Professional Documents
Culture Documents
Essential
Tremor:
The
Other
ET
Sharon
Harrison
2003
©
2003
Sharon
Harrison
Abstract
This thesis provides a comprehensive understanding of the most common and perhaps
least known movement disorder called essential tremor. Written primarily for the general
researchers and movement disorder specialists in the field of neurology. The focus is
educational, in order to raise awareness and sensitivity to the challenges posed by this
criteria and the challenges to differentiate this disorder from others involving tremor.
Personal stories are provided to bring special attention to the physical and social
disabilities and their great impact on quality of life. The paper concludes with a call for
further research from physicians and patients alike, with the hopes for a greater
knowledge of essential tremor, new treatment options and perhaps, eventually, prevention
strategies.
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Table of Contents
Introduction 6
Assessment/Diagnosis 12
Causes
Brain 14
Genetics 15
Environmental 16
Disabilities 16
Physical 17
Cognitive 17
Psycho/social 18
Treatment
Pharmaceutical 18
Neurosurgical 20
Holistic/Stress Reduction 21
Personal Stories 24
Conclusion 32
Appendix A 34
Appendix B 35
Appendix C 36
3
Table of Contents
Annotated Bibliography 53
Bibliography 61
4
Figure Captions
5
Introduction
long line for my preferred mid-morning snack of hot chocolate and a crunch bar. Not
content with the sweetness in these items separately, upon reaching a table in the
beckoning quad, I will ceremoniously dip the candy bar into the hot beverage until it just
begins to melt. “Life is good,” I think to myself. This is our ritual, my best friend and I,
and we have done this since winter began. And yet today, something is different. As I
take my leave from the snack window and head to the nearest table, the hot chocolate
sloshes over the side of the cup and onto my hand and wrist. As my girlfriend chuckles,
not unkindly, the hot beverage burns my skin. My embarrassment burns hotter still. I am
15 years old.
A year later, I am about to have my ears pierced for my sixteenth birthday. Before
the clinician takes the punching appliance to my ears, she must mark the point with a red
felt-tipped pen. She tells me I have to keep my head still, and I tell her I cannot. She asks
why, and I cannot tell her that either. I laugh and say, “Just do your best.” She looks
skeptical, but I assure her that if the holes are crooked, it will be my fault - not hers.
I am applying for my first ‘real’ job as a cashier in a loan office. The manager
smiles at me and asks me to calculate a long list of numbers on an adding machine. There
are two problems here. I have never used an adding machine, and in fact, my college
preparatory classes left me woefully under-qualified for this clerical position. However,
the bigger problem is that my hands are shaking so badly that I take a very long time and
make numerous mistakes. After many starts and stops, I am red-faced but I finish the
task. I leave the interview elated – I got the job! Much later, as the manager and I become
6
good friends, he tells me a secret. “Your hands were shaking so badly, I thought you were
an amphetamine addict!” “Why did you hire me?” I shot back. “Because I thought you
present to myself to again seek answers to my questions. I am fed up with the intermittent
shaking in my hands and head and even more so by the way my previous doctors have
dismissed my symptoms with a wave of their steady hands. “It’s just stress,” they say,
“You’ve got to calm down.” As I begin to relate my story as I have done many times
new. I am mistaken. “You have a movement disorder. It is exacerbated by stress but not
It is quite likely that for most of the general population the term ET conjures up
visions of an adorable alien in a Steven Spielberg movie who moved us to laughter and
occasionally, tears. And yet, for a surprisingly large part of the population ET takes on a
very different meaning. This ET also elicits a wide range of emotions, but they are more
entertainment.
common movement disorder and yet few outside of those personally affected have ever
heard of it. A disorder that is manifested most often by tremors of the head, hands and
voice, it is often mistaken for Parkinson’s disease. In fact, ET is 20 times more common
than Parkinson’s and, in varying degrees, may affect as much as “6% of the general
population” (Louis, 2002b, p.5) and among those over age 65, “the prevalence may be as
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high as 23%” (Louis, 2002a, ¶ 1). Despite these large numbers, researchers note that as
few as 10% actually seek treatment. The reasons for this are unknown, but it is theorized
that the tremors may not yet have progressed to the point of causing noticeable disability
and/or the patient has sufficiently adapted their movements by using “two hands or
performing tasks more slowly” (Louis, Wendt, Albert, Pullman, et al., 1999, Comments
section, ¶ 1). Quite likely, especially in the elderly, tremor may simply be seen as “an
accepted and inevitable consequence of normal human aging” (Louis, 1999, ¶ 2).
Although one of the earliest accounts of the disorder has been traced back to the
late eighteenth century, the exact cause of essential tremor is yet unknown (Louis,
2001c). Ongoing research has pinpointed four areas of the brain that are likely suspect.
Two studies also note a possible degeneration of important brain chemicals. This most
recent data suggests that essential tremor may be much more similar to Parkinson’s
Aside from the functional disability caused by the tremors, which may affect
everyday activities such as eating, writing and shaving, new research has also found a
(Victor & Ropper, 2001, p. 101). As a result, ET’s affects on the social life are profound,
and it is not uncommon for those with this disorder to withdraw from all but their closest
differentiate this disorder from other movement disorders, especially Parkinson’s disease.
8
A discussion of the specific tools used in the clinical assessment will follow. I will
compile the latest research into the suspected causes of this disorder, the role of genetics
caused by essential tremor, I will note the physical, cognitive, as well as the social.
Various medical treatment options will be detailed, including oral medications, botulinum
injections and two types of neurosurgery. I will end the discussion of treatment
and biofeedback. The importance of stress management will also be highlighted as well
as hopes for future research. Although this paper is predominately research based, I also
hope to bring a personal voice, albeit a tremulous one, to describe the challenges of living
with essential tremor. As I have shared my own story to introduce this topic, I will also
share the stories of people whom I have come to love and admire. It is my hope that
raising awareness in the general population will result in a renewed interest in research,
more satisfactory treatment methods and, ultimately, in an increased quality of life for
functioning and mobility (Baylor College of Medicine, n.d.). According to Dr. John
Caviness (2000), Associate Professor of Neurology at the Mayo Clinic, most of the
restrict movement, such as Parkinson’s, rather than those that exacerbate it. However,
such hyperkinetic disorders are much more common and they often cause severe
9
disabilities. These disorders are also difficult to diagnose as there are often overlapping
areas of the brain which facilitate such erroneous movement (Caviness, 2000).
Essential tremor is the most common of the movement disorders causing tremors,
progressive, although studies note that the symptoms may progress more rapidly in
patients whose first symptoms begin after age 60 (Louis, 2000). The use of the term
essential implies that the tremor is the only significant finding of the disorder (Summit
Medical Center, n.d.), however, new studies alluding to cell death (Louis, 2002b) may
misnomer and will be addressed more fully in the sections relating to disabilities and
personal stories.
Classification of Tremor-Types
difficulty lies in distinguishing between the tremor types. For this reason, tremors are
classified in various ways: the two most important according to conditions that activate
the tremor and body part affected (Evidente, 2000). A third way to classify tremors is
through measuring the speed (frequency) of the tremors. This is usually denoted in Hertz
(Hz.).
Perhaps the most obvious classification is between a tremor that occurs when the
limb is at rest (resting tremor) or during activity (action tremor). Parkinson’s patients
most often display a resting tremor, as shaking can be noted when the hand is completely
supported in the lap. These tremors typically calm considerably when the hand is used.
