Primary orthostatic tremor (POT) is a rare neurological movement disorder characterized by a rapid, high-frequency (>12 Hertz) tremor that primarily affects weight-bearing muscles of the legs and trunk. To measure the degree of shaking, each shake of the affected body part is one tremor cycle. The frequency of a tremor is how many cycles you have in one second. The international unit for cycles per second is a Hertz (abbreviated Hz). Thus, POT usually causes 12 tremor cycles per second, but this can vary from person to person. The defining feature of this condition is that the tremor occurs exclusively when standing still, and improves during walking, sitting, or leaning. It creates a profound feeling of unsteadiness and imbalance, prompting the person to immediately walk, sit down, or lean against something for relief. Because the tremor is so fast, it is often not visibly obvious to others, making it a "hidden" disability often detectable only with electrophysiologic testing. It is a relatively new condition, with symptoms discovered and established in 1984, and research is ongoing to improve understanding.
Primary orthostatic tremor (POT) is a rare neurological movement disorder characterized by a rapid, high-frequency (>12 Hertz) tremor that primarily affects weight-bearing muscles of the legs and trunk. To measure the degree of shaking, each shake of the affected body part is one tremor cycle. The frequency of a tremor is how many cycles you have in one second. The international unit for cycles per second is a Hertz (abbreviated Hz). Thus, POT usually causes 12 tremor cycles per second, but this can vary from person to person. The defining feature of this condition is that the tremor occurs exclusively when standing still, and improves during walking, sitting, or leaning. It creates a profound feeling of unsteadiness and imbalance, prompting the person to immediately walk, sit down, or lean against something for relief. Because the tremor is so fast, it is often not visibly obvious to others, making it a "hidden" disability often detectable only with electrophysiologic testing. It is a relatively new condition, with symptoms discovered and established in 1984, and research is ongoing to improve understanding.
The exact prevalence of POT is unknown, although it is estimated to affect < 1 per million individuals. It predominantly affects older adults > 50 years old, with women representing about 64% of cases.
| Name | Abbreviation |
|---|---|
| Orthostatic Tremor | OT |
| Shaky Leg Syndrome (colloquial term, sometimes confused with Restless Leg Syndrome) |
The cause of primary orthostatic tremor is idiopathic (unknown). It is considered a neurologic disorder rather than a muscle disease, involving abnormal oscillator activity in the cerebellum or brainstem. POT may be a variation of another movement disorder called essential tremor, which is inherited in an autosomal dominant pattern, but research is still ongoing. Most cases are sporadic, occurring in people with no family history of the disorder.
POT appears as shaking and lack of balance when standing up, and is associated with an intense and disabling sense of unsteadiness, fear of falling, discomfort, dizziness, shaking, trembling sensations, and/or trembling of limbs and trunk. Symptoms while standing are reduced or abolished by walking, leaning on support, sitting, and lying down. Patients often shuffle, wobble, or seek support when standing. Despite feelings of imbalance, actual falls are relatively rare. Many patients develop anticipatory anxiety in situations requiring prolonged standing, such as waiting in line or social gatherings.
POT diagnosis is based on clinical examination and surface electromyography (EMG). A history of an inability to stand still, relieved by walking is the strongest initial indicator. EMG electrical activity measurements in weight-bearing muscles showing a tremor frequency > 12 Hertz are considered to be the “gold standard” criteria for establishing a diagnosis. Because the tremor is fast and difficult to see, POT is frequently misdiagnosed. Additional testing such as brain MRI or laboratory tests are often performed to rule out other conditions.
There is no cure for POT, and treatment focuses on symptom management. Responses to treatment vary greatly among patients.
Medications: Clonazepam is considered a first-line treatment in reducing the amplitude of tremors; anticonvulsant seizure medications like Gabapentin or Primidone are also commonly prescribed.
Lifestyle and assistive devices such as use of a cane, walker, or carrying a portable folding stool can dramatically improve quality of life and provide immediate relief when out in public.
Avoidance of prolonged standing.
Physical therapy to improve balance.
For severe, refractory cases where medications fail, surgical options such as deep brain stimulation (DBS) targeting the thalamus have been used.
POT is generally a slowly progressive disorder. Over time, the duration a person can stand still may decrease and the tremor may spread. Many patients achieve partial symptom control with medication. The inability to stand can make everyday tasks and social engagements highly challenging. The condition is not fatal and does not alter life expectancy.
National Organization for Rare Disorders (NORD): Primary orthostatic tremor.
Orphanet: Primary orthostatic tremor.
There is also a Facebook Group called 'Primary Orthostatic Tremors' that has a lot of the same people in it. There is a lot of daily chatter about OT, I'm a member of both groups.... Thanks for your reply.
It's not very active. Believe the most activity is over at https://www.orthostatictremor.org .
I just got an email saying they were going to be updatng this site and wondered if it was still active. I see there has only been one post and that was in 2010!! There is lots of new info, although nothing to help solve our problems, but we need people to get together to make an awareness of OT!
Has anyone had difficulty renewing their Driver's license? I have resorted to using a walker. Recently when I went to renew my Driver's license I was asked why I needed the walker. When I was honest and told the gentleman that I had Orthostatic Tremors he typed it into the computer and told me that he would have to report this to Tallahassee, Florida. This happened in June of 2010. I was told that I would receive a Medical Report form in the mail. I have not yet received it. I found the number online for the Medical Review Board in Tallahassee and then called them. When I spoke with them they told me that they have not made a decision yet whether to send me the medical report form. They said they needed to know if the disease is progressive or not. I know that it is progressive, however, this time I kept my mouth shut. They said they would get back to me that afternoon. Which was July 20. It is now July 22 and still no word. I called again today and left my name, Driver's license number and phone number. Still no call. I did get a 30 day extention on my license on July 20th to try and resolve this matter. I have an appointment with my doctor on August 3, 2010. Hoping they can straighten out this mess if I can't do it on my own. I have a Mother with Alhezimer's Disease that I have been helping care for that lives in another town. I am worried that I will not be able to get around to help out.
CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.
Enrolling is easy.
After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.
Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.
Visit sanfordresearch.org/CoRDS to enroll.
Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.
Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.
Created by wendygsd | Last updated 20 Feb 2017, 01:08 AM
Created by sharonmathews | Last updated 23 Jul 2010, 02:59 AM
Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.
FIND YOUR COMMUNITY
Our rare disease resources include e-books and podcasts
Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.