Neuromuscular Diseases

Paroxysmal myoplegia is a serious neuromuscular disease

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myoplegia Paroxysmal myoplegia, or how to enter the stone - and return.

And what is there only in the old Russian epic: incredible flights without any adaptations, and unhindered passage through the walls, and many amazing evidences of miraculous healing - there is everything!

There is also a curious story standing on a special place, illustrating the theme: paroxysmal myoplegia( periodic paralysis).

Bylin narrates: sitting on the stove well done. Thirty years and three years he was - he was a paralytic. And then he got up on his feet and became a great Dukhobor. His name was Ilya Muromets.

Why would suddenly? Yes, just the water drank in the right time, yes from his native land. The Magi insisted.

Two questions to the epos: what kind of paralysis is this, then from the bucket of plain water passed? And what kind of water is it that she puts on her feet?

Is not it about periodic paralysis speech? And is it not about the water that is rich in potassium?

paroxysmal myoplegia

Contents

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  • Etiology and variants of the pathology
  • How it happens or about the pathogenesis briefly
  • Common "roots" - but different "leaves"
    • Hypokalemic form
    • About paroxysms of hyperkalemic myoplegia
    • About paroxysms of normokaliemic nature
  • Than confirm or the word to the diagnostician
  • Treatment strategy

Etiology and variants of the pathology of

In the etiology of neuromuscular pathology, referred to as paroxysmal myoplegia( or periodic paralysis), the crucials content of potassium in the blood and in the muscular exercise required for reducing structures.

But talking about it should always be narrowly specific. For it should be specifically about one of the significant differences in the course of the pathology variant, which differ in the saturation of the body with potassium:

  • or low - hypokalemic variant( discovered by Shakhnovich and Westphal, 1882-1885);
  • or high-variant hyperkalemic ( studied by Hamstorp, 1956);
  • or normal - normokaliemic form of flow( existence of which was confirmed by Poskanser and Kerr, 1961).

Among other causes leading to the emergence of secondary, symptomatic family myoplegia, cases are noted:

  • thyrotoxicosis;
  • adrenal neoplasms with the phenomena of hyperaldosteronism and insufficiency of these glands;
  • pyelonephritis;
  • of hypothalamic syndrome;
  • conditions accompanied by dehydration, desalination( with vomiting and diarrhea, from excessive use of diuretics and laxatives, with malabsorption - a syndrome of lack of absorption), or due to excess body content of certain salts( barium, in particular).

The essence of the pathology is in a state of immobility, which comes as a result of the "periodic" paroxysms covering the body of the transient paralysis of the musculoskeletal( skeletal) musculature.

How did Ilya Muromets finish his life? From the stove's stone, having dethroned his body, mature, and stone-immobile, he left again for the stone. Petrified, became again immovable. For the hero is tired of being rich.

As it happens, or about the pathogenesis briefly

An initial theory of Conn, explaining hypokalemic periodic paralysis by intermittent hyperaldosteronism-genetics in neurology, is an episodic increase in the excretion of aldosterone by the adrenal cortex, and is confirmed by the result of using antagonists of this hormone in the treatment of this pathology, has now been revised.

Biochemists and physiologists have found out: for the development of paroxysms of myoplegia, shifts in the level of aldosterone in the blood are a secondary condition.

The main issue here is the permeability of the cell membranes of myofibrillar membranes of skeletal muscles for water with sodium and potassium ions contained in it.

When the balance of these two elements within the cell is disturbed, metabolic and energy disorders begin in the structures of both the muscle tissue itself and in the areas of neuromuscular synapses, while the muscle contraction in the occurrence of the block in neuromuscular transmission is impracticable. To restore this ability of the muscle, it is necessary to return the state of ion balance within it to a stable rate.

And since periodic paralysis of the capacity of muscle membranes for ions is vetoed by a genetic determinant, a clinic of one or another version of the indicated neuromuscular pathology develops.

Common "roots" - but different "leaves" of

Common in the pathogenesis of each form of myoplegia is the autosomal dominant mechanism of inheritance of the state, the question is only in the degree of penetrance of the gene:

  • with hypokalemic - penetrance of the gene is incomplete, low;
  • with hyperkalemic - high;
  • normokaliemic variant - intermediate between the first two, with varying degrees of penetrance of the gene in each individual case.

Symptoms that are characteristic of paroxysmal myoplegia will depend on the form of its course.

Hypokalemic form

The most common and most studied type is often found among men, with a peak of manifestations between the ages of 10 and 18, but with single cases at an older age of 30 years. Until a certain time this form of the disease does not manifest itself in any way.

The onset of muscle paroxysm may result from:

  • of uncontrolled alcohol intake;
  • excess physical stress;
  • non-compliance with diet( with an excessive amount of carbohydrates and / or salt).

Seizure of tachypnea For women, "fatal" for the onset of paroxysm of myoplegia are either the 1st day of menstruation, or the period of 1-2 days before they begin.

Waking up in the morning or at night, the carrier or carrier of the pathology feels in a state of paralysis that has spread not only to the skeletal musculature of the trunk, neck and limbs, but with a serious degree of severity diverging and the muscles are mimic and even respiratory.

The seizure with a duration from hours to days( on average from 2 to 4 hours) is manifested not only by the muscular hypotension and loss of all types of tendon reflexes, but also by the severity of vegetative disorders in the form of:

  • tachycardia and tachypnea;
  • hyperemia of the face and other areas of the skin with the release of profuse sweat the entire surface of the body;
  • excessive thirst - polydipsia.

