The family myoplegia

Myoplegia family (synonym: myoplegia paroxysmal, myoplegia periodic, paroxysmal paralysis, periodic paralysis of the limbs) is a disease of the nervous system, characterized by the development of periodic bouts flaccid paralysis of skeletal muscles, leading to complete immobility of the patient.
Genetics family mioplegii well studied on large families where the disease is transmitted from generation to generation (dominant). Men suffer more often than women, and the disease occurs in them heavier.
During the attack in the blood decreases the level of potassium and disrupted the intracellular metabolism in skeletal muscle and mineralnych synapses. Bouts of immobility develop suddenly, in the midst of perfect health, and last from several hours up to 1 - 2 days. Initially affects the proximal parts of the limbs, then distal, and the muscles of the neck. The muscles of the face in the process is not involved. Fade tendon and periosteal reflexes. Patients completely immobilized. The frequency of attacks range from a few over a lifetime to weekly or even daily. Attacks provoked taking copious quantities of fatty and sweet food, especially adopted before bed, large physical stress, sometimes excitement.
The disease begins in the second decade, and to the elderly age attacks become more rare.
Diagnostics family mioplegii in family cases does not represent any difficulties. In sporadic cases in the diagnosis should be aware of the attacks, sometimes when developing chronic pyelonephritis, which is also the loss of potassium, and when the tumor or adrenal hyperplasia. Status similar to attack the family mioplegii, can meet and treatment of diabetes with insulin, an overdose of ACTH.
The prognosis of the disease in respect of life favorable, but full recovery does not occur.
The treatment is carried out on the background of the corresponding mode (you need to avoid provocative factors, especially overeating). In the interictal period applies disodium salt adenosine triphosphate intramuscularly in a dose of 1 ml 1% solution every day, 30 to 40 injections per course. It is recommended that a diet rich in potassium salts (potatoes, prunes and others). During the attack appointed caffeine, neostigmine (1 ml 0.05% solution), 10% solution of potassium chloride by 1 table, L. every 2 hours.
Cm. also Hereditary diseases, Paralysis, paresis.

Myoplegia family (myoplegia familialis, from the Greek. mys, myos - muscle and plege a shot, defeat; synonym: myoplegia paroxysmal, myoplegia periodic, paroxysmal paralysis, periodic paralysis of the limbs) is a family disease of the nervous system, characterized by the development of periodic bouts flaccid paralysis of skeletal muscles, leading to complete immobility of the patient.
The etiology of the disease is not completely understood. A significant role is played by genetic factors (genetic research data see below, section Genetics), however, there are sporadic cases.
The pathogenesis of the disease difficult. Recently suggested pituitary-diencephalic nature of the process with the disorder of the Central vegetative regulation, leading to periodic disorder of metabolism. During the attack in the serum decreased levels of potassium and disrupted the intracellular metabolism in skeletal muscle and mineralnych contacts. It is also possible pathogenetic proximity of mioplegii to myotonia (see), the gradual development of which is observed in patients with myoplegia.
Pathological anatomy. In cases ended with lethal outcomes have been identified vacuoles in the Central part of the muscle fibers, once considered an artifact, as well as increasing the number of cores sarcolemma.
Over and symptoms. Bouts of immobility develop suddenly, in the midst of perfect health, and last from several hours to 1-2 days. Initially affects the proximal parts of the limbs, and then and distal muscles of the trunk and neck. The muscles of the face remains intact. Altitude attack fade tendon and periosteal reflexes-patients completely immobilized. Noted the disappearance of elektrovozoremontnij nerves and muscles paralyzed limbs. Consciousness remains. Sensitivity is not upset. In severe cases, the process involved bulbar and respiratory muscles and develop changes on the part of the heart muscle that are logged when electrocardiographic examination. During the attack there is a series of vegetative disorders: flushing or pallor of the face, rash, drowsiness, a sense of heat, cooling of the extremities, thirst. At the end of the attack, a large amount of urine with low specific gravity. At the time of the attack marked a significant decrease in the content of potassium in the blood serum and the high sugar content. The frequency of attacks range from multiple his whole life to the weekly, even daily. Attacks provoked a hearty welcome, especially before bedtime, fatty and sweet food, can occur at rest that followed the great physical stress or anxiety. The disease begins in the second decade of life, old age attacks become more rare.
In some cases, the attacks of mioplegii not accompanied by lowering and raising the level of potassium in the blood serum. On this basis, Gamstop (I. Gamstorp) allocated gipercalziemicescoy form of mioplegii called "hereditary episodic adinamii". This form of bouts of immobility last a short time, are developing more frequently during the day and provoked hunger. Beginning of the disease falls on the first decade of life.
The genetics of mioplegii well studied in large families (Fig. 1-3), where the disease is correct autosomal dominant pattern in a number of generations. Sometimes occur omissions generation, which gave grounds for some authors suggest in some cases recessive course of inheritance. Men suffer more often than women, and the disease they suffer severely. We must assume that some sporadic cases of mioplegii applies to new mutations.
Diagnosis of mioplegii in family cases does not represent any difficulties. In sporadic cases, you must be aware of attacks that can develop chronic pyelonephritis, when there is a loss of potassium. Periodic bouts adinamii can appear and in cases of tumors or adrenal hyperplasia. Close to this condition occur in the treatment of diabetes with insulin overdose of adrenocorticotropic hormone (ACTH) in the treatment of a disease Itsenko - Kushinga, etc.
The prognosis of the disease in respect of life favorable, but full recovery does not occur.
In the treatment of mioplegii significant place should be given to diet and sleep. The abundance of food with high content of carbohydrates, sedentary lifestyle, prolonged sleep can provoke an attack of mioplegii. In the interictal period applies adenosinetriphosphoric acid 1 ml, 30 to 40 injections per course. It is recommended that a diet rich in potassium salts. During the attack prescribed injections prozerina (1 ml 0.05% solution), mestinon (0.06 g 3 times a day). In cases of hypokalemia is given 10% solution of potassium chloride 1 tbsp. spoon every 2 hours, giperkaliemii when using 10% solution of calcium chloride 10 ml intravenously, as well as diet, poor potassium.
Cm. also Hereditary diseases.

Fig. 1. Family with hereditary myoplegia. (By B. N. Mankovsky.)
Fig. 2. Family with hereditary myoplegia. (Schmidt.)
Fig. 3. Family with hereditary myoplegia. (Brisset.)