Periodic family paralysis

Periodic family paralysis (paroxysmal family myoplegia) is a hereditary disease characterized by sudden onset of temporary attacks of flaccid paralysis of the limbs.

Etiology, pathogenesis. The cause of the disease is unknown. At the heart of pathogenesis lies the transient violation of the potassium level in the blood.

Symptoms, course. The first attacks of muscle weakness appear in childhood or young age. In most cases, the attack begins in the early morning. Muscle paralysis usually reaches a maximum within an hour, symmetrically captures the limbs (predominantly the proximal parts); Sometimes the weakness of only the lower extremities is observed. Bulbar, oculomotor and respiratory muscles are usually not involved in the process. Paralysis sluggish, with hypotension and areflexia. Sensitivity persists. Consciousness is not violated. Typically a significant decrease in the level of potassium in the blood. Paralysis lasts from a few minutes to several hours. In the interictal period, those suffering from this paralysis are practically healthy. Thus, the disease can be considered benign. Only in very rare cases, the involvement of respiratory muscles in the process can lead to death.

Differential diagnosis with other forms of acutely developing paralysis (spinal stroke, polyneuropathy) is based on the frequency of seizures of paroxysmal myoplegia and strict selectivity of flaccid paralysis (without other symptoms of nervous system damage). Finally, the diagnosis revealed sharp hypokalemia at the height of the attack. In some cases, periodic paralysis is observed with a normal or even increased amount of potassium in the urine (kidney failure in particular).

Treatment. During an attack, 5-10 g of potassium chloride are prescribed. When respiratory insufficiency-in / in the introduction of potassium. With the preventive purpose, potassium chloride is shown several times a day inside, as well as the systematic intake of diacarb.