What really is a dystonic storm?

August 19, 2016

Edited excerpt from the book: Diagnosis Dystonia: Navigating the JourneySpecial thanks to contributing author, Ruth Curtis, for research and personal accounts from her experience with dystonic storms.

This should actually be titled ‘dystonic storms’, plural, because it is rarely a single event in one’s life. If you have a dystonic storm, you will likely have another, then another, and then it will probably become a way of life unless you are able to work with your doctor to reduce their frequency. The good news is that it is fairly rare for someone with dystonia to have real dystonic storms, according to the available literature.

It may come as a surprise to learn that dystonic storms are rare because we hear the term pretty regularly. Unfortunately, this term is often used loosely and incorrectly, usually to describe increased symptoms (e.g., having a more symptomatic day than the day before, increased pain and other symptoms after an activity, increased shaking of the head or tremors in the hand, or more pronounced awkward postures throughout the day). Real dystonic storms are an entirely different experience, almost a different world, than the usual ups and downs of living with dystonia. Understanding storms is critical for the individual having them, the people close to them, and first responders and emergency room medical personnel so they administer proper care.

So what is a true dystonic storm?
A true dystonic storm, or status dystonicus, is a rare, potentially life-threatening complication of severe generalized dystonia. It is characterized by relentless, sustained, severe dystonic muscle contractions that may require emergency medical attention (1). Most cases of severe dystonic storms occur in persons who have generalized dystonia that is complicated by other conditions such as metabolic disease, secondary effects of a traumatic injury, or additional neurological conditions (2-4).

During an attack, people do not lose consciousness and are aware of their surroundings, but they may not be able to communicate, as the muscles of the face and larynx are often involved. If breathing or swallowing is affected by a storm, the person may require emergency medical attention. In very severe cases, individuals may be sedated with medication or need temporary mechanical ventilation to support breathing. In very rare cases, when drugs have not worked, deep brain stimulation has been successful for some in reversing dystonic storms (4).

According to the Dystonia Medical Research Foundation (DMRF) [many other sources share similar information], rarely patients with dystonic symptoms develop increasingly frequent and intense episodes of severe generalized dystonia called dystonic storms or status dystonicus. They continue to explain that through anecdotal accounts (as it appears the amount of scientific data in this area is lacking) relatively mild to moderate dystonic storms are a fairly regular occurrence for some individuals with generalized dystonia, especially secondary dystonias. These lesser storms often do not require emergency attention (4).

Many people with genetic forms of dystonia and those with generalized dystonia have periodic to frequent non-emergent dystonic storms as a way of life. Those who have dystonic storms on a regular basis may not consider it an emergency unless they are having issues with their airway. If in pain, they typically have pain medication for just that purpose and take it if they can. Many times they just ride out the storm with whatever inner strength they have and then go on with their day if possible. Unfortunately, because their storms are often significantly painful and exhausting, it is difficult to carry on with their day as planned.

According to the above information, dystonic storms clearly differ from increased symptoms of dystonia. One of the main differences is that many dystonic storms resemble tonic/clonic (grand mal) seizures, but are not actually seizures. The patient is awake and aware of everything their body is doing, without their permission, in a rush of neural seizure-like energy. Most of the time it involves the entire body, including muscles that are not usually dystonic at baseline.

Dystonic storms, as mentioned, are also not common among the majority of people with dystonia. They are primarily associated with people who have generalized dystonia or episodes of generalized dystonia, and for these people they can be a common occurrence. However, many people with other forms of dystonia use this term, mainly when their symptoms become aggravated from their baseline.

Patients also use the term “storm” to describe an episode where they have symptoms of increased anxiety and more muscle tension than usual, often brought on in uncomfortable environments. This is not a dystonic storm. These episodes more closely resemble a panic or anxiety attack, or symptoms that get worse from stress triggers. Most of us with dystonia tend to experience an exacerbation of symptoms in these scenarios, and if you live with true dystonic storms as a part your life, stress is a major trigger.

It is important that we use “storms” in the proper context because to most doctors, a dystonic storm conveys a serious medical emergency. We don’t want to overstate our symptoms and then be discounted as hypochondriacs; nor do we want to receive treatments in excess of what we actually need. By the same token, it is important for us to tell our doctors about increased episodes of symptoms that are not consistent with our regular daily symptoms for which we are primarily treated. Other treatments or coping skills may be available to us to help ward off such episodes.

Describing dystonia is difficult, let alone all of the nuances within dystonia. If we want others to listen and understand what we are experiencing, we need to use consistent terminology to describe our various symptoms and experiences. “Storms” is but one example. Work also needs to be done to clarify the meanings of words such as “squeezing”, “pulling”, “twisting”, “turning”, and “jerking.”

