ORTHOSTATIC TREMOR AWARENES
September is Primary Orthostatic Tremor (POT) AWARENESS month — an extremely rare neurological movement disorder.
The realities of living with POT:
I sit in my car and debate walking inside. Anywhere in a place of business, will they have a seat? Mobility chair? Will there be a line? What is my level of ability right now in this moment, regarding my standing capacity or slow lingering walk? I do not possess an optimum balance in a casual stride, I am aware of each step. I feel insecure and vulnerable. I move leg to leg while at a stop, this pushes against the standing leg tremors, like walking in place, a movement that helps. I lean on a counter for support or a shopping cart. I have around 10-15 minutes before I feel weak and need to sit down. Breathing in slowly and out slowly, I push against anxiety. Where can I sit? I don’t want to explain — I need a chair. I can sit, but I cannot stand. I could walk fast if my legs did not hurt with age, combined with the progression of OT stiffness and muscle pain. Slower walking permits the elevated hertz of the OT leg tremor to activate, so it is not a solution as some research indicates. Research speaks of dizziness upon standing — on the global POT support group it is spoken as a desperate need to sit down. Few mention dizziness. Fatigue comes quickly, exhaustion with an overwhelming need to sit, a feeling like melting to the floor. When at home I lay down. Recovery takes a while, after too much activity. Younger POT patients can walk fast, this pushes against the rapid hertz leg tremors. I stumble a bit, with a slower orthostatic tremor that causes awkwardness. (I attempt to explain it to any nurse or doctor. Most do not have time to understand.)
I walk into the building. Sitting in a lobby recharges my stride, or gait as they call it. If I do not have far to walk and then sit I will make it. I make it out of the building. I made it all look good. I am exhausted. A medical team has no clue if they do not lean in to learn from the patient.
My movement disability is one of the most rarest in the world. The specialized movement neurologist says — I would doubt if any regular neurologist has ever heard of POT (Primary Orthostatic Tremor)
I respond — yes, I know this to be true as — the global support group has confirmed in numbers — medical professionals have never heard of Orthostatic Tremor (OT).
They turn and look away from what they do not understand.
When I walk into a medical office, I say ‘Orthostatic Tremor’…on repeat, like they do on some of those annoying info commercials.
Because awareness matters.
It is progressive.
It is a cerebellum mishap — most research indicates.
It is under-diagnosed because it is under-researched…and under-researched…because it is under-diagnosed.
Clonazepam was prescribed at the time of my official diagnosis by Dr. Rodolfo Savica at Rochester, Minnesota Mayo Neurology. I aimed high when I realized the area of which I lived was medically clueless. I had googled my symptoms, and ask my PCP — Have you ever heard of Orthostatic Tremor? He had not, I ask him to look it up. I had been in his office with my legs shaking within 30 seconds upon a standing position, it ceases upon sitting or walking. After multiple visits exhibiting this. I ask him to give me a referral to Mayo. Knowing Mayo had orchestrated the most research on OT.
Clonazepam is sadly the first line of go-to, prescribed on my following up appointment with movement specialist of Mayo Neurology, .5mg of clonazpam twice daily as a starting dose to be increased as needed. I have never had it increased. It has served me well enough. I was told my PCP would provide this RX maintenance.
It is very difficult to successfully communicate POT on a local medical level to physicians. It is difficult to express Clonzepam as a maintenance drug for POT. The POT patient feels isolated within the lack of awareness and knowledge in the local medical fields. I was told my PCP would manage this as I needed. The first time I had this RX filled was mail-ordered in Arkansas. I received 1 week supply with no refills. I was in tears trying to explain this is a maintenance RX.
This awareness article is for MEDICAL FIELD AWARENESS.