10
Conversely, the essential tremor is generally inactive in the resting state and only
begins when the hand attempts a voluntary activity such as writing, eating or buttoning
clothing. An action tremor noted during these types of movement is more specifically
referred to as a kinetic tremor. And when the height of the tremor increases “during the
pursuit of a target” (Zesiewicz & Hauser, 2001), [as in touching a finger to the nose], it
may be termed an intention tremor. Although the kinetic tremor is often more severe in
ET (Brennan, Jurewicz, Ford, Pullman & Louis, 2002), there is also a postural
component. This type of action tremor becomes visible when the arms are held against
gravity, as in a fixed position, outstretched from the body (Zesiewicz & Hauser, 2001).
Another way to classify tremor is according to the part of the body that is
affected. In this respect, essential tremor may involve tremors of the hands, which are
generally more severe in men. Head tremors, which are typically more common in
2000). There also may be tremors of the voice, and even of the facial muscles including
the jaw and lip area (Burke, 2001). There are occasionally tremors of the legs, as well
(Bradley, 2001). To add to the complexity, there is also a type of action tremor associated
with a condition called dystonia that is caused by an abnormal twisting and tightening of
the muscles of the neck (Zesiewicz & Hauser, 2001). Tremors caused by dystonia may
also initially be mistaken for essential tremor (Louis, 2001a). While some researchers feel
that it is not uncommon for a patient to present with both conditions simultaneously (Lou
& Jankovic as cited in Baylor College of Medicine, 2001, p. 3) and the personal stories of
ET patients seem to support this, other notables in the field are doubtful that two distinct
neurological disorders would occur in the same patient. This only underscores the
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importance, as well as the difficulty, of distinguishing between the many types of tremors
Once tremors have been classified in the ways previously mentioned, the noting
of tremor speed may help to confirm the diagnosis of ET. Although there is some
crossover between various tremors, ET tremors generally measure 4-8 Hz, which is faster
than the tremors of Parkinson’s, (3-5 Hz) and slower than tremors brought on by extreme
Assessment/Diagnosis
While there are no definitive body scans or blood tests for diagnosing essential
tremor and the similarity of tremor types may add confusion, Dr. Elan Louis, Assistant
teaching videotape to aid in the diagnostic process. They note six tests in which tremor
severity can be calculated, both when holding a limb against gravity and during voluntary
movement. The tests include “arm extension, pouring, drinking, using a spoon, [touching]
finger-to-nose, and drawing spirals” (Louis, Barnes, Wendt, Ford, Sangiorgio, Tabbal,
Lewis, Kaufmann, Moskowitz, Comella, Goetz & Land, 2001). A handwriting sample
may also facilitate the correct diagnosis, revealing the ‘action’ nature of the essential
tremor which may in time become “large…and illegible” (Evidente, 2000, ¶ 12).
Conversely, in the Parkinson’s sample, there is no visible tremor and most notably, the
handwriting typically becomes very small. Note the spiral drawings, called the
Archimedes Spiral and the handwriting samples (Baylor College of Medicine, 2001) in
12
Because there are no specific tests for essential tremor the “diagnosis is based
a result, the patient’s own anecdotal history of the disorder is vitally important. One
question involves the age of onset. Although the tremor is extremely common in the
elderly it can begin at any time, with 1 in 20 cases occurring in childhood (Louis, Dure &
Pullman, 2001). Interestingly, the symptoms most often occur in the second and sixth
tremor in the early stages also help to rule out Parkinson’s disease, although, as noted, the
biggest difference between the two is that an ET tremor begins when movement is
attempted, rather than during rest, as in Parkinson’s. The tremors of ET are also fairly
symmetrical, and even if they initially affect only one arm, they will generally affect the
other as well (Burke & Hauser, 2001). They also usually begin, quite surreptitiously, in a
finger. Parkinson’s tremors generally begin on only one side of the body, typically the
thumb, and are much more noticeable at onset (Lieberman, n.d.). An early history of
intermittent tremor occurring only during times of exacerbated stress also points to ET,
but tremors of both essential tremor and Parkinson’s disease completely disappear during
Aside from the characteristics of the tremor itself, perhaps the two most important
findings derived from the patient’s personal history are the presence of a family member
with ET and the calming effects of a small amount of alcohol. The genetic component
will be addressed more fully in the following section; however, the role of alcohol as a
essential tremor will note a diminishment of tremor with minimal alcohol intake.
13
Conversely, alcohol has little or no effect on a Parkinson’s resting tremor (Victor &
Causes
Brain
Although Dr. Rodger Elble of the Southern Illinois University School of Medicine
has noted that researchers “struggle with the elusive origins of tremor,” it is hoped that
new brain technology will eventually be helpful (2000, ¶ 8). However, as the specific
area of the brain responsible for ET remains yet unknown, most studies have focused on
the oldest parts of the brain, noting increased activity pertaining to the areas of the
inferior olive, the red nucleus, the thalamus and the cerebellum (Evidente, 2000). Other
research noted definite sensorimotor cortex involvement but remained inconclusive about
more specific involvement (Hellwig, Haubler, Schelter, Lauk, Guschlbauer, Timmer &
Lucking, 2001). In an attempt to clarify the specific areas of the brain that are believed to
be involved in ET, Dr. Elan Louis noted the following: “These are nerve fiber pathways
that start in the inferior olive, [the] brainstem. They then travel to the cerebellum, and
then end up in the thalamus. This pathway is thought to be abnormal in ET” (personal
communication, August 14, 2002). Figure 3 in Appendix C indicates the suspected areas
Comparative brain research has also found differences in the ET brain, involving
chemical largely responsible for the activation of ‘fight or flight,’ has been found in
14
increased amounts in four areas of the ET brain (IETF, 2001). GABA, an inhibitory
chemical necessary to movement, has been found to be deficient in the cerebellum. Two
very recent imaging studies also found that cells containing a chemical called
N-acetylaspartate die at an accelerated rate in that same area of the brain (Louis, 2002c).
associated with cognition (Jessen, Block, Keller, Flacke, Lamerich, Schild & Heun,
2001). However, in the ET patient, it may be more specific to the tremor itself (Louis,
doctors note that the “chemical basis for ET is very poorly understood” (Louis, 2002c, p.
4).
Genetics
report that as many as half of all cases may have a genetic component. Heredity is by
affected individual will have a 50% chance of also developing the disorder” (NCBI, n.d.).
