ET is ET is ET…or is it? Don’t Confuse Patients

”What’s in a name? That which we call a rose by any other name would smell as sweet…” – Shakespeare

Once upon a time, someone came up with a name for uncontrollable tremors that could not be attributed to any known cause like Parkinson’s disease, dystonia, or drug withdrawal. Such tremors were most often observed when the affected person was trying to do something like reach for a glass of water or button a shirt—but the tremors tended not to be there when the body part was at rest. Since the involuntary shaking wasn’t associated with another condition, it was assumed they had their own “essence.” And, since they didn’t seem to be life threatening, they were considered “benign” (that is, not harmful to life). Thus, the name Benign Essential Tremor was coined and lasted for many decades.

More recently, it has been recognized that these movements are anything but benign. They can be severely disabling and inflict extreme harm on a person’s quality of life, self-esteem, emotional wellbeing. Added to that, the tremor state can progress and eventually involve more parts of the body, make balance and movement difficult, and is linked with cognitive decline. The word “benign” was dropped, and now the clinical term is simply Essential Tremor (ET).

 

A new term for ET that’s hard to classify

Even though ET is recognized as the most common movement disorder, it can be challenging to diagnose because there are many different tremor types that can resemble one another. It has even been suggested that when a person with tremors first seeks medical help, the clinician should not be too quick to make a diagnosis. Instead, it could take up to three years to see if other symptoms show up that suggest an underlying neurological or other condition.

One system of classifying tremors was developed by an international consensus panel in 1998, but within two decades it became apparent that it was inadequate to cover all the observed types, variations, and personal or family histories that occurred. Therefore, from roughly 2013-16 global experts, in dialogue with each other, conducted research and worked on developing a more comprehensive yet flexible characterization model along two axes: Axis 1 is clinical features and Axis 2 is etiology (causes).[i]

In the process, they identified “soft” tremor signs that had been lumped into the 1998 classification of ET, but which this later team felt did not meet ET criteria, so they proposed the term ET plus :

Some studies have included patients with neurological signs of uncertain relationship to tremor (i.e., “soft neurological signs”), such as mild memory impairment, impaired tandem gait, and subtle body posturing that could be dystonic. There is no consensus on which of these additional signs are acceptable within the definition of ET. In clinical practice, the interpretation of soft signs is subjective and left to the investigator. We propose to clearly label these cases with soft signs as ET plus.[ii]

 

A rose by a different name?

ET expert Dr. Elan Louis takes issue with the ET plus name, calling it “problematic terminology.” In a Feb. 2020 article, he points out that retaining a narrow definition/description of ET while sidelining a mixed bag of added but uncertainly defined neurological signs that may or may not be seen in ET cases will “greatly complicate or make impossible studies of the descriptive epidemiology of ET, association studies, cohort studies, and clinical trials in ET.”[iii] As I see it, he’s implying that calling ET by any other name will still have the same impact on patients’ lives.

My own concern is that the term ET plus will confuse patients, and in some ways make them question or second-guess their own experience. When I visit online ET peer support discussion forums such as our own Essential Tremor Awareness & Support facebook group, I see that those who participate and compare their symptoms and experiences play a huge part in validating and affirming each other. Many report that they feel like their doctors somehow aren’t listening or lack compassion and understanding of their problems living with ET. They aren’t looking to each other for a name for what’s happening to them, but rather to be heard and believed without judgment. They are open to suggestions from each other, perhaps even more than advice from their doctors. I wonder if it would be perceived as rather demeaning or insulting to be told by a doctor that when they feel, say, internal trembling while lying in bed at night, it’s a “soft sign”—as if it’s not truly a part of ET because the doctor can’t find it listed that way in a diagnostic manual? Would a vague ET plus category raise needless doubt in their mind?

I don’t know the answer. I do wonder if the international experts who worked long and hard to clarify diagnostic criteria also consulted with patients for their experience. Our patients who come to Sperling Neurosurgery Associates for our Neuravive® MRI-guided Focused Ultrasound treatment know they have ET and they hope to regain control of their lives. No matter what you call ET, hope is hope is hope. It’s just that simple.

NOTE: This content is solely for purposes of information and does not substitute for diagnostic or medical advice. Talk to your doctor if you have health concerns or questions of a personal medical nature.

[i] Bhatia KP, Bain P, Bajaj N, Elble RJ et al. Consensus Statement on the classification of tremors. from the task force on tremor of the International Parkinson and Movement Disorder Society. Mov Disord. 2018 Jan; 33(1):75-87.
[ii] Ibid.
[iii] Louis ED. “Essential Tremor Plus”: A Problematic Concept: Implications for Clinical and Epidemiological Studies of Essential Tremor. Neuroepidemiology. 2020 Feb 5:1-5.

 

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