This is where patients, doctors, researchers, national organizations, and patient support groups can work together to better define the terms we use and then utilize various forms of media to reach the masses. Collaboration with one another is highly beneficial for scaling all our challenges. We need to continue advocating so we can reach a point where doctors do not need to Google “dystonia” during an office visit!

References
1) Singer, H. Mink,J. Gilbert, D, Jankovic, J. (2010) Movement Disorders in Childhood, Philadelphia, PA: Elsevier, Inc.

2) Mishra, D. Singhal. S. Juneja. M. (2010) Status Dystonicus: A Rare Complication of Dystonia, Indian Pediatrics, 15, 883-885
3) Frucht, S.J. (2012). Movement Disorder Emergencies: Diagnosis and Treatment. (p. 127). New York, NY: Humana Press.
4) DMRF, Retrieved from: https://www.dystonia-foundation.org/what-is-dystonia/frequently-asked-questions/frequently-asked-questions-symptoms

________________________

Tom Seaman is a Certified Professional Life Coach in the area of health and wellness, and author of the book, Diagnosis Dystonia: Navigating the Journey, a comprehensive resource for anyone suffering with any life challenge. He is also a motivational speaker, chronic pain and dystonia awareness advocate, health blogger, and volunteers for the Dystonia Medical Research Foundation (DMRF) as a support group leader, for WEGO Health as a patient expert panelist, and is a member and writer for Chronic Illness Bloggers Network. To learn more about Tom’s coaching practice and get a copy of his book, visit www.tomseamancoaching.com. Follow him on Twitter @Dystoniabook1 and Instagram.

 

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6 responses to “What really is a dystonic storm?”

  1. E says:

    I am one of the few who has TRUE dystonic storms. I would never use the word “Storm” to describe a day with worse symptoms , etc. a storm is just that. It is raging, violent, relentless and destructive through out. I have gotten so bad that the past couple weeks I’m up to 2-3 storms a day. My family knows that unless I stop breathing and need CPR they are not to call for medical assistance. Two DIFFERENT occasions I have been called and treated like a drug addict (I never used ANY in my life, and at 43 have had maybe 20 drinks in my entire life). Because they didn’t know what a dystonic Storm was!!! There is not very much that can be given with all the laws changing. I can only have my low dose pain meds (I would never want anything high). Baclofen which is the muscle relaxer of choice for dystonia treatment, is starting to give me swollen legs hands and feet as well as fat and edema!! I am allergic to flexeril the other common muscle relaxant. I used to get clonazepam for my panic attacks (caused by the frontal lobe due to neurological disease) and dystonia prevention/control. But I’m from Oregon and with all the law changes one was taken from me. Since clonazepam cant treat the pain but the pain med CAN relax the muscles (kinda), that is the medication I am allowed.
    Generalized dystonia with dystonic storms (true) are a horrific way to live life. They are also progressive. The longer you have them the stronger, longer and more painful they are!

    Picture your head bent all the way backwards until it is laying on your back, your back bent in an almost U shape , limbs, face, neck, chest muscles etc all doing what they want at the same time pulling, twisting, snapping you in many different angles and directions….. dislocating shoulders, feeling like your spine is breaking, jaw feeling like it’s broken, unable to see because of the dystonia in the eyes …… that is a small picture of a TRUE dystonic Storm.

  2. MARJORIE OROURKE says:

    thank you Tom for this info. I understand what a true storm is now. Anxiety causes so much stress in my life. I have a new counselor and I loaned her my copy of your Dystonia book. love mommarge.

  3. Leslie Theunissen says:

    I can definitely answer this one. Mine have landed me in the hospital for several days. I get severe twisting with circulation problems causing severe burning and pain. I have jerking and tremors. I have seizures so bad that I do become unconscious. Heart rate and blood pressure sky rocket plus major swallowing issues at that time. It takes me about a week or 2 to get my strength up. It’s a nightmare! If you ever have a true dystonia storm you will definitely know it.

    • Tom Seaman says:

      Leslie- Thank you very much for sharing your experience. I am so sorry you have to endure such intense storms, but I know that you explaining what you go through will help others reading this. Thank you!!

  4. Patti Hayes says:

    I work 12 he night shifts. I sleep horribly, I often get severe cramping, twisting, jerking spasmatic sx in my feet, lower legs at times radiating into my thigh muscles. I am not able to walk or move my legs much when this happens. Then pain is so excruciating it brings me to tears, I writhe in the pain it is agonizing and very scary. These sx are becoming more and more frequent, I am seeing my PCP to talk about this. Could it be dystonia? Sometimes I don’t want to go to sleep as this is when the sx are the worst…

    • Tom Seaman says:

      Hi Patti- I am very sorry you are experiencing this. The cramping and pian you decribe is very familiar to me, but I do not know if it is dystonia. It is possible. I would get a referral from your PCP to see a neurologist who specializes in movement disorders so you can get a proper evaluation and hopefully diagnosis. Good luck!

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