What is potassium aggravated myotonia?

Potassium-aggravated myotonia is a disorder that affects muscles used for movement (skeletal muscles). Beginning in childhood or adolescence, people with this condition experience episodes of sustained muscle tensing (myotonia) that prevent muscles from relaxing.

What triggers myotonia?

What causes myotonia congenita? This disease is caused by mutations in the gene for a chloride channel that’s necessary for shutting off the electrical excitation that causes muscle contraction.

How does myotonia affect the body?

People with this disorder often have prolonged muscle contractions (myotonia) and are not able to relax certain muscles after use. For example, a person may have difficulty releasing their grip on a doorknob or handle. Also, affected people may have slurred speech or temporary locking of their jaw.

Does myotonia hurt?

Affected individuals may experience myotonia, abnormal stiffness, cramping, and/or pain within certain affected muscles, particularly with exercise, and abnormal muscle enlargement (hypertrophy). The disorder is transmitted as an autosomal dominant trait.

What is Paramyotonia?

Paramyotonia congenita is a disorder that affects muscles used for movement (skeletal muscles). Beginning in infancy or early childhood, people with this condition experience bouts of sustained muscle tensing (myotonia) that prevent muscles from relaxing normally.

What causes Hyperkalemic periodic paralysis?

Hyperkalemic periodic paralysis is caused by mutations in the SCN4A gene and is inherited in an autosomal dominant manner. Diagnosis is based on clinical symptoms including the increase of blood potassium level during an episode, but normal levels of blood potassium level in between episodes.

What muscles are affected by myotonia?

Although myotonia can affect any skeletal muscles, including muscles of the face and tongue, it occurs most often in the legs. Myotonia causes muscle stiffness that can interfere with movement.

What are the signs and symptoms of myotonia congenita?

Myotonia congenita is a genetic disease characterized by the inability of the skeletal muscles to quickly relax after voluntary movements. Symptoms typically begin in childhood and vary from person to person. They may include muscle stiffness, muscle weakness, and attacks of weakness brought on by movement after rest.

What does myotonia feel like?

The main symptom of myotonia congenita is stiff muscles. When you try to move after being inactive, your muscles spasm and become rigid. Your leg muscles are most likely to be affected, but the muscles of your face, hands, and other parts of your body can also get stiff. Some people have only mild stiffness.

Is muscular atrophy painful?

Muscle Atrophy: Signs, Symptoms & Treatments. Muscle atrophy refers to the loss of muscle tissue caused by a long-term lack of physical activity. Individuals with this condition experience mobility issues, pain, and discomfort, reducing their quality of life.

Is Paramyotonia congenita painful?

The severity of the muscle stiffness depends on the individual; some patients experience painful myotonia, while others experience painless myotonia. This condition becomes worse with exposure to cold and alleviated by warm temperatures. In addition, it can become more severe with exercise.

What is Hypokalemic periodic paralysis?

Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Attacks cause severe weakness or paralysis that usually lasts from hours to days.

What is potassium-aggravated myotonia?

Potassium aggravated myotonia is a group of diseases that causes tensing and stiffness ( myotonia) of skeletal muscles, which are the muscles used for movement. The three types of potassium-aggravated myotonia include myotonia fluctuans, myotonia permanens, and acetazolamide-sensitive myotonia. [1]

Why does myotonia get worse after exercise?

Myotonia causes muscle stiffness that worsens after exercise. In this disorder, episodes of myotonia may also be triggered (aggravated) by eating foods that are high in the mineral potassium, such as bananas and potatoes. During these episodes, stiffness occurs in skeletal muscles throughout the body.

Why do bananas cause myotonia?

In this disorder, episodes of myotonia may also be triggered (aggravated) by eating foods that are high in the mineral potassium, such as bananas and potatoes. During these episodes, stiffness occurs in skeletal muscles throughout the body.

What are the signs and symptoms of sodium channel myotonia congenita (Pam)?

Patients with sodium channel myotonia congenita, also known as potassium-aggravated myotonia (PAM), present with episodes of generalized stiffness secondary to myotonia. The disorder is quite potassium sensitive, with worsening of symptoms by potassium ingestion but in most patients no worsening with cold.