Regardless of September being Orthostatic Tremor Awareness Month — It deserves awareness. These are quotes from leading research articles on this rare under-researched disabling neurological movement disorder of Orthothostatic Tremor:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5039949/
“Orthostatic tremor (OT) remains among the most intriguing and poorly understood of movement disorders. “
“Owing to its rarity, the current understanding of OT is limited and is mostly based on small case series or case reports.”
“Scientific understanding of OT is in its infancy.”
“OT is not widely recognized by physicians who are not movement disorders experts, which often results in misdiagnosis for the unfortunate patients, who then may be subjected to inappropriate or unnecessary tests and treatment.”
https://clinicalmovementdisorders.biomedcentral.com/articles/10.1186/s40734-016-0048-5
“Primary orthostatic tremor is a rare disorder that is still under-diagnosed or misdiagnosed”
“We review the clinical signs of POT that may help to increase the awareness of doctors and improve the diagnosis accuracy”
“Nevertheless, this condition is relatively easy to diagnose when you listen carefully to your patients, as they often report the main clinical features: lower body tremor activated upon standing (with feelings of unsteadiness and decreased time immobile in the upright position) which is improved by walking and absent when sitting or lying down”
https://www.nebraskamed.com/orthostatic-tremor-imagine-being-able-to-walk-but-not-stand
“Never heard of orthostatic tremor (OT)? Most physicians haven’t, let alone the general public.”
In collecting articles and research studies, I discovered most of them to be outdated. Many are articles of opinions of research studies. There are articles of updates and republishing. Comparatively, nothing new. Cerebellum involvement studies. Classification opinions regarding hertz values. All very interesting articles and resources, but many are simply outdated.
Mayo Neurology research has delivered the most current conclusive research of 2019;
https://tremorjournal.org/article/10.5334/tohm.513/
Clonazepam has been the first-line drug of treatment used for many years and seems to present good results for many and not so good for many. The downside; our body builds up a resistance maybe as OT progresses. Many patients seem to have an intolerance to the side effects. The Mayo Movement Disorder Neurologist was careful to tell me that treatment is very limited, with sometimes minimal results.
It seems most patients who have had OT for a long time, chose to not take medication, due to side effects, mainly drowsy with slower thought processing — brain fog. We are all different. Clonazepam, Gabapentin, and propranolol seem to be a combo with many OT patients. Primodine is the second line of treatment.
In more recent years there has been a push for DBS (Deep brain stimulation). I have observed on the support boards of those who have had DBS; that it’s invasive and expensive, and insurance does not pay for it. I have heard of successes and also not-so-successful stories with DBS procedures. I believe it probably is a last resort for the more severe cases and patients with medication-refractory OT (a term used when they are not responsive to medication.) DBS is a neurosurgical procedure that uses implanted electrodes and electrical stimulation to treat movement disorders. Maybe future improvements will come with DBS and insurance will be forced to consider the quality of life improvement.
One of the newer technologies in process of treatment
https://institutducerveau-icm.org/en/actualite/a-new-non-invasive-therapy-for-the-treatment-of-primary-orthostatic-tremor-pot-trans-spinal-electrical-stimulation/ a quote from the above article.
“A study led by Jean-Charles Lamy, member of the Mov’it team and operational manager of PANAM the platform for the exploration of movement at Paris Brain Institute has just shown, thanks to research, that trans-spinal electrical stimulation improves both the time in a standing position and reduces the amplitude as well as the frequency of tremor. This study is published in the journal “Movement Disorders”.
Below are Dr. Karen D. Sullivan’s Orthostatic Tremor videos, she is very informative and gifted in speaking directly to OT patients. She is a strong advocate for patients with Orthostatic Tremor. Below are her YouTube videos that have helped so many patients better understand OT. She is a doctor of Neuropsychology.