There are conflicting studies, however. A small control-based study of ET patients, their
children and other relatives found that “relatives of ET patients [were] five times more
likely to develop the disease than are members of the population and ten times more
likely if the [ET patient’s] tremor began at an early age (Louis, Ford, Brucht, Barnes, X-
Tang & Ottman, 2001). Such disparities are explained by one researcher who noted that
“despite the high prevalence of the disease, families that are informative for genetic
15
Although a specific ET gene has not yet been identified, genetic researchers have
February 12, 2003). One pertains to Icelandic families and another in four generations of
Americans (Burke & Hauser, 2001). Linkage has also been established in a third family
communication, February 12, 2003). Due to this highly genetic nature of the disorder, ET
Environmental
non-genetic forms of the disorder. Researchers note that in this “sizable proportion of ET
cases” environmental concerns may be the cause of tremor (Louis, 2001b). Although
researchers are only just beginning to study pesticides, lead, mercury and naturally
Disabilities
Although researchers are not yet certain of the definitive cause of ET, the
disabilities from this disorder are readily apparent. The fact that ET does not appreciably
shorten the lifespan of those affected and, in fact, may even be associated with increased
longevity (Baylor College of Medicine, 2001), has prompted some in the medical field to
refer to this disorder as ‘benign.’ Gratefully, more recent studies note that this is entirely
misleading and, in fact, suggest that those with ET “endorse greater social,
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disruption of work, home and recreational activities” (Busenbark, 1991, as cited in
Lacritz, Dewey, Giller & Cullum, 2002). More specifically, in a study of both physical
and social disabilities caused by inherited essential tremor, it was revealed that “60% of
individuals did not seek employment, 65% did not dine out, 30% did not use public
transportation, attend parties, shop alone or partake of a favorite hobby and 20% stopped
Physical
The physical disabilities from ET are derived from the tremor itself and
specifically correlate to tremor severity. A mild tremor may cause little or no physical
challenge; however, a severe tremor can greatly impede the type of fine motor dexterity
necessary for writing, eating and drinking, dressing, (Louis, 1999) inserting a key in a
lock, signing one’s name, threading a needle, etc ( Louis, et al., 2001). More extreme
difficulties are noted for those in professions requiring steadiness, such as “surgeons,
artists, musicians and drafters” (Evidente, 2000, ¶ 11) and of these, many are forced to
Cognitive
Aside from the variable physical limitations imposed by ET, new research is also
pointing to possible cognitive deficits. Mild impairment was found in the areas of
fluency and aspects of problem solving.” These findings were similar to the cognitive
17
Psycho/Social
upon tremor severity, and the cognitive effects mildly so, the social aspects of the
disorder can be devastating. In a study on social phobias secondary to ET, it was noted
that even for those whose tremors do not cause undue physical challenges, the
embarrassment from tremors when eating or writing in public often exact a high toll
(Schneier, Barnes, Albert & Louis, 2001). Researchers found that social phobia is present
this disorder note the importance to “diagnose and effectively treat ET in order to reduce
Treatment
Pharmaceutical
There are a variety of ways to treat essential tremor including the use of
medications and surgery. There is no medication specific to ET; however, many were
found to be helpful ‘serendipitously’ when treating for another illness and noting a
propranolol (Inderal), which was predominately used to treat high blood pressure, is now
a first-line treatment for ET (Louis, 2001a). For those whose tremors do not respond to
propranolol or for whom it is contraindicated (for elderly patients or those with low blood
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variably effective. Although there have been numerous studies of a variety of
(NCBI, n.d.). They are not without potentially serious side effects, however, including
low blood pressure and depression with propranolol use, and dizziness, nausea and
vomiting with primidone (Burke, 2001). For this reason, these and all medications for ET
(Neurontin) and topiramate (Topamax). Although they have not shown the general
(Alprazolam) are used primarily to treat the anxiety of social situations rather than the
tremor itself. As these medicines are potentially addictive, they are not recommended for
long-term use (Baylor College of Medicine, 2001). And although the effectiveness of
small amounts of alcohol has been noted as a diagnostic tool, there is often a ‘rebound’
effect after about two hours, in which the tremors may actually increase (Baylor College
of Medicine, 2001). However, doctors note that “the benefit of a glass of wine or a beer
prior to any engagement that might aggravate tremor should not be overlooked as a
Other medications have been used to treat ET including those for glaucoma and/or
depression (Baylor College of Medicine, 2001). Despite this variety of medications, even
those medications generally considered to be most effective for treating ET are less than
ideal. As one researcher noted, “even the front-line agents [propranolol and primidone]
19
are ineffective for a large proportion of the people who take them, and there is almost no
way to predict who will or won’t benefit from a particular medication” (Louis, 2002b, p.
1).
Medicine, 2001). Because of resultant muscle weakness when used for upper extremities
(Burke, 2001), they may prove to be more effective for head and voice tremors. Although
the use of Botox may provide optional treatment to those who cannot tolerate
medications, disadvantages include the high cost of the drug and the need for repeated
Neurosurgical
For patients with extremely debilitating tremors that do not respond to other
treatment modalities, two types of surgery have been used, both targeting the thalamus.
The older technique, called thalamotomy, involves destroying the overactive cells of the
thalamus that cause the tremors, (Tatter, n.d.). Although the advantages to this type of
neurosurgery include a long history of use and little postoperative maintenance, this
procedure is permanent and the adverse effects may include brain hemorrhage, muscle
weakness, speech disturbance and memory loss (Burke, 2001). A newer form of the
surgery called deep brain stimulation (DBS) may have fewer and less severe side effects.
[with] rapid pulses of electricity” (Tatter, n.d.). This technique costs more and also
(Tatter, n.d.). However, because the implant is temporary, “even if the stimulator is not
20
correctly placed, serious permanent neurological deficits can be avoided” (Baylor
College of Medicine, 2001). The pacemaker is typically turned off at night (via a
magnet) when tremors naturally cease and reactivated in the morning. The batteries that
power the pacemaker will eventually “need to be replaced, calling for a subsequent,
although minor, surgery” (Tatter, n.d.). Side effects from deep brain stimulation include
numbness and tingling, difficult speech, gate disorders (Baylor College of Medicine,
2001) and a foreign body implant that may be subject to infection (Tatter, n.d.). Although
DBS appears to result in fewer side effects than thalamotomy, one research study noted
the high rate of “device failures” and suggested that long-term evaluations be undertaken
(Burke & Hauser, 2001, ¶ 35). Although the surgeries can drastically reduce tremors for
as many as three years (IETF, Fall 2002, p. 4) it is important to note that surgery on one
side of the brain will only help the tremors on the opposite side of the body. Bilateral
surgeries are occasionally performed, however, only at the increased risk of side effects
Holistic/Stress Reduction
The role of medications and surgery in tremor control are extremely important.
For patients who cannot tolerate the inherent side effects and for others who do not wish
to expose themselves to added risk, alternative treatments may also be quite beneficial.
study did note the successful treatment of a patient with head tremor in three sessions,
21
equilibration” (de la Torre, 1989). However, the age of the study, the lack of subsequent
studies and the resolution of tremor in three visits remain troublesome, prompting one
Despite the fact that acupuncture treatment specifically for tremors has not been
validated by this research, acupuncture and other holistic methods geared toward stress
reduction can be quite beneficial. These measures target the release of noradrenaline,
(already increased in the ET brain), which results in the sympathetic nervous arousal
known as ‘fight or flight.’ Their effectiveness is not in diminishing tremor per se, but by
keeping the tremor from exacerbating under stress. In auricular acupuncture, in which ear
points correspond to bodily organs and functions, two such points may result in stress
reduction: Shenmen or “spirit gate,” which produces a generalized calming effect and the
Sympathetic Point, which balances sympathetic nervous arousal (A. Hasper, L.A.c.,
practice recommends a more thorough treatment, using the points mentioned above as
well as points that would “nourish the blood and yin of the liver on an ongoing basis.”
She also stresses dietary changes and the use of meditation to keep the “nerves settled
and calm.” And although Shpiesel realizes the chronic nature of this disorder and views
condition from worsening…[and possibly begin] to reverse the condition” (J. Shpiesel,
22
(EMG) or muscle biofeedback can bring muscular tension to the patient’s awareness,
even after the patient perceives complete relaxation. The use of neurofeedback, a more
specific type of biofeedback targeting brain waves, may aid the tremor patient by training
Although each of these methods may be viable forms of treatment, they require
clinical guidance and may be quite expensive. Conversely, autogenic training, which also
targets the sympathetic nervous system is easy to learn, can be practiced in minutes a day
and does not require clinical assistance or machinery. Developed in the early 1900s by
autogenic training incorporates the use of mental exercises and diaphragmatic breathing
to induce relaxation and “facilitate the natural self-healing mechanisms that already
Although there is little research into the specific treatment of essential tremor
using more holistic measures, it is likely that numerous other treatment modalities geared
toward relaxation will offer some benefit. Dr. Andrew Weil, physician, author and
founder of the Center for Integrative Medicine in Tucson, Arizona, highly recommends
adopting a practice of relaxation and suggests such methods as meditation, tai chi,
changes may also be of benefit. Eliminating stimulating foods such as those containing
caffeine is often warranted. Using covered glassware, straws and weighted utensils can
aid in feeding. Functional tools such as word processors and special computer ‘mouse’
23
Personal Stories
research on essential tremor, various coping strategies and the impact on the quality-of-
life of those affected, hard facts and percentages too often remain nothing more than
statistics on a page. For this reason, I turn to the intimate stories of those individuals who
have been living with the challenges posed by essential tremor. The stories have been
selected to show the variability of this tremor disorder, the frustrations and, hopefully, the
“Janet” (personal communication, October 18, 2001) is a 46 year old nurse and
mother of two children who has had tremor for 17 years. She has been diagnosed with
both essential tremor and dystonia, which often causes severe shaking of her head, neck
and sometimes her shoulders. Although she once described herself as “very social” she
notes that this is no longer the case “since this condition took over my life.”
