Perinatal care in a patient with diagnosed Westphal's disease

Abstract

Hypokalemic periodic paralysis (HOKPP) belongs to the group of genetically conditioned myopathies, inherited in an autosomal dominant manner. Pathology is associated with a fall in blood potassium levels (hypokalemia). The disorder is linked to the region of chromosome 1q 31-32 in the gene encoding the dihydropyridine (DHP) receptor and the unit of the alpha-1 calcium channel. Impaired function of ion channels and sodium-potassium pump lead to secondary depolarization of cell membranes and further lack of muscle reaction to nervous stimulation. The Westphal's disease is characterized by the occurrence of quadriplegic paralysis associated with hypokalemia. In the severe form of the disease, a generalized paralysis may occur with involvement of respiratory muscles and arrhythmias. The factors causing the seizure include: sudden temperature changes, cold, long-term immobility, vigorous physical exercise, stress, viral infections. Prevention consists of avoiding risk factors and treatment based on the supply of potassium preparations and the use of mechanical lung ventilation.