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Periodic paralysis (PP) is a rare genetic disorder. It causes sudden attacks of short-term muscle weakness, stiffness, or paralysis. These attacks may affect the whole body or just one or two limbs.
There are several different forms of PP. They all involve defects in ion channels. These are gateways that let charged minerals (ions) such as sodium and potassium flow into and out of your cells. In PP, the ion channels sometimes fail and the muscle cells don’t work properly.
PP attacks can start in childhood or adulthood. They may happen after hard exercise or other triggers. Depending on the form of PP that you have, the symptoms may be mild or severe, and they may last for minutes or days. Sometimes, the disease may slowly get worse over time and cause permanent muscle damage.
The main forms of PP are:
Most forms of PP affect the skeletal muscles. These are the muscles you control to move. Andersen-Tawil syndrome can affect the skeletal muscles and the heart muscle, making this form of PP more dangerous than most other forms.
The different forms of PP all result from genetic defects. These defects result in ion channel defects. The ion channels normally control the way that charged minerals (ions) such as potassium, sodium, and calcium enter and leave your muscle cells. This flow of ions is a central part of how your muscles work.
Each muscle cell has a covering, or membrane, that keeps the inside of the cell protected. When your nerve tells your muscle cell to contract, it sends a chemical signal that opens a gate to allow sodium ions to flow into the cell. The rush of charged ions changes the electrical charge inside the cell. This causes a wave of current through the muscle fiber. Calcium ions pour out of sacs in the cells and make the muscle fiber contract. Potassium channels open up, sending potassium ions out of the cell. This causes the muscle to contract.
In PP, the ion channels have flaws that can disrupt the process. The muscle cells then fail to contract or relax in response to the nerve signals.
There are dozens of different defects that can cause PP. These affect sodium, calcium, or potassium ion channels. There are also many unidentified PP defects. In most cases, you can get PP if just one parent has the condition (this is dominant inheritance).
Your symptoms may depend somewhat on the form of PP you have. You may first notice symptoms in childhood or in adulthood. You may have attacks often or rarely. In some forms of PP, people tend to have less attacks as they get older. Generally, people with PP may have symptoms such as:
The different forms of PP may cause the following:
In general, your symptoms may differ, depending on the form of PP and the particular gene change that you may have.
Your doctor will take a medical history, and ask about your recent symptoms, past medical problems, and family medical history. Your doctor will do a physical exam and assess your muscles. You may need tests such as:
You may first see your primary doctor but then get referred to a neurologist who specializes in diseases like PP.
Treatment aims to reduce the number and severity of attacks. Ways to manage PP may include:
You may need frequent monitoring to measure your blood levels of potassium. A combination of controlled diet, medications, and lifestyle changes may help you manage PP.
PP is a rare disorder that causes sudden, temporary attacks of muscle weakness, stiffness, or paralysis. There are several different forms of PP. These all involve gene defects in ion channels. Certain charged minerals such as sodium and potassium rapidly flow into and out of your muscle cells through these portals.
Tips to help you get the most from a visit to your healthcare provider:
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