Janet shared that her mother had essential tremor but that it was never explained
to her or her brother. Because talking about her mother’s shaking head was considered to
be “taboo” in the family, Janet was both embarrassed and worried for her mother. “Most
of the time she just looked nervous and out of control. Other times I thought she might
die from her shaking.” Janet had never known her mother’s official diagnosis until she
herself was diagnosed at age 29. Janet notes that her brother also has the disorder, but
adds that he does not let it “have a negative impact on his life.” Aside from worrying that
her own two children may manifest signs of this disorder, Janet also worries about the
affect of her tremor on the children and hopes to spare her children any embarrassment:
“When they were young the tremor was not too bad and I could control it by tightening
24
my neck muscles…and eventually developed …dystonia.” Until six years ago, Janet
stated she could “still hide her tremor.” Now, she states, “I hide myself.”
Like many with essential tremor, Janet tried numerous medications only to find
that they “either didn’t work or the many side effects outweighed the benefits.” Also like
least two glasses of wine and continue the ‘flow’ of alcohol in order to minimize the
tremor. Because I know my tremors will be worse afterward [due to the rebound effect of
the alcohol], I generally only accept those social invitations that are worth the trouble.
Janet is a registered nurse and would like to go back to work but is hesitant to do
so. She explains her reticence by noting that her embarrassment from the tremors even
impact everyday activities. “I see how other people sometimes stare at me as I fumble to
write a check and my head shakes away. I fear that they will think I am an alcoholic… or
that I will collapse at their feet in a seizure.” Although pain is not usually associated with
essential tremor, with the addition of dystonia as in Janet’s case, she notes that her “neck
muscles are in constant spasm because I try so hard to control the tremor.”
Janet also commented on the ability of high profile people to educate the public,
as actor Michael J. Fox did for Parkinson’s disease. Katherine Hepburn, who is often
erroneously believed to have Parkinson’s disease actually has essential tremor (Wang-
Cheng, 1998). And although the actress has chosen not to discuss her illness publicly,
Janet hopes for increased awareness of this disorder, as well. “It would help me feel more
25
As Janet explained more about how the disorder impacts her quality of life, she
noted with a laugh that even getting ready for the day is a challenge. “I put on my eye
makeup by holding my chin to steady it so that I won’t poke my eye out with the mascara
brush!” As the smile leaves her face, Janet’s head shakes again. But this time, it is out of
resignation.
diagnosed with essential tremor until ten years ago, she noted that her symptoms actually
began when she was in her teens. Now, at age 78 and recovering from major surgery for
the compression of her spinal cord, Robin looks back on a childhood that was shaped by
this disorder:
became more aware of it, I attempted to hide it, and it began influencing my
became more obvious, but still I never spoke of it, even to best friends. Nor was it
ever mentioned to me. I became more tense, more angry with myself and others
and more determined to hide the thing over which I had no control.
Like Janet, Robin also noted a correlation between an attempt to control her head tremor
I began turning my head to the right, avoiding looking at people straight on and [it
compensation, the large muscle on the left side of my neck became very strong
26
and distended, pulling my head to the left, causing my head to be off-set on my
difficult to write, use a fork, or carry a cup or plate. These additional problems
Robin’s family history also includes a parent with tremor. Although her father
was diagnosed with Parkinson’s disease, Robin notes that because his “hand tremor did
not show when his hands were at rest, he too, may have had ET.”
Although Robin has tried many medications and has had Botox injections on three
different occasions, she notes that only Klonopin has been “somewhat effective in
university movement disorder specialist advised Robin to undergo DBS surgery, which
she refused. “That was two years ago. Since that time I decided to ‘come out of the
closet’ with my … problems, talk about them to friends, and to get on with my life.”
However, complications arose three months ago resulting in emergency cervical spine
surgery to combat the threat of paralysis. Robin notes a connection with this latest
development and essential tremor. “I believe the ET compensation led to the torticollis
with the off-set head and the spinal curvature which finally compressed on my spinal
cord. I think the ET wearing on the cervical discs caused them to disintegrate.”
The impact of ET in Robin’s life has been quite profound and, like Janet, Robin
hopes that increased awareness will bring more effective treatment options for herself and
others:
some extent but never relieved me of the tension, stress and social pain associated
27
with feeling “different.” ET is an extremely destructive disease and deserves just
as much research and attention as Parkinson’s disease. [In its severity], ET causes
physical and emotional pain, an inability to reach one’s potential and ultimately
tremor in childhood. Now a 49 year old businessman with a wife and two children of his
I was only eight years old when I knew something was different about how I
would play games with my schoolmates. When my friends gathered to play pick-
up-sticks, I only resigned myself to being defeated again. Each time, when my
turn required me to move a particular stick away from the pile, I would instead
upset the pile and make it easy for my mates to move in and win. I also had
difficulty with the paint-by-numbers kits that my sisters used. I could never keep
hindsight that they did not require the fine motor movements that were so difficult for
him.
I did notice that my hands would shake in gross terms after intense physical
activities. Otherwise, the right hand would shake primarily at the fingertips. Only
when I was at family events or sports banquets was I reminded that the shakiness
in my hands was still present. I would catch the concerned looks of those around
me and would only say that I was a little nervous-- even though nothing could be
28
As John grew older, so did his concern for the shakiness in his hands. He was
drafted into Viet Nam and “when I took my pilot physical the Air Force physician
commented on my shaking hands but did not note it in my file. It was not mentioned
again.” When John entered into a sales career which required an increasing amount of
time spent in front of clients, his concerns were finally answered by a diagnosis of
essential tremor. “I remember the doctor even told me that millions have it, but so few
want to admit it.” To John, that fact made it seem much more crucial. “Just the sound of
John is taking propranolol, with good results “To this day, when public speaking
or presentations arise, I take my little blue pill and I am set.” John recently attended his
first ET support group and was not surprised to find that most in attendance were over 75
years old. “I know there are many [men] like me in age and action, but until we let them
know it is ok to have [ET] and discuss it, I am afraid it will remain the closeted shakes!”
