Primary Hypokalemic Periodic Paralysis (also known as primary periodic paralysis or PPHP) is a rare genetic disorder characterized by episodes of muscle weakness or paralysis, typically accompanied by low levels of potassium in the blood (hypokalemia).
The condition typically begins in childhood or adolescence, but the severity and frequency of episodes can vary widely among affected individuals. The muscle weakness or paralysis tends to occur suddenly and may last for hours or even days. During these episodes, the affected individual may experience difficulty moving or performing voluntary muscle contractions, such as grasping objects or walking. The weakness or paralysis often affects the muscles in the arms, legs, trunk, and rarely the muscles involved in respiration.
Primary Hypokalemic Periodic Paralysis is caused by mutations in specific genes that control the movement of potassium in muscles. These mutations disrupt the normal balance of potassium ions inside and outside muscle cells, leading to an increase in muscle excitability and subsequent weakness or paralysis. Episodes can be triggered by various factors, including stress, certain medications, high-carbohydrate or high-salt meals, or rest after exercise.
Treatment for Primary Hypokalemic Periodic Paralysis focuses on preventing or managing episodes of weakness or paralysis. This may involve avoiding known triggers, adopting a low-carbohydrate and low-sodium diet, and taking certain medications that help maintain potassium levels within a normal range. With appropriate management, most individuals with the condition can lead normal, healthy lives between episodes of weakness or paralysis. However, it is important to note that the condition is lifelong and requires lifelong management.
