Paroxysmal myoplegia is a neuromuscular disease characterized by a sudden increase in pain localized in the skeletal muscles. The disease is very serious, causing immobility.
What is the reason for his appearance? Are there any prerequisites? What symptoms can be considered alarming? How is diagnosis and treatment carried out? These and many other questions must now be answered.
A bit of history
The pathology in question was first described in 1874. After 6 years, a detailed description of this disease appeared. I. V. Shakhnovich and K. Westphal determined the hereditary nature of the pathology. By the way, at that time the disease was named after them. In the 19th century, myoplegia was called Westphal-Shakhnovich disease.
Time passed, research continued. In 1956, J. Gamstorp studied the hyperkalemic form of the disease (it will be discussed in detail later). This was medical progress. Of course, the disease was given the name "Gamsthorpe's disease".
Just 5 years later, scientists Poscanzer and Kerr made a discovery - they identified a third form of the disease. This is a normokalemic form, which is extremely rare. So few cases are known to medicine that treatment tactics remain undeveloped to date. It was only possible to prove that Diakarb (carbonic anhydrase inhibitor) can help cope with an attack.
Also, experts managed to find out that myoplegia in some cases is secondary, symptomatic. It can arise as a consequence of such pathologies:
- Tumors of the adrenal glands.
- Pyelonephritis.
- Thyrotoxicosis.
- Adrenal insufficiency.
- Diseases of the gastrointestinal tract, accompanied by vomiting and diarrhea.
- Hyperaldosteronism.
- Malabsorption syndrome.
It has also been proven that paralysis can begin to appear in people who have previously had barium poisoning, as well as in those who have abused laxatives and diuretics.
Pathogenesis
Today, the mechanism of development of paroxysmal myoplegia is still the subject of study by physiologists, biochemists and physicians around the world.
Previously it was assumed that this pathology occurs due to increased excretion of aldosterone. The version seemed plausible, since antagonist therapy gave a positive result. However, later it was proved that all changes in the concentration of this hormone are of a secondary nature.
There are speculations regarding geneticdeterministic violation of the permeability of membranes of cell membranes and myofibrils in striated muscle tissue. Allegedly for this reason, water and sodium ions accumulate in the vacuole, and due to the change in balance, neuromuscular transmission is blocked.
If this is the reason, then the pathology can be considered similar to Thomsen's myotonia. So far, the assumptions regarding the dysfunction of cell membrane channels are the most logical and scientifically sound.
Hypokalemic myoplegia
A little attention should be paid to the classification. Hypokalemic paroxysmal myoplegia is the most common form of the disease. It is an autosomal dominant inherited disorder characterized by incomplete gene penetrance.
The risk group includes people aged 10 to 18 years. Pathology is much less common in people over 30, and more often in men than in women.
Neither objective nor subjective signs of hypokalemic paroxysmal myoplegia are determined. But the following factors can provoke an attack:
- Overeating.
- Alcohol abuse.
- Physical surge.
- Eating a lot of s alt or carbohydrates.
Women often have seizures on the first day of their period. Or 1-2 days before it starts.
Seizure
The paroxysm of the hypokalemic type can make itself felt in the morning or at night. Upon awakening, a person realizes that he is paralyzed. Neck, limbs, muscles - everything is constrained. If athe case is severe, the paralysis can even affect the facial or respiratory muscles.
Other manifestations include the following symptoms:
- Sharp muscle hypotension.
- Loss of tendon reflexes.
- Hyperemia of the face.
- Tachycardia.
- Easy breathing.
- Polydipsia.
It is important to note that the attack is also accompanied by disorders of the cardiovascular system. ECG readings change, systolic murmurs appear. But consciousness persists.
An attack can last from one hour to 1-2 days. But such cases are rare. Most of the time it goes away in 2-4 hours. At the end, there is a slow increase in muscle strength, which begins with the distal extremities.
It is possible to quickly restore lost functions if the patient contributes to this with his active movements.
How frequent are seizures? In severe cases, they occur daily. This provokes the appearance of chronic muscle weakness. This phenomenon often leads to malnutrition.
Hyperkalemic myoplegia
This form is much rarer than the above. Paroxysmal myoplegia of this type is characterized by autosomal dominant inheritance (high penetrance). Interestingly, in some cases, this pathology did not pass in families for four generations.
The disease affects both males and females with equal frequency. But this paroxysmal myoplegia in children is most common, it practically does not affect adults. Babies are at riskunder the age of 10.
Provokes an attack of hunger or rest after prolonged physical exertion. First, paresthesias occur in the facial region, then they occur in the limbs. Weakness spreads to the distal legs and arms, and then to other muscle groups. In parallel, there is tendon areflexia and hypotension. Symptoms are as follows:
- Increased heartbeat.
- Excruciating thirst.
- Hypertension.
- Profuse sweat.
- Autonomic disorders.
As a rule, such an attack does not last more than two hours. It is very important to provide assistance in a timely manner, because due to what happened, a person may be completely immobilized (this is called plegia).
Normokalemic myoplegia
This is an extremely rare pathology. The number of cases described in the literature can be counted on the fingers. Paroxysmal myoplegia of this type has a hereditary autosomal dominant nature.
