Friday, August 19, 2016

What really is a dystonic storm?

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, PhiladelphiaPA: 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 YorkNY: 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

25 comments:

  1. Well described. I think the term is loosely used and certainly needs to be differentiated from a panic or anxiety attack.

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    1. Thank you. It's a tricky thing to understand so I hope this helps clarify it a little.

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    2. Right up until our daughter passed away. Her myoglobin numbers (how hard her muscle had worked ) would be the same as if she had ran 3 marathons a day...

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    3. Oh my goodness! That is the first I had heard of such a thing happening. I am so sorry for your loss.

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  2. Weatherin the storm may be tough at times!
    But the storms settle down at times.
    And Dysonai will never define me!
    Thank God for my wonderful neuoro team of Dr. David Sommer!
    Sharon Stone Mazurek

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    1. It sounds like you have a great neuro team! How do you help control your storms? (I removed your other post below since it was a duplicate).

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    2. It is not always easy Tom!
      I take one day at a time.
      And I had DBS in 2000. Just after it passed clinical trials
      I have a great support group of family and friemds

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    3. One day at a time is great advice! I have a great support system also. It is priceless!!

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  3. Wow! Such great info thank you so much. I seem to have learned more from this site than in 15years and 10neuros. I believe I am in the middle of a storm now. A new med was introduced and all doody broke loose. Resting tremors out of control, head movement out of control especially when tired and or stress. Eating and swallowing difficult already now more so. Have lost 15lbs in a month. Pain has gone up to solid 7-8all the time. I do take meds I am getting injection as well but I needed to be down and resting my body didn't get the message so I don't think the last one worked. I am having visual changes as west and my eyeglasses are a year old. The PA. Wants me to just wait and see on the meds. But I know my body. The Dystonia is MUCH worse. The comments about being like waking seizures? Yes!! I have seizures and mine are mellow compared to the violent shaking that dislocates my ribs. I am glad I am not alone or crazy. There is an answer. This time I am talking with my Dr. I can't keep this up. Thank you for spreading the message.

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    1. Thank you! That is very humbling to hear! If you don't have one, I encourage you to get a copy of my book, Diagnosis Dystonia: Navigating the Journey. You can find it at www.diagnosisdystonia.com. That is where the lion's share of info is on this topic and much more dystonia related info.

      Although there are no meds for dystonia as you know, was the med introduced for dystonia or something else? Some meds can cause Dystonic storms and an increase in symptoms. I think it is important we listen to our bodies and sometimes override what the doc or PA wants us to do, unless they can provide evidence that it will help and the pattern of our symptoms is consistent with experience with other patients. BTW... you are not crazy and not alone. There are countless people in your corner who are here for you. Just reach out and we will do all we can. Perhaps join some online (or local) support groups if you haven't already. They can be of great help. Thanks again!

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  4. Wow! Such great info thank you so much. I seem to have learned more from this site than in 15years and 10neuros. I believe I am in the middle of a storm now. A new med was introduced and all doody broke loose. Resting tremors out of control, head movement out of control especially when tired and or stress. Eating and swallowing difficult already now more so. Have lost 15lbs in a month. Pain has gone up to solid 7-8all the time. I do take meds I am getting injection as well but I needed to be down and resting my body didn't get the message so I don't think the last one worked. I am having visual changes as west and my eyeglasses are a year old. The PA. Wants me to just wait and see on the meds. But I know my body. The Dystonia is MUCH worse. The comments about being like waking seizures? Yes!! I have seizures and mine are mellow compared to the violent shaking that dislocates my ribs. I am glad I am not alone or crazy. There is an answer. This time I am talking with my Dr. I can't keep this up. Thank you for spreading the message.

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    1. You are not going crazyTom!
      The pain iss intense for me a lot!
      Even after DBS. We are all in this together!
      If you have another E-mail I could communicate better with you!
      Please realize I am thinking about you and trying to send positive enregy your wat!

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  5. Any other info you have would be great. Thank you.

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    1. Hi Nina- There is a list of posts on the right hand side, but the majority of information is in my book, Diagnosis Dystonia: Navigating the Journey. You can find it at www.diagnosisdystonia.com.

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  6. Right now Tom My Dystonia is really challenging me a lot!
    I am in constant fear of turning my neck the wrong way because of my spinal column!
    My left eye is closed and I just cut my thumb on a knive!
    My neighbor fixed that for me, but the navigation tonight is like a GPS going the wrong way!
    And storms are moving into this area and that type of "Weather related storm' always makes my
    dystonia worse
    Once I can get into a comfortable position I can manage the "Dystonic Storm" easier
    I did not want to tell you about the parents who lost a son!
    DBS only works on a young person once the brain is fully developed.
    I saw a young man at a conference in Boston before and after his surgery and he is doing really well!

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    1. I am very sorry Sharon. Sometimes fear can make us tense up even more which can exacerbate our symptoms. Have you read my book? I have a section in one of the chapters that talks all about this and some of the new info that trauma experts have researched regarding fear, pain, fear response in the tissues of the body. Very interesting and very applicable to dystonia. I hope you are able to keep your symptoms to a minimum and get comfortable.

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  7. I would like to get the book, but I cant afford it right now! My library must have it.
    I probaly should see a trauma expert or go to a pain clinic
    "This too shall Pass!'

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    1. Unfortunately most libraries do not carry it, but you can request for them to get a copy.
      I like your attitude!

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  8. I would like to get the book, but I cant afford it right now! My library must have it.
    I probaly should see a trauma expert or go to a pain clinic
    "This too shall Pass!'

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  9. Well done. Appreciating the work that went into this.

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    1. Thanks very much! I really appreciate that!

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  10. This is a great article that explains dystonic storms very well. I have had dystonic storms a couple of times a year, ever since I was a young boy.

    When my parents would take me to see the doctor they would normally test me for hypoglycemia and send me on my way.

    I saw many doctors throughout my life and was eventually diagnosed with Dystoina at the age of 49.

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    1. Thank you very much! That is the first I have heard of someone being tested for hypoglycemia after a storm. I hope you have been able to manage your dystonia symptoms and storms. I can't imagine going that long without a diagnosis. Thanks again for your comment!

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  11. Great article! Thank you for sharing it with us.

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