Gradually muscular strength returns, and this process originates in the most remote areas of the limbs, and as soon as possible restoration of muscle functions is facilitated by undertaken volitional attempts to active movements on the part of the patient himself.

On the frequency of myoplegic paroxysms - it can vary significantly: from daily attacking and having the character of myopligic seizure status in a severe variant, to cases with constantly experienced moderate muscular weakness with strengthening it in the morning hours.

The latter type of disorder contributes to the development of myopathic syndrome, leading to muscle hypertrophy, especially in the proximal segments of the limbs.

An abortively flowing variant with the involvement of only the legs, either one limb or one half of the body, is also possible.

About paroxysms of hyperkalemic myoplegia

The type of paroxysmal paralysis, which is much less common in form due to hypokalemia, often traced to 4 generations of the "pedigree", inherited equally often by people of both sexes, with a manifestation of pathology in the first decade of life.

Provoking paroxysmal moment, is the onset of hunger, including overtaking resting after vigorous physical work: paresthesia appears in the face and limbs, the weakness of the muscles of distant distal segments of limbs, rapidly spreading to the whole musculature of the body, including the facial one, gradually joins.

In addition to the mandatory areflexion of the tendons and general muscle hypotension, paroxysm manifests itself as a powerful autonomic dysfunction in the form of: hypertension and myoplegia

  • expressed palpitation;
  • profuse sweating;
  • arterial hypertension;
  • expressed thirst.

Even the most prolonged paroxysm of this type of periodic paralysis does not take away from those suffering more than 2 hours of life.

About paroxysms of normokaliemic nature

A type inherited like the other two, but described with the example of only four( !) Families, debuting in childhood at the first ten years of life.

The normokaliemic form of paroxysmal myoplegia is a seizure of varying degrees of severity: from a moderately severe but muscular weakness that grasps the whole body to a comprehensive paralysis involving facial muscles.

In some patients, the athletic type of body build is due precisely to muscle hypertrophy due to the presence of this form of myoplegia.

A moment provoking the paroxysm of myoplegia with normokaliemic nature can be both hypothermia and a state of calm rest after intensive training or other physical activity.

For a fit, a slowly growing weakness, embracing the muscles and just as( or even more) slowly decreasing its intensity towards the end of the attack, is characteristic.

This type is characterized by a significant duration of time of one paroxysm - from 1 or 2 days to 2-3 or more weeks.

Than confirm or the word to the diagnostician

In the presence of typical paroxysms characteristic of periodic paralysis, with the absence of neurologic symptoms in the period between attacks, with a characteristic family history-anamnesis, the diagnosis of paroxysmal myoplegia is not difficult.

Questions arise when:

  • first emerged paroxysm;
  • case sporadic category;
  • attacks of abortive, atypical nature.

Biochemical blood tests in the period between seizures have no effect. blood biochemistry

However, this analysis demonstrates:

  • with hypokalemic form - decrease in potassium content in blood serum to 2( or even lower) meq / l versus the norm of 5.5, slightly reduced phosphorus level and an equally pronounced rise in blood glucose;
  • with hyperkalemic variant - a significant increase in potassium level with not a sharp drop in blood glucose;
  • with normokaliemicheskoy myoplegia - no changes in blood levels of potassium, as well as phosphorus and glucose( however, in the period between seizures, they are also normal).

When EMG( electromyography) is performed with myoplegia of any nature, there is no bioelectric muscular activity.

In a variant with a significant frequency of seizures of hypokalemic paralysis leading to the development of myopathic syndrome, a study of a muscle biopsy that reveals vacuolar myopathy is being undertaken.

The diagnostic test with intravenous infusion of the investigated solution of potassium chloride is also competent - with the development of a typical paroxysm for 20-40 minutes, it is found that he has a hyperkalemic variant of periodic paralysis.

Given the genesis of the disease, the diagnosis must be confirmed and verified by a geneticist;compulsory are also consultations with the endocrinologist and neurologist to exclude myoplegia secondary nature.

Treatment strategy

Treatment depends on the form in which paroxysmal myoplegia occurs.

In the presence of a hypokalemic version, the ingestion of a 10% solution of Panangin chloride in the rhythm is suggested to approximate the period of paroxysmal outcome: every hour - from 1 to 2 tablespoons, replaced by either intravenous infusion of the Panangin solution or by ingestion.

In the period between attacks, a good effect is given by taking Veroshpiron( 200 to 300 mg during the day), however, long-term use should be avoided in order to avoid side effects.

In the case of resistance to the drug and for other reasons, it can be replaced with Diacarb( with a daily dose of 0.25-1.25 g in 2-3 doses), or with a potassium-sparing Triamterene( dose 0.2 g / day).

Food should be with a reduced content of table salt, with a reduced caloric intake due to carbohydrates, with the introduction of prunes, dried apricots, cheese, and dairy - foods with large amounts of potassium.

With the development of paroxysm of myoplegia of hyperkalemic etiology, it is quenched by intravenous infusion of a 10% solution of calcium chloride in a 40% glucose solution with an adequate dose of insulin.

A good effect from the inhalation application of Salbutamol has been noted, which both stops seizures and warns them. Diakarb

In the period between seizures, Hypothiazide, Diacarb, is effective.

The diet should be with a minimum of potassium-containing foods, but with lots of carbohydrates and sodium( salt), breaks between meals should not be too long - paroxysms are provoked by hunger.

In terms of treatment of normokaliemic myoplegia, the most common recommendations are: use, as necessary, of Diacarb, an increase of up to 10 g of sodium intake - table salt.

Therapy of symptomatic periodic paralysis is the treatment of the underlying pathology and the restoration of electrolyte equilibrium.

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