Dennis J. Bonner, MD

Paroxysmal Pain Disorder

Paroxysmal Pain Disorder

Paroxysmal Pain Disorder

 
 

Paroxysmal pain disorder is a rare condition in which you start to experience intense flushing and skin redness along with attacks of pain. This pain is experienced in different parts of the body. Paroxysmal pain usually lasts around a few seconds or minutes, but some people may experience it for a longer time, and that’s something to worry about. Why? Because it’s not easy going through it. This disorder features major pain attacks, and in turn, it starts to affect the entire body.

The pain may start from one area, but it generally travels to different parts of the body, increasing the intensity of the pain. There are many things that could trigger Paroxysmal pain, but most of it has had to do with genetics and day-today lifestyle behaviors. According to a report carried out by the National Library of Medicine this disorder apparently only affects 80 individuals

This type of pain is said to derive from the peripheral nervous system as it connected to the brain and spinal cords to your muscles and cells. These cells usually detect diffenrt sensations, touch , pain and smell in your body. This seems like quite a worrisome disorder, and if you feel like you’re prone to having it or need to learn more about it, then read further below. 

What is Paroxysmal Pain Disorder

Though you got a small glimpse and understanding of what Paroxysmal pain disorder is, lets get a bit further to really understand the nature and cause of this painful disorder. 

Paroxysmal pain disorder derives from the peripheral neuropathy system accompanied with flushing, intense pain attacks on different areas of the body, and skin redness. These are the three major symptoms associated with Paroxysmal pain and can get a bit tough to handle. There are many things that ignite this type of pain.

Usually, the pain starts in the lower body and this usually happens much earlier in life if you are prone to such a disorder. The pain starts to appear when it’s caused due to a bowel movement. As you age, the location of the paroxysmal pain starts to change, and mainly begins to affect your face and head.

Other triggers include emotional distress, sudden changes in the temperature, consumption of cold beverages and eating spicy foods regularly. While these factors may trigger the pain, the major reason for having it is because of the SCN9A gene. When mutations start to exist in this gene, you tend to experience paroxysmal pain. Paroxysmal pain disorder is very frequent in younger patients and tends to remain a lifelong sickness. The diagnosis is made under very efficient measures using clinical history and gene testing. 

History of Paroxysmal Pain Disorder

The Paroxysmal pain disorder was first discovered and described by medical researchers in 1959. This happened when the doctors noted intense pain in the jaw, eye, and rectum of their subjects. In 1972, DR R.E Dugan carried out his own research on the disease and was initially named as the “familial rectal pain syndrome”. 

For 33 years this disorder was named as the familial rectal pain syndrome in different journal and medical practices. In 2005, clinicians renamed the disorder after they realized the name doesn’t take into account the symptoms experienced in this disorder. 

Symptoms of Paroxysmal Pain Disorder

As mentioned earlier, there are a few main symptoms linked with Paroxysmal pain disorder and these symptoms can at times get out of hand, putting the patient in a very uncomfortable position. One of the main symptoms of Paroxysmal pain disorder is burning episodic pain in the ocular, rectum, and mandibular regions. The pain usually starts to appear in the center of these areas, and gradually tends to spread to other parts of the body.

This type of burning pain is unbearable, and some have described it worse than the pain at childbirth. Along with the pain, patients also experience intense flushing, which starts making you feel extremely warm. The symptoms tend to appear differently on each patient. When infants experience the paroxysmal pain attacks they often react with non-stop crying and screaming caused by injections, consumption of certain foods and oral medication, and wiping the prenum. This then results in flushing and intense pain in the area of the pain. 

Symptoms such as hyper salivation are also common, especially when the attacks exist in the mandibular region. Leg weakness is also another symptom which is mainly triggered due to leg trauma. Tonic non-epileptic seizures are one of the non-physical symptoms of this disorder and are very common in infants or usually experienced at an earlier age.

Sometimes in older children, the paroxysmal pain attacks are also followed by stiffness and apebna, so you can expect some inconsolable screaming, as the pain is pretty intense. As mentioned earlier, these pain attacks usually last for a few seconds or a minute. However for some, it may persist till a month. During the attacks, patients usually struggle with constipation which is another symptom of Paroxysmal pain disorder.

Constipation usually occurs due to the apprehension of the attacks. However, this symptom is not likely to fade away with age with the help of effective coping mechanisms such as stool softeners. According to the Human Phenotype Ontology database 30%-79% of people exercise constipation during paroxysmal disorder while 80%-99% of people experience seizures.

Diagnosing Paroxysmal Pain Disorder 

Diagnosing Paroxysmal pain disorder is very challenging as this is a genetic and rare disease, so it’s hard coming up with the exact diagnosis. However, to determine that you’re suffering from Paroxysmal pain disorder, your healthcare professional will take a good look at your medical history. Going through the medical history in depth will give them a slight hint if you could hold the gene for this type of disorder. Following the medical history, the doctor will also check for your symptoms and get different lab testing and physical exams to make the right diagnosis. 

One of the main testing resources is the Genetic Testing Registry (GTR) which gives you information regarding the different genetic test for this pain disorder. Other than that, when it comes to deeper investigations, there is proper study on your blood and urine between attacks. EKG recordings are taken during attacks that may usually show sinus tachycardia.

What Causes Paroxysmal Pain Disorder?

The causes behind paroxysmal pain disorder and the symptoms that come along with it have a lot to do with genes. After much research, researchers were able to pin point out the main cause of this disorder and that was a mutation in the SCN9A. The mutation in this gene is accompanied by an inability to deactivate voltage-gated sodium channel NaV1.7. 

This inactivation leads to prolonged and repetitive firing, causing an increase in pain and all activity linked to the sympathetic nervous system. When the same gene has a different mutation, it is said to react in the total opposite direction, which is lack of pain. 

Treatment of Paroxysmal Disorder

There is no particular treatment for paroxysmal pain disorder so far.  However, there are a few things that could maybe help in controlling the symptoms. Because of its genetic background, you will have to live with this disorder being able to control its intensity. The best way to receive any type of treatment for this disorder is by visiting a medical professional.

These experts will provide you detailed counseling and will not only help diagnose the disorder but also find ways on how you can control or tolerate the symptoms. You will no doubt come across many healthcare professionals, but it is best to go to someone who has experience in treating or diagnosing patients with Paroxysmal pain disorder, as this is a very rare disease and very few doctors come across it.

How is The Pain Described as?

The pain that derives from the paroxysmal pain disorder is sometimes described as worse than the pain experienced during childbirth. PEPD consists of episodic burning attacks in certain parts of the body, and within time you start to experience flushing and irregular heartbeats. The pain varies from person to person, as some experience it for a few seconds while others have to bare it for a month or more. According to a report published in the Journal of Neurology the paroxysmal pain disorder is ranked as the pain experienced during child birth and sometimes even worse than that. 

Final Words 

Paroxysmal pain disorder is relatively a very rare disease whch is why you may have not heard of it before. That said, it’s best to stay aware of the different symptoms, and before doing anything on your own, acquire immediate health consultation. Hopefully with the help of this article, you now understand what Paroxysmal pain disorder is.