Although the physical and social challenges of essential tremor may be quite
severe and may necessitate adaptations, retraining or even early retirement, at least one
woman credits her career in stress management to her tremor disorder. Citing the
“Wounded Healer [who] finds wholeness as healing flows through him or her to others”
(p. 7), Ann Bennett Sturgis, PhD, (2001) notes that she studied a multitude of healing
therapies in an attempt to help herself deal with the challenges of tremor. As a result, she
now uses those techniques to help others. Although her symptoms began early in
childhood and initially “seemed an impediment in her life,” she explains that she is now
29
I am well convinced that this ‘tremor’ that I have had so many years has led me to
blessing of connection to people and creatures of the earth. This is a long, long
way I have come from being at war with the tremor, thereby increasing it and
feeling angry and unhappy with it. It is such a fine signal telling me when I need
to manage my thoughts, words, images, and choices with the creative intelligence
Clearly, acceptance is key to living well with tremor. This point is reiterated by
artist Jim Fuess, who-- despite essential tremor-- maintains both his career and his sense
of humor. Jim’s particular brand of artwork is created with squeeze bottles rather than a
brush and he boasts of once completing a painting in three minutes; the catch was that it
took him 24 years to learn how to do it! In an interview for the New York Times, Fuess
It’s obvious when I’m in public. People ask, I tell them; I don’t volunteer. It just
doesn’t come up. I’ve had the tremor since I was born. [It primarily affects]
mostly my hands. It can affect your voice, arms, legs, head, individually or
collectively. There are medications, and I’m afraid of what they would do to me
personally and artistically. They all have side effects [and] not everything works.
Lethargy is one of those things that scares the hell out of me. (Fuess, as cited in
When asked about his specific day-to-day activities, Fuess responded with humor:
potatoes [to stabilize the peas on the fork]. Other than that, you know, I don’t let it
30
affect me. I don’t mix nitroglycerine in the backyard” (Fuess, as cited in Kuehl,
Although the ability of both Sturgis and Fuess to rise above the challenges of
essential tremor may be more the exception than the rule, researchers and patients alike
are indeed looking to the future with optimism. Dr. Elan Louis, whose research is
invaluable to both doctors and patients, recently received a bequest to fund further brain
research. Although he notes that essential tremor “is among the least studied and most
a new study which will research the “pathological…[and] the neuro-chemical basis for
ET” (2002b, p. 5). Louis (2002b) is also in the process of developing an ET brain bank,
where post-mortem brains can be compared to patients without ET. These studies will
“emphasize the cerebellum and brainstem because they may be starting points for ET” (p.
5). Should these studies confirm that there are neurodegenerative changes involved in this
disorder, Dr. Louis then hopes to address the possibility of prevention strategies (2002b).
Specifically, a new imaging technique called DaTSCAN has recently been approved for
use in Europe. This technique “allows identification of structures inside the body” and
may aid the diagnostic process by more easily differentiating between essential tremor
There may be one other area of hope for the future of ET: stem cell research.
Political and religious concerns aside, these cells typically can be used to mimic any cell
that is needed in the body. Although research on stem cells have not specifically
31
addressed essential tremor and it would likely be many years in the future, if at all, Dr.
I think stem cells would make sense, although this is now closer to science fiction
than science. If, indeed, cells have died in the [ET] brain, then replacing those
cells would probably be of value. However, these cells would need to form the
2002)
Conclusion
Despite the fact that essential tremor has affected the lives of Samuel Adams and
general public. With the compilation of this extensive research, it is hoped that this will
no longer be the case. Although the exact cause of essential tremor has not been
determined, researchers are fairly confident that the areas of the brainstem, thalamus and
the cerebellum are mostly likely suspect. And for the first time, recent research suggests
that a neurodegenerative process may also be involved. Genetic studies, although limited,
have discovered at least two chromosomal linkages for ET and further research into the
However, despite the value of Botox injections and surgery for a small population
tremor patients remain only minimally effective. As such, the physical and cognitive
disabilities inherent in this tremor disorder test the knowledge of researchers as well as
the resilience of patients. The impact on quality-of-life issues, however, may be the
32
biggest challenge of all-- not necessarily in having the disorder, but in living with the
Although the purpose of this thesis paper is solely to educate the general
population about the disorder known as essential tremor, it is hoped that the ramifications
of this education are much more far-reaching. With continued interest in research, the
exact cause of this disorder may eventually be realized. At that point, more effective
treatment modalities likely will be developed. And then, perhaps prevention strategies
will not be far behind. And for those who have witnessed their own trembling head or
hands and have not yet sought medical attention, may the research examined here provide
the impetus to invest in one’s own healthcare. And, at the very least, if this newfound
knowledge opens an empathic dialogue between neighbors and strangers alike, then the
challenges of living with this disorder may become easier to bear. Will people with
essential tremor choose to mix nitroglycerin in their backyard? Absolutely not. However,
when tremors are met with sensitivity and acceptance, with healthy curiosity but not
judgment, then quite possibly that soup-eating contest may someday become a reality.
33
Appendix A
Figure 1. Spiral drawings are often used in neurological assessments for essential tremor.
(see p. 12). This one was drawn by a 78 year old woman with ET. (Jean, personal
Figure 2. The first handwriting sample, tremulous but normal in size, was written by the
essential tremor patient above. The second sample is an example of micrographia, the
small handwriting typical of Parkinson’s disease. (see p. 12). (BBSF, 1998, p. 15).
34
Appendix B
Table 1
voice
Tremor type Postural, kinetic, rarely resting Resting > postural, kinetic
(speed)
(Slowed movements)
35
Appendix C
Researchers note that the loop (in yellow) involving the structures of the
brainstem, the cerebellum and the thalamus are abnormal. Their functions are as follows:
Brainstem: The portion of the brain closest to the spinal cord. It contains the
medulla, pons, and the midbrain and controls many of the involuntary functions that keep
us alive.
functions, the primary role of the thalamus is to relay sensory information from other
36
Addendum
the research compiled in this thesis, but to give additional voice to the great variability of
symptoms and the challenges posed by essential tremor. It has been noted throughout this
paper that the diagnostic process is a difficult one. Although there is some disagreement
in the likelihood of a patient having two distinct neurological disorders (such as dystonia
and ET), the following stories suggest that many patients have been diagnosed as such. I
have every confidence that ongoing research by a dedicated few will resolve such
discrepancies. In the meantime, however, these stories reflect a deeper truth; one born out
of personal experience.
To those amazing women and men who have contributed their stories, I thank you
Jeremy
shaking at about age 40. I had a pretty good idea that it was hereditary because my
mother and her brother had the problem. It was thought to be due to some sort of brain
deficiency. So, I lived with it as it got worse. Around 1992 I began drug treatments. After
trying them all, I gave up and had the DBS operation in September of 1998 on my left
side - so that my right hand was helped. I have been incredibly better since then with the
exception of the last year. When my left hand began to get worse, I asked the experts
about performing surgery on that side also. They were reluctant to do so because some
who underwent bilateral surgeries have experienced unforeseen difficulties with speech
and balance. I don’t need any more difficulties than I now have, so that’s it for me. Now,
37
I am still incredibly better, but less so as the tremor’s strength increases. I guess I am a
work in progress.
Leon
I am too shaky to write much. Started age 10, now 69 (worse last 10 years).
Despite tremors and severe hearing loss, managed two PhD’s and psychologist license.
Still in practice. Went through most of my life working around impairments. Still manage,
though harder.
I wrote back to Leon and wished him well. I told him that he was an inspiration.
or adjusted to numerous bodily assaults because bed is boring. I also enjoy exercise,
which helps. I learned to love learning, and with handicaps, it’s the only place I could
excel.
Mimi
I am a married 47 year old female with two children, ages 20 and 12. I have a
positive family history of tremor for two aunts; one deceased and one with Parkinson’s
disease. Two years ago I noticed that when I was using an eating utensil my hand was
shaking slightly as I raised it to my mouth. I thought I had just overdone some weight
workouts. A couple of months later I was raising my cup to my mouth and, much to my
surprise, my cup started to violently shake. Off to the doctors I went and was told I had
essential tremor.