She has severe symptoms. The disease can manifest as moderate weakness or complete paralysis affecting the facial muscles. Hypertrophy is possible, accompanied by the formation of an athletic physique. Paroxysmal myoplegia occurs in children, occurs during the first 10 years of life.
Triggered, as a rule, by intense physical activity or hypothermia. It can also occur due to too much sleep. Manifested by a slow increase in weakness. These sensations pass even more slowly.
This form of the disease is also distinguished by its duration. The attack can pass either in 1-2 days, or in a few weeks.
Diagnosis
She doesn't present any particular difficulties. Diagnosis of paroxysmal myoplegia does not even require specific measures. The disease manifests itself as a typical course of seizures and the absence of neurological disorders during periods of "calm". Plus, there is always a family history.
However, there are sometimes difficulties in establishing a diagnosis. They occur with the initial appearance of a paroxysm, the abortive nature of the attacks, or in a sporadic case.
The diagnosis is verified by a geneticist. It also requires consultations with an endocrinologist and a neurologist.
Periodically you need to donate blood for biochemical analysis. In the periods between attacks, deviations are not detected, but with paroxysms, a reduced serum potassium level is observed - up to 2 mEq / l and even lower. There is also a decrease in the amount of phosphorus, an increase in sugar.
But this is with the hyperkalemic form of paroxysmal myoplegia (ICD-10 code - E87.5). In the case of hypokalemia, a low sugar level and an increased potassium content are recorded.
Sometimes electromyography is done. It in any form allows you to detect the absence of bioelectric activity.
It is also possible to introduce a solution of potassium chloride to a person. If after 20-40 minutes paroxysm begins to develop, then the patient has a hyperkalemic form of the disease. Of course, the solution is administered for diagnostic purposes.
With frequent attacksmay do a muscle biopsy. It often helps to identify the vacuolar form of pathology.
Why do we need diagnostic measures for paroxysmal myoplegia, the symptoms of which already indicate its presence? In order to differentiate the disease from hysteria, myopathy, Conn's disease, Landry's paralysis, as well as from spinal circulation disorders.
Therapy for hypokalemic disease
We should also talk about the treatment of paroxysmal myoplegia. Therapy is carried out differentially, much depends on the form of the disease.
If, for example, the disease has a hypokalemic form, then the attack is eliminated by taking a 10% solution of potassium chloride. You need to use it every hour. Injections of magnesium and potassium aspartate solution are recommended.
When a person's seizures are not disturbed, he needs to take the diuretic Spironolactone. But with great care. In women, it can cause hirsutism, often disrupting the menstrual cycle. Men often suffer from gynecomastia and impotence. Therefore, "Acetazolamide" becomes an alternative. Many experts consider Triamteren to be effective as well.
Diet
It is extremely important to stick to proper nutrition. If a hypokalemic type of paroxysmal myoplegia is diagnosed, it is necessary to reduce the amount of carbohydrates and s alt consumed. It is better to refuse the latter altogether.
It is also necessary to enrich the diet with foods that contain sufficient amounts of potassium. To the "record holders"The contents of this item include:
- Potatoes.
- Dried tomatoes.
- Beans.
- Dried apricots.
- Prunes.
- Avocado.
- Salmon.
- Spinach.
- Pumpkin.
- Oranges.
Tea, soy, cocoa, wheat bran, nuts, seeds, wild garlic and garlic, leafy vegetables, bananas, mushrooms and dairy products are also high in potassium.
Lifestyle
Since we are talking about the symptoms and treatment of paroxysmal myoplegia of the hypokalemic type, we need to pay attention to this topic.
In addition to diet, it is important to follow a certain lifestyle. It is necessary to exclude heavy physical exertion and any overvoltage. But a long stay at rest is also contraindicated. It's important to make a compromise. You can make periodic trips to the pool or evening walks in the fresh air into your regimen.
It is also important to avoid overheating and hypothermia. The temperature should be comfortable.
Hyperkalemic disease therapy
The attacks of this disease are relieved by injection of a 40% glucose solution with insulin or calcium chloride (10%).
Sometimes there is a good result after the inhalation of "Salbutamol". It is believed that this method not only stops seizures, but also prevents them.
During remission periods, the patient should take Acetazolamide or Hydrochlorothiazide.
Diet
In hyperkalemic paroxysmal myoplegia, the causes and symptoms of which were listed earlier, it is necessary to limit the use of all foods fortified with potassium. Enrichment of the diet with carbohydrates and s alt is highly recommended. In general, the provisions are completely opposite to the diet indicated for hypokalemic disease.
In no case should you allow the feeling of hunger to appear, since it is precisely this that provokes seizures. That is why it is important to switch to fractional nutrition, eat at least once every few hours. You can’t overeat, you just need to maintain a full state.
Forecast
It cannot be denied that the disease in question impairs the quality of life and causes problems for the patient. But in general, the prognosis is favorable. Cases of death at the time of seizures are extremely rare. In addition, following therapeutic recommendations and leading the right lifestyle, you can quickly stop them.
And of course the treatment of symptomatic periodic paralysis is important. It involves the treatment of the underlying pathology, as well as the restoration of electrolyte balance.
