particular by the manifestation of such a basic sign of its weakness, as we already noted above, and the increased fatigue that arise in the skeletal muscles in the case of prolonged work or intense stress. The buildup of muscle weakness occurs when you repeat the movements, especially if they are performed at a fast pace. This leads to fatigue of the muscle, as a result of which she simply ceases to obey, which, in turn, leads her into a condition similar to complete paralysis. It is in a similar, because as the state is reversible — enough to rest, after which the functionality is restored. After a night’s sleep, the patient completely and completely comes to normal state of health. A few hours after the awakening, the growth of myasthenic symptoms begins.
Myasthenia gravis can manifest itself in three main forms:
- An ocular myasthenia gravis;
- Myasthenia gravis
- Myasthenia gravis generalized.
Initially, the affected muscles are innervated by the cranial nerves. Subsequently, the lesion can spread to the muscles of the neck, muscles of the limbs and trunk are affected to the least extent.
The first signs of the disease are the lower eyelid, and also in double vision, resulting from the external muscles of the eye, the muscle, due to which the upper eyelid and the circular muscle of the eye are raised. In the event that the patient after waking can freely and completely open the eye, the subsequent blinking with a progressive pathology provokes a weakening of the muscle, because of what the eyelid «hangs». These signs are characteristic, as can be understood from the symptomatology, for the ocular form of myasthenia gravis.
As for bulbar myasthenia, it is characterized by the defeat of muscles innervated, respectively, by bulbar nerves. Actual manifestations of symptomatology in this case is a violation of chewing and swallowing. Speech also undergoes changes, acquiring nasal, hoarseness and hoarseness. In addition, it also becomes quiet, moreover, depletion of the voice will occur until the speech becomes completely silent.
Most of the patients have a generalized form of the disease, in which the beginning of the process affects the oculomotor muscles, and then the other muscles are also involved. The first muscles that begin to suffer from the generalized form of myasthenia are the mimic muscles and neck muscles. Such features of the course of the disease lead to the fact that the patient becomes difficult to hold his head. It is noted the acquisition of a characteristic facial expression, in which there is a kind of transverse smile, deep wrinkles appear on the forehead. An extreme symptom is salivation.
Next, the introduction of the process of symptoms in the form of weakness of the muscles of the extremities. This leads to difficulties in walking, besides this the patient loses his ability to self-service. In the morning, his condition is improved, by the evening there is a deterioration. Any of the subsequent movements of the patient is given all with great and great difficulty. With the passage of time, muscle atrophy appears. The proximal parts are most affected, that is, the hips and shoulders. There is also a rapid depletion of tendon reflexes, which, after rest, are restored.
Myasthenic crisis: symptoms
Myasthenia gravis, as we have already determined, is a progressive disease, which implies, respectively, an increase in the symptomatology characteristic of this disease, as well as an increase in its severity. The severe form of myasthenia leads to such conditions as myasthenic crises.
By the term «myasthenic crisis» is meant a sudden onset of weakness, concentrated in the region of the pharyngeal and respiratory muscles. This leads to pronounced violations of breathing — it becomes wheezing and rapidity. Also, the pulse becomes more frequent, the pronounced character becomes salivating. It is important to note that paralysis, which under this condition in severe form, experiences respiratory muscles, is a direct threat to the life of the patient.
Given the chronic and progressive nature of myasthenia gravis and the severity of its manifestations, cases of disability are often noted, respectively, when the first anxious symptoms occur, it is necessary not only careful observation, but also appropriate treatment of the patient.
Myasthenia gravis diagnosis
Electromyography is used to diagnose the disease, with the help of which it becomes possible to detect a myasthenic reaction. Also used pharmacological and immunological tests, computed tomography, with which the organs of the anterior mediastinum are examined.
Treatment of myasthenia gravis
In the treatment of myasthenia, the main emphasis is on the use of anticholinesterase drugs (proserine, kalimin). Dosages determined by a specialist must be observed without fail, because otherwise an overdose may develop with the development of cholinergic intoxication (that is, a cholinergic crisis), which is manifested in twitching, cramping, narrowing of the pupils and slowing the pulse, as well as in spasms and pains In the abdomen, in salivary salivation.
Treatment with anticholinesterase drugs is carried out for years with the necessary dosage adjustment in accordance with the severity of the manifestations of the disease. As potentiation of the action of these drugs, potassium salts are also used, the delay in the potassium body is provided by targeted therapy. The severe course of the disease involves the use of cytostatics and hormone therapy.
Significant effectiveness is noted with «pulse therapy», which uses large doses of hormonal drugs with subsequent gradual decrease in them. Immunosuppressants (cyclophosphamide, cyclosporine, aziotioprine) are also used. If a patient is diagnosed with thymoma, surgical intervention is necessary.
Severe cases of myasthenic crisis require the need for artificial ventilation and plasmaphoresis in resuscitation facilities. In addition, the introduction of immunoglobulins, ephedrine and proserin is required.
As for nutrition, a special diet is not foreseen in it. The only thing that is the main recommendation in this regard is the consumption of foods saturated with potassium (potatoes, raisins, dried apricots, etc.).
In case of occurrence of symptoms characteristic for myasthenia, it is necessary to consult a neurologist.