My tremors progressed from just my right hand and arm to my neck. This affected
38
involved and then the legs, although they didn’t affect my walking. The tremors were
always of a very low-key variety. The neck tremor was the most annoying and tiring. And,
I tried to talk my way through the tremors in my throat, or, more accurately, force my
I have seen an Ayurvedic practitioner twice and they have been heaven-sent!
Their diagnosis is that one of my doshas [metabolic principles] is out of balance – in this
case VATA [governed by ‘wind’ and responsible for movement]. Vata is light, dry, cold,
fast, and airy – therefore, to counteract the tremors I must do the opposite. I eat heartier
meals with soup and bread, creamy foods, warm foods. I move a lot slower, calmer and
keep myself in a more relaxed state. I cover my head with a hat in the wind and cold to
keep myself from getting ‘scattered’ and to keep my heat in. I dress much warmer than I
used to. I sleep at least 8 hours a night. I minimize stressful situations in my life by being
more assertive as to what upsets me rather than letting people push me past my comfort
zone. I have had a couple of Ayurvedic whole body messages with sesame oil. The result
I have a great deal of respect for this approach since the tremors make themselves
more noticeable when I am cold, tired, stressed and underfed. For a year now, I have
Today my tremors are so gentle that you would not be able to notice them. I
recently just returned from a two week yoga retreat where I also saw an Ayurvedic
39
practitioner. In fact, I am hardly aware of even the tremor in my right hand, which is
I have modified my lifestyle quite a bit. I soothe and pamper myself by healthy
lifestyle choices. In fact, my husband pointed out that through modifying my lifestyle I
have duplicated what the beta blockers attempt to do: slow one down by decreasing one’s
heart rate.
I choose not to closely identify with my condition. I do not want to internalize that
positive thinking and affirmations and a very healthy lifestyle. I have told very few people
about my condition and initially only because it was somewhat obvious. I also did not
want to use it as a crutch or an excuse for making decisions in my life. It has made me
aware that I cannot take my good health for granted and to enjoy the miracle of everyday
life.
Catherine
The tremors began in 1996 when I was 48 years old. I noticed them in my left
hand while taking a class in Swedish massage. Although I did not feel nervous about
taking the class, I dismissed the tremors thinking that perhaps I was feeling more nervous
than I thought. Then in July 1998, I had an industrial injury that affected my back. This
overly zealous move created a herniated disk in my lumbar and sacral spine area. This
stress and depression. Ultimately, the tremors took over and were bilateral. Within a
three-month period after my back injury, my mother passed away. Soon after, a diagnosis
40
of another neurological problem arose; glaucoma was added to the stressors of my
everyday living.
barrage of tests that included blood tests, urine, MRI of my brain, neurological tests
I had no idea what essential tremor was, nor did I have any idea as to how bad it
control my tremors, I went home. (The medication, primidone, was discontinued due to
unacceptable side effects.) Still, I was mystified by my diagnosis and began my own
search on the Internet. After an extensive search I found the home page of the
International Tremor Foundation. I learned so much from what IETF had put on their
WebPages and decided that I had much to be thankful for since my disorder was not
Parkinson’s disease.
had some discrepancies when I compared my tremors to those of our support group
members. Yes, I had some tremor when using my hands to pour liquid into a cup or when
carrying a cup and saucer to the table. However, I found that I also had a resting tremor
in my left hand and my left leg developed a tremor. I also noticed a presumed weakness
in my left side and that my gait had become shorter. The difficulty I experienced raising
myself out of a chair had become quite noticeable and I had also fallen a couple of times.
I felt very unstable. Then my husband noticed I was not swinging my left arm when I
walked. It was clear that there was more to my diagnosis than essential tremor. Over
41
time I realized my tremor was also predominately one-sided. I began to think that the
anxiety and the stress of my industrial injury, my mother’s passing and diagnosis of
The doctor confirmed my dual diagnosis. I not only have ET, but I also have
Parkinson’s disease. The definitive diagnosis came after he prescribed Sinemet for my
tremors. Within three days I called the doctor and exclaimed that I had no idea the
medication would work so quickly to alleviate my tremors and the painful stiffness in my
shoulder and neck muscles. (In fact, I felt the results of the medication within the first
disease!”
I guess that was the ultimate test. If one responds to the medication it is usually a
It has been a long time since my Swedish massage class. A lot has happened
since then. I feel so very grateful and blessed for all I have learned since that fateful day
in 1996.
Jennifer
I am a 51 year old female who has had ET from birth. My mother had it very
badly. I feel that doctors cannot comprehend the different symptoms of ET in each
individual. All the symptoms are similar, yet are quite varied. The doctors seem to sink
into the strict rule of the medical books, but with ET everything is different everyday.
When it comes to the brain and how it all works, no one, and I do mean no one, can
understand the intricate electrical or emotional circuits of the grey matter that collects
42
all of our memories and emotions and helps us to forget or remember.
As a child I was always shy. I did not know my mother until I was 21 years old.
My father was an alcoholic. Life was difficult. At school when the teacher would ask for
me to come before the class and write on the board, I would become petrified. My hands
would shake and the thought of my classmates seeing this and then making fun of me was
not pleasant. I was chosen for an outstanding book report in the second grade and was
asked to go before a group of teachers to share it. I sat in sheer terror of going in front
of anyone as I did not want to be the center of attention. Standing in front of the teachers
often brought on a nosebleed. I wanted to die. As they tried to clean me up I just bled
more and shook more. I flunked second grade due to my handwriting and, I'm sure, due
to my worry that performing well would set me apart and require me to be the focus of
My father took me to the doctors to find out why I shook so much. All I remember
is that the doctor said that I would never be fat in my life. At the age of eight this was of
no importance to me. However, he was correct, as I have been thin all my life. I also
never smoked cigarettes in high school like my friends. I just wouldn't look cool with a
shaky cigarette in hand. I did find that marijuana or other mild downers helped calm me
down. It felt so good to not have my insides shake to death. To be normal like everyone
else! To be able to think straight! Sometimes I just couldn't think. I couldn't talk.
I had surgery on my spine 1 1/2 years ago. A disk was removed and I have never
been the same since. While in the hospital in recovery I began to go into shaking
seizures. The doctors did not know why and the small amounts of tranquilizers they gave
43
me did not help. They were more concerned that I would become addicted to the drugs
than stopping my tremors. This misplaced concern did not make sense to me.
I have found out that being under anesthesia also irritates ET. My voice is very
affected now. I have about 60% of my voice most of the time. I stay home because I
never know when my shaking seizures will start and my voice will disintegrate. I had to
go in front of the social security officer and judge to be declared unable to work. This
was very frustrating as most people do not know of ET or how disabling it is.
I feel that stress does bring on more symptoms and the doctors need to be more
open to the emotional side of the life of someone with ET. We may not need a wheelchair
Annie
My ET began with a head tremor when I was 45. My family doctor had no idea
what was causing it and he sent me to a neurologist. I related that my elderly father’s
head shook, as did his mother’s, but I thought their shaking was just old age. The doctor
determined in a very few minutes that what I had was “familial palsy.” This was in
1973, years before I heard the term essential tremor. However, from the description of it,
I was certain he was right. The doctor told me that I must learn to live with it, as there
was no medicine for it except tranquilizers. I was told that these were addictive and
I took the doctor at his word and tried to live with it until 1996 when I read about
a support group and joined it. Every member except me was on medication. I went to
another neurologist who diagnosed it as torticollis. I didn’t think I had torticollis because
my grandmother did, and in her pictures her head was always bent to one side. A third
44
doctor agreed with the first and said I had ET. I was prescribed propranolol and have
In 1980 I began having trouble with my voice. I went to a speech therapist and
when I was comfortable in his presence my speech did get better, but only with him. He
released me from his care because he could not do anything for me. Still, answering the
phone in a one-person office was terrifying for me. I have found that when I get to know
a person well and feel comfortable with them that I am able to speak almost naturally.
