Normo-, Hypo-, and Hyperkalemic (KPP) paralysis
What is it?
It is a heterogeneous set of muscle disorders characterized by episodes of weakness (up to paralysis) lasting from several minutes to several hours, which occur sporadically and irregularly. In some cases, these disorders can be associated with episodes of muscle stiffness, sometimes up to real myotonia (inability to relax the muscle for a few seconds after its contraction). The muscles of the limbs are usually affected. The disorder usually begins in childhood or adolescence and tends to decrease with age (after age 40).
Which are the symptoms?
In most cases, these are hereditary conditions resulting from mutations of ion channels, so much so that we are now talking about cannelopathies. Hereditary forms are usually transmitted in an autosomal dominant manner, so the probability of inheriting the same disease as the parent is 50% for each child. In a fair percentage of cases these forms are associated with altered values of potassium in the blood (hyperkalemic or hypokalemic forms), the altered values of which are however found more frequently only during attacks. The diagnosis is essentially based on the clinical manifestations, to which genetic analysis can be associated, with the search for mutations in the genes involved.
Among the forms considered in this grouping are:
- periodic hypokalemic paralysis
- normokalemic periodic paralysis
- hyperkalemic periodic paralysis
- Andersen-Tawil Syndrome (ATS)
How is it diagnosed?
Suggested exams
How is it treated?
The therapy is aimed at relieving the symptoms of acute attacks and trying to prevent new ones, and includes both pharmacological compounds and changes in eating and lifestyle habits. The interventions can be specific for each type of periodic paralysis.
Where do we treat it?
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