Orthostatic Webinar preview https://youtu.be/ea6yHr4ssPw
Orthostatic Tremor https://youtu.be/i58rPitK5uw
Orthostatic Tremor and Gait https://youtu.be/iU3dOqBkoHA
She is also on Facebook called, I CARE FOR YOUR BRAIN
Below is a fairly current (2019) research on DBS (from Mayo Clinical Research)
https://cp.neurology.org/content/10/4/324
I noticed one of the researchers, Dr. Bryan Klassen, the Movement Disorder Neurologist who administered my EMG (electromyographic) analysis at Mayo. The classic test of determining and confirming OT.
Mayo is an impressive American medical legend, globally. Ken Burns produced a 2-hour video of its iconic history. It can be accessed on PBS.
https://www.pbs.org/video/the-mayo-clinic-faith-hope-science-czhdtb/
This is an article By Diego Torres-Russotto, M.D., Neurologist – he leads out in the Orthostatic fight with a wealth of knowledge – he is our advocator.
There is also a series of YouTube videos to be found of OT conferences he has been presented at. (Search them out on UTube)
https://www.nebraskamed.com/orthostatic-tremor-imagine-being-able-to-walk-but-not-stand
With his opening quote-
“Never heard of orthostatic tremor (OT)? Most physicians haven’t, let alone the general public. But for the small number of adults who develop OT around midlife, it’s very real, very life-limiting, and very, very hard to explain to others.
Patients with OT can walk quite well at a brisk pace.
(Nope Diego, we need better research — some of us can not walk quite well at a brisk pace, some of us have progressed into gait problems, with muscle tightness and weakness and so our walk is slower)
But if they slow down or stand still they feel that their legs no longer hold them up! It has been compared to riding a bicycle: you’re fine when you’re riding along, wobbly when you slow down, and unable to stay upright when holding still.”
(The above word picture is true – you slow down on a bicycle and you fall over without propping.)
In this article, he mentions Gloria Nelson MacWright quoted in tribute to her, “After her diagnosis in 2000, Gloria Nelson MacWright created a website called http://www.orthostatictremor.org to see if there was anyone else out there. She began hearing from others worldwide, and now over 1,100 people have registered on the site to create the Orthostatic Tremor Community.”
Below is the site that she contributed for awareness and help.
https://www.orthostatictremor.org
I appreciate that she logged her journey for us in this article below;
https://www.orthostatictremor.org/my-experience.html
And also join in with interactive support on Gloria’s resource of helpful support;
https://www.orthostatictremor.org/phpBB3/index.php?sid=8d6b685202e07d15e323c3a001458729
This U.K. National Tremor Foundation site is a great source for aiding to inform others with simple details;
https://tremor.org.uk/orthostatic-tremor
If you go on Facebook and search; Orthostatic Tremor groups, you will find several that have very random postings, from the U.S and the U.K., I found support in them all helpful, although Australia has the most volume in traffic postings and people involved, I appreciate their support and the work they put into helping encourage and inform their members of Primary Orthostatic Tremor on Facebook.
Below is our National source for calculating rare diseases
2021 Orthostatic Tremor has been documented with 390 cases in our nationwide. (US)
https://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_alphabetical_list.pdf
Once again Mayo Clinic has completed the most current, conclusive article to date. It would be my go-to for valid information.
https://tremorjournal.org/article/10.5334/tohm.513/
OT, being a marginalized community within research — support groups are essential. We learn from one another.
Hopefully, researchers may learn from us through observations in our groups.
Research you are welcome, we invite you.
Go to the support groups and study patients with their experiences.
Observe the ages, there are many young patients with OT.
Observe that many of us who are older, tell of an inability to stand for very long when we were younger.
Observe that we have spinal issues, include that in your research.
KNOW that our medical communities do not know of or have ever heard of Orthostatic Tremor.
Being very thankful for research, I pray for clinical curiosity to be a driving force among the many researchers and all physicians please lean in toward this care of knowledge for Orthostatic Tremor.
Please help us in making Orthostatic Tremor KNOWN, so that diagnostics have a path to us.
AWARENESS and UNDERSTANDING are essential to this disability.
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