The voice tremor has been more embarrassing to me than the head tremors or the hand
tremors which developed nearly 10 years ago. I do have trouble signing my name at the
I have worked part-time until I was 70. I am now 75. However, I have cried over
jobs I didn’t get and I always felt that it was because I told the truth about my ET. Some
people think my voice is due to a stroke. If I had one, I never knew it. Several of the
doctors I have seen over the years told me that my vocal cords are partially paralyzed.
I have discovered that I will not speak up in a group because of my voice tremor. I
belonged to a book club in my former hometown and I faithfully read the books, but I
very seldom spoke up at the meetings because my voice would shake. I gave a few
reviews over the years, but always had to use my notes. Even then, my voice quivered. I
finally gave up. I will not take Botox injections because they don’t seem to help everyone.
I had to give up my hobby of making ornaments for a few years, but I was able to
make some again last year. It gave me so much pleasure. I have not seen a neurologist in
about five years, but because both my head and hands are shaking again, I will do so. I
45
am sure I can get the name of a good doctor from my new support group or from our
dedicated leader.
Dennis
I am 71 years young and belong to a support group. I was the youngest of eleven
children raised on a farm. My mother said that I had always been jumpy. Since she had a
difficult childbirth with me, she blamed herself and went to her grave believing she was
the cause of my shaking. ET manifested itself in me with full blown tremors at age
thirteen. Over the years I have seen numerous doctors but was not diagnosed until I was
54 years old. When I received the diagnosis, I went home and cried. I finally could put a
ET caused a great deal of mental anguish over the years. I was made fun of and
ridiculed by children during my school years. I could not understand why I had these
shaking arms and hands. No other part of my body has been affected. I determined very
early in life that this was not going to totally control me. I also suffered the wrath of
some school teachers about my hand writing. I did participate in sports and was quite
successful at it, but in the back of my mind I always wondered what I could
have accomplished had I not had tremors. The same applied later in life as far as my
occupation was concerned. I did remain employed until I was sixty-five and decided to
retire because the tremors were becoming progressively worse. Had it not been for ET, I
I had one brother who had shaky arms and hands. My father had minor tremors. I
have one sister who has tremors in her voice and head.
46
I did serve in the U.S. Air Force during the Korean War. I lied to get in. I told
them the tremors were present because I was nervous about my physical. I served four
years in spite of the tremors, but again, there was much ridicule from some of the
throughout my entire life. The public and much of the medical profession do not fully
understand ET. When I tell some of my physicians I have ET, they say, "Oh yeah, I know
what that is." They may know what it is, but I don’t think they understand how it affects
us.
I choose to live my life in spite of what people think. I still refuse to hibernate
because of ET. I participate where I wish. If people think it is funny, then they have a
bigger problem than I. One of the positives I have gained from ET is that it has made me
a more sensitive and compassionate individual. I do find that most people understand
when I take the time to explain ET. Many are quite helpful, especially servers in
I am currently taking both propranolol and primidone. This helps control my ET,
but not completely. I would not be able to function without the medication because I
would need assistance in dressing and eating and many other daily tasks.
Laura
I was 45 years old when my oldest child went off to an out-of-state college town
where my daughter also lived. For eight years my husband and I made that long drive,
but at one point I began to have difficulty keeping my eyelids from shutting as I looked
out the window into the outdoor brightness. On our last trips, I was wearing two or three
47
pairs of sunglasses simultaneously, and my eyes still would not behave. This is my first
In my 50’s, I began doing a lot of hiking. When I returned to my car, I would try
to write down the name of a bird we saw or a restaurant someone told me about. I found
that I couldn’t write. I could not take control of my hand movements. The only marks I
could make on the paper were jagged lines. At an annual physical exam, I asked my
doctor about my hand-shaking and asked if it was just a part of the aging process. She
When my mother died, the blepharospasm became so severe that I was driving
around with one hand holding one eye open, while the other eye remained tightly shut. I
learned that if I pushed into the area above and to the side of my eyeball, the squeezing in
of that eyelid would be less severe. But I was a very handicapped driver and I began to
opt out of activities which required me to drive. As anyone in the U.S. knows, this is
severely limiting! I also could not sit in a restaurant facing a window if I wanted to look
at my companion’s face. Facing bright light, tiredness and physical or emotional stress
Over the course of two years, I had exams by three different reputable
ophthalmologists. The first one tried treating me for allergies; the second one for ‘dry
eye.’ When I asked the third doctor to be a detective (because ordinary exams were
glands of the eyelids]. I finally returned to the first and most trusted doctor who watched
the muscle action around my eyes and confirmed the diagnosis of blepharospasm.
Ironically, I had come to the same conclusion by doing my own research in the library!
48
The doctor observed, helpfully, that this disorder was affecting the muscles around my
medical center. Instead, I once again referred to my home medical book for laypersons.
In a reference under Essential Tremor, it stated that the eyelids could also be affected.
order to see the tremors that began when I faced the sunlight. Although she
acknowledged the tremors, she told me that there was nothing she could do for me. In
other words, in her opinion, I did not have a neurological disorder. (I was making this
up???)
movement disorders. She took my history and watched the blepharospasm when I opened
the blinds on the window. Her diagnosis was focal dystonia for my eyes, and either focal
The specialist recommended treatment with Botox, but I decided against it. I tried
Cogentin for the blepharospasm, which is working fairly well. I also take propranolol for
the hand tremor, which dramatically stops it. Because propranolol apparently does not
affect focal dystonia, the specialist felt that the hand tremor is most likely ET. Although
my hand tremors affect my handwriting, I have never had difficulty holding a glass of
liquid. And unlike many with ET, alcohol does not affect my tremor in any way. I do have
a warning about Cogentin. Early on, I was taking this medication twice a day. On two
occasions I forgot to take it with dinner and took it before bedtime instead. On those
occasions, I had terrifying hallucinations in the middle of the night. There was never a
third time!
49
Presently, at age 62, I have limited my driving to our local area only. On our
vacation last summer, I attempted to drive again and it was a mistake. There was no
accident, thank goodness. Afterward, I asked my husband how he felt while I was driving.
He hesitated for a minute and, ever the diplomat, replied, “Very alert!” So, we no longer
depend on my driving assistance, except to get myself to the grocery store, to my local
friends and to the BART station. I also do not drive between 2-4 pm, when my body gets
I have had to increase my medications from the time I first began taking them and
I suspect the years ahead will see changes in this condition and in the treatment of it. But
This contribution was originally written to the parents of a two year old boy who
had been diagnosed with ET. I received permission to include it here and do so with an
Keith
I have had ET since very early childhood. I am now in my early 60’s. My mother
had it, as did her father. My brother has it also. Now one of my daughters, age 34, is
beginning to experience a tremor in her right hand. As we come from a rather proud
Irish Catholic family, I have always figured ET was given to me so that I would manage
to keep my ego under control and understand that it is important to be somewhat humble.
My tremor exhibits with a left-to-right jerk of my head and, about five years ago, I
began to notice a tremor in my right hand and arm when I worked out in the yard or
otherwise did strenuous activity. That hand tremor is now progressive but I can still drink
50
a hot cup of coffee without spilling. My head tremor is becoming more amplified and time
will only increase my “shaking.” However, I feel I have been prepared for whatever
physical motion I assume because my mother had the good sense and compassion to
Mom never talked about my tremors but if someone else would bring up the
subject, she would just smile and say, “That is Keith’s way of getting a little more
attention.” My family simply accepted me and my tremor as that which identified who I
was. If they could have seen me without any tremor they would probably have wondered,
imagine that an increase in severity would result in a more self-conscious attitude. But in
1) My tremors are part of me and I will always try to be the best person I can be
no matter what! (And believe me, I have made a lot of mistakes trying to get to this
acceptance level.)
