Migraine Triggers and Comorbidities: Dystonia
Last updated: September 2023
What is Dystonia?
Dystonia is a neuromuscular disorder of the brain.
It is a disorder of the brain that affects the muscles, but is not a disease of the muscles or nerves. The dysfunctional part of the brain is called the basal ganglia. Its job is to tell voluntary muscles what to do. In Dystonia, it is thought that the part of the brain that tells the body’s muscles to relax isn’t working properly.
Dystonia is categorized as a movement disorder. Another example of a movement disorder people are often more aware of is Parkinson’s Disease. Other related disorders include akathisia and dyskinesia. The physicians best trained to diagnose and treat dystonia and related disorders, are called Movement Disorder Specialists.
What causes Dystonia?
The National Institutes of Health (NIH) describes dystonia like this:
“Dystonia is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements or abnormal postures. The movements may be painful, and some individuals with dystonia may have a tremor or other neurologic features. There are several different forms of dystonia that may affect only one muscle, groups of muscles, or muscles throughout the body. Some forms of dystonia are genetic but the cause for the majority of cases is not known.”
15 genes have been found to cause dystonia at this writing, and research continues into its cause and treatment.
Additionally, diagnoses of dystonia called Tardive Dystonia and Acute Dystonic Reactions can be caused by medicines, many of which are used in Migraine treatment and/or prevention. It's difficult to treat Migraine without using these medicines, making dystonia an important subject in which both patients and physicians should be educated. Here is a list of medicines that can cause these reactions. It is incomplete, but includes the reason the medicine causes the problem. Other medications not yet listed that have those effects, may also cause this reaction.
Dystonia may be primary (not caused by another disease or disorder) or secondary (caused by another disease or disorder including medicines).
Dystonia may appear suddenly or over a period of time. It may be temporary or permanent. It is not particular who it attacks, and patients range in age from newborn to old age.
The level of disability caused by dystonia ranges from slight stiffness in a single muscle which may come and go, to constant generalized body-wide spasms and complete disability. Spasms can be mild, or severe enough to tear tendons and ligaments and fracture bones. Level of pain associated with dystonia symptoms ranges from none at all to extremely severe. In severe cases, dystonia can affect the muscles necessary for breathing, requiring immediate emergency attention.
Dystonia is not well recognized, even among neurologists. Although it is one of the most common movement disorders, it is thought to take an average of approximately 15 physicians and 5 years for appropriate diagnosis.
Dystonia and Migraine
Many with dystonia find their spasms are a significant trigger for their Migraines. Headache specialists in the know are finding many un-diagnosed cases of dystonia in the Migraine population, making the condition important to look for and rule out. Headaches caused by dystonia (not migraines with this trigger) have their own special diagnosis in the International Classification of Headache Disorders (ICHD).
One of the most common types of dystonia found in Migraineurs is cervical dystonia, also called torticollis. Torticollis can be primary or secondary. This type of dystonia can involve one or more of the muscles of the cervical spine, neck and upper body including shoulders and upper chest.
One type of genetic dystonia called Dopa Responsive Dystonia often affects children and is frequently misdiagnosed as cerebral palsy which can be profound. When properly diagnosed, these children are given medicine (Sinemet aka carbidopa/levadopa) which can almost eliminate their symptoms, changing their lives forever.
Secondary dystonia is treated first by treating the primary condition causing the dystonia symptoms. If results are insufficient, the symptoms are treated similar to primary dystonia.
Primary dystonia which is localized to small areas, is often treated with onabotulinumtoxinA (Botox). The injection of toxin paralyzes the muscles that are in spasm. The release of spasm results in a reduction of or end to the patient’s associated pain. In those patients for whom Botox is insufficient, or for those patients who have become immune to the toxin, there are other similar toxins such as Myobloc (onabotulinumtoxinB) that can be utilized. These injections are only useful when a small area is affected with spasms however.
Other systemic medicines such as anti-spasmodics can be used with some success in patients. Some find the side effects difficult to live with. These patients will sometimes find relief with a pump that delivers medicine directly into their CSF (cerebral spinal fluid). For those that are refractory to these treatments or cannot utilize them, DBS (deep brain stimulation) may be an option. DBS involves the implantation of neurostimulators deep inside the brain. Wire leads run down the neck to batteries located usually below the collar bone in the chest.
Some patients agree to have surgery to *release* muscles that are in spasm. In this procedure, muscles (and nerves) are permanently cut to relieve the symptoms. This surgery is only useful to a small subset of patients, often those with cervical dystonia.
Dystonia in the 2011 Scottsdale Headache Symposium
The 2011 Scottsdale Headache Symposium this past November specifically addressed dystonia in Migraine patients, particularly the identification of patients needing further evaluation and possible treatment for their comorbid condition. As a dystonia patient who has worked for years toward awareness, I was thrilled to be there to see the presentation Head and Neck Examination of a Migraineur bring focus to a problem the presenter, Dr Jack D Schim, MD stressed was being missed too often.
The importance of diagnosis
Fortunately the use of injectable toxins such as Botox for chronic Migraine may help some dystonia patients who remain undiagnosed, however appropriate diagnosis of this comorbid condition would assure the patient receives appropriate treatment which may include additional or different specific injections for their dystonia, or other adjunct treatments such as those mentioned above. Should Botox be ineffective for their spasms, other toxins are approved and usually then covered by insurance.
One patient’s experience with dystonia and Migraine
I have diagnoses of dystonia (genetic), tardive dystonia (the result of medication that was given to me during surgery and to treat my Migraines) and acute dystonic reactions. I had generalized (body wide) storms for about a year which went undiagnosed. When I was taken to the emergency room after a long night doing an excruciating back-bend in my living room, the doctor didn’t recognize what was happening and it was assumed I was drug seeking. Months later, and after surgery, I had an acute reaction in the hospital where many doctors and nurses rushed to see what was happening to me. Despite my dramatic and lingering back-bend on the bed, none could diagnose me until a neurologist was consulted a day later, telling me that most physicians can’t recognize it when they see it, even in a teaching hospital. I did not believe my diagnosis and suffered an additional year before seeing the neurologist again after an EEG.
I was lucky. I don’t know how many doctors saw me before my diagnosis, but it only took about 2 years which is much less than many of my dystonia friends. I have several ruptured discs as a result of my storms, but I am now well controlled and rarely have problems that interrupt my life. My biggest issues are related to tardive and acute reactions as they severely limit the medications I can take for my frequent Migraine attacks. This diagnosis also is a red flag for ER and other physicians who often ignore me or consider me a drug seeker and deny treatment or try to trick me into taking medications that will further hurt my already damaged brain.
My dystonia is a trigger for my Migraine attacks. I hope soon to see if Botox may be a solution to my chronic Migraines as well as my cervical, facial and shoulder spasms.
In the past year, has insurance made it difficult to get your migraine treatment?