Edited excerpt from the book: Diagnosis Dystonia: Navigating the Journey. Special 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
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
4) DMRF, Retrieved from: https://www.dystonia-foundation.org/what-is-dystonia/frequently-asked-questions/frequently-asked-questions-symptoms
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