2) When my children begin to “shake” (and, as one has, more undoubtedly will), I
will share my feelings honestly but not overtly. In other words, when all is said and done,
it should be treated as “no big deal.” Because that is what it is - or what it really should
be!
3) Keep in touch with others who have this condition. Having common ground is
always more comfortable and new medical advances are interesting to share and discuss
51
4) Love is what this life is really about. If you love your child with ET, they will
pass on that love to others. And ET, in the middle of such love, will be only a slight
inconvenience.
52
Annotated Bibliography
Brennan, K., Jurewicz, E., Ford, B., Pullman, S., Louis, E. (2002). Is essential tremor
study. Movement Disorders, 17(2), 313-6. Retrieved July 23, 2002, from Medline.
The authors note that although essential tremor manifests itself with both kinetic
limb is held against gravity] in nature, rather than kinetic [noted during
cases, it was noted that the kinetic tremor was rated twice as severe as the postural
tremor. As a result of their findings, the authors suggest that kinetic tremor be
Burke, D. & Hauser, R. (2001). Essential Tremor. eMedicine Journal, 2(11), pp. 1-20.
In this 20-page article the authors detail the most current research into essential
sex and quality of life issues and genetic preponderances. Differential diagnoses
are presented to note the association between ET and other disorders such as
alcohol in the treatment of ET. The authors end with a discussion of the various
53
Deuschl, G., Bergman, H. (2002). Pathophysiology of nonparkinsonian tremors.
Parkinson Disease and notes that although these disorders make up the second
largest group to be treated with neurosurgery, much is yet unknown. The authors
note the four basic principles from which tremors, or oscillations originate and
state that although they often interact with one another to produce a specific
discussion are essential tremor, dystonic tremor, cerebellar tremor, Holmes tremor
disease. He notes the exacerbation of tremor with stress or fear, and the role of
notes that those in occupations requiring fine motor skills are often forced to quit
their profession. The author provides a table for the various medications used to
treat this disorder and ends with a discussion of the two most common surgeries,
thalamotomy and deep brain stimulation. It is his contention that although both
surgeries are equally effective, the deep brain stimulation has fewer side effects.
54
Kuehl, C. (2000, October 22). Despite a disorder, a creator with many hats. New York
Times p.14.
The author interviewed artist and part-time curator Jim Fuess who has had
tremor is a story of a man with mild tremor who has not let it become a disability.
The article showcases not only Mr. Fuess’ handling of the disorder with humor,
but his artistic talent and his big heart. He hopes to have his artwork reproduced
onto greeting cards, donating the proceeds to the International Tremor Disorder in
Kansas. Although the artist admits to tremor of his hands, he is still able to create
Lacritz, L., Dewey, R. Jr., Giller, C., Cullum, C.(2002). Cognitive functioning in
After summarizing the prevailing research on essential tremor, the authors note
the unfortunate and misleading use of the term ‘benign tremor.’ They embarked
on a study of the neurocognitive functioning of patients with severe ET, and used
patients with Parkinson’s disease as controls. The results showed that the ET
group performed below the mean in cognition flexibility, figural fluency and
problem solving were also diminished in this group. The authors concluded that
there is a definite correlation between severe ET and mild cognitive deficits. The
study also suggested that various areas of the brain, those correlated with specific
55
Lieberman, A. (n.d.). The National Parkinson Foundation, Inc. How is Parkinson disease
different from essential tremor? Miami, FL: NPF. Retrieved July 28, 2002, from
http://www.parkinson.org/pdvset.htm
This layperson’s article clearly notes the major differences between Parkinson’s
including the minimizing effects of alcohol to tremor and the genetic correlation.
A very brief discussion of front line drugs is included as well as links to other,
891.
This article details the clinical workup of a 66-year-old woman with essential
tremor who presented with severe restrictions to her activities of daily living as
well as in her employment as a writer. The author describes the process for
of the disorder, age of onset and rate of progression. Various treatment modalities
author makes the point that although a reduction in the size of the tremor is
important, the best judge of whether treatment is effective is to the extent that it
56
The author notes that although ET can be traced back thousands of years, much is
yet unknown about the pathology of the disorder. Although it is generally agreed
that this disorder has a genetic component, the author states that ET is not
completely genetic. This study discusses the similarity between tremors caused by
found in both meat and vegetable-based foods; and manganese. It is the author’s
Declaration of Independence may have had one of the earliest known cases of
essential tremor. The author cites numerous references that Adams was bothered
by a hand tremor as early as his 40’s, which progressed to the point that he had
others write his correspondences for him. The early age of onset, its manifestation
upon initiating movement, as well as the fact that Adams’ daughter and her
children reportedly also had tremor, ruled out Parkinson’s and subsequently
brought the author to the conclusion that Adams’ tremor was likely due to
essential tremor.
57
Louis, E. (2002a). 1H magnetic resonance spectroscopic imaging to identify metabolic
The author notes that biological markers for essential tremor have not yet been
or death. The author hopes that an upcoming study of 26 ET cases will more
clearly identify such biological markers, enabling the design of long-term studies
neurodegeneration.
The author challenges both doctors and patients with essential tremor to become
advocates, raise awareness and support ongoing research for this disorder that
affects such a large part of the population. Noting that many of the frontline
medicines unsatisfactorily manage the disorder, the author calls for new research
to prevent the disorder as well as explore other therapeutic treatments. The author
ET, noting that essential tremor may be more like Parkinson’s than originally
believed. He concludes his article by explaining the need for more post-mortem
examinations and notes that a brain bank is being established to better understand
58
Schneier, F., Barnes, L., Albert, S. and Louis, E. (2001). Characteristics of social phobia
among persons with essential tremor. Journal of Clinical Psychiatry, 62(5), 367-
372.
The authors note that although social phobias as a result of a medical disorder are
excluded from the DSM-IV social phobia criteria, they can be nonetheless
primary social phobias (social phobias diagnosed prior to onset of ET) were
compared with essential tremor patients with secondary social phobias (occurring
after diagnosis of ET). In the primary social phobia group, over 80% noted that
their social phobia was not related to ET. Conversely, 62% of those with
secondary social phobia attributed their phobia symptoms entirely to ET. Fears
and avoidance specific to the tasks of eating, drinking and writing in public were
also much greater in the secondary phobia group. The authors conclude that social
substantial minority of patients, more commonly among those patients with early
regardless of the severity of tremor. The authors suggest that further studies be
made to note the viability of treating the social phobia as well as tremor.
South Shore Neurologic Associates. (n.d.) Essential tremor. NY: SSNA. Retrieved
tremor/tremor-2.html
This general article written for the layperson distinguishes essential tremor from
59
their potential side effects. Other treatment options are discussed including Botox
injections as well as nondrug therapy options such as the use of light weights
strapped to the wrists to dampen the tremor. The article concludes by assuring
patients that essential tremor is not attributable to a diminished mental state, nor is
http://www.home.mdconsult.com/das/arti...7301&sid=115829192/N/245496/
1.html
In this 28-page document, the authors discuss various types of tremor, their
tremor, the authors classify the tremors by various means: the body part affected,
speed and height of tremor, specific posture that elicits tremor and the
tremors of essential tremor including those of head, hands, voice, legs and chin.
They follow with diagnostic, clinical and treatment (pharmaceutical and surgery)
specifics for each type. Tremors from other sources are detailed as well.
60
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