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Primary Orthostatic Tremor

What is Primary Orthostatic Tremor?

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.

 

Synonyms

  • Orthostatic Tremor
  • Shaky Leg Syndrome (colloquial term, sometimes confused with Restless Leg Syndrome)

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.

Acknowledgement of Primary Orthostatic Tremor has not been added yet.

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.

Diagnostic tests of Primary Orthostatic Tremor has not been added yet

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.

Tips or Suggestions of Primary Orthostatic Tremor has not been added yet.
  1. Benito-León, J., & Domingo-Santos, Á. 2016. “Orthostatic Tremor: An Update on a Rare Entity.“ Tremor and other hyperkinetic movements (New York, N.Y.), 6, 411. https://doi.org/10.7916/D81N81BT.

  2. National Organization for Rare Disorders (NORD):  Primary orthostatic tremor.

  3. Orphanet:  Primary orthostatic tremor.

  4. Cleveland Clinic: Essential tremor

Is Anyone Still Active In This Group? Created by wendygsd
Last updated 20 Feb 2017, 01:08 AM

Posted by wendygsd
20 Feb 2017, 01:08 AM

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.

Posted by hollang
19 Feb 2017, 04:44 AM

It's not very active. Believe the most activity is over at https://www.orthostatictremor.org .

Posted by wendygsd
18 Feb 2017, 04:49 AM

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!

Driver's License Created by sharonmathews
Last updated 23 Jul 2010, 02:59 AM

Posted by sharonmathews
23 Jul 2010, 02:59 AM

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.

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I have had tremors since I was in my late 30's when under stress. For many years, I was diagnosed with Essential Tremors, as my mother had them. As my tremors progressed, I had a lot of foot and...
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male coc. 72 yrs old. have been dealing wth ot for 8 yrs,retired,usnavy vet 10 yrs,trade was heavey eqpt mechanic,was in the seabees wintered over at antartica 1961/62 deepfreeze at byrd station.i...

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Is Anyone Still Active In This Group?

Created by wendygsd | Last updated 20 Feb 2017, 01:08 AM

Driver's License

Created by sharonmathews | Last updated 23 Jul 2010, 02:59 AM


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