Understanding ET

UNDERSTANDING ET

What Are the Symptoms of Essential Tremor?
The primary symptoms associated with essential tremor include:

Uncontrollable shaking that occurs for brief periods of time
Shaking voice
Nodding or shaking head
Tremors that worsen during periods of emotional stress
Tremors that get worse with purposeful movement
Tremors that lessen with rest
Balance problems
Not able to write your name

Never heard of essential tremor?
Perhaps it's because so many other conditions compete for headlines. Advocates for people with ET have not had the benefit of a celebrity spokesperson such as Michael J. Fox or Muhammad Ali, who both have Parkinson's disease. The most famous person with ET was the late actress Katherine Hepburn, who parlayed her disorder into an intriguing component of both her vocal and on-screen personae. Other notables with ET include retired Supreme Court Justice Sandra Day O'Connor and the late West Virginia Senator Robert Byrd, who passed away last summer.

Although the disease is not fatal, it can cause severe disability.

WHAT IS ESSENTIAL TREMOR? Essential tremor is a progressive neurologic condition that causes involuntary rhythmic trembling of the hands, head, tongue, voice, and less often the legs or trunk. Like Parkinson's disease, ET is considered a movement disorder. The disease is caused by abnormal communication—sometimes called a “misfire”—between certain areas of the brain including the cerebellum, thalamus, and brain stem.

Tremors may be more prominent when the person is highly active or anxious. A vicious circle can occur with ET: The person fears embarrassment in public, which in turn makes the tremor worse. As a result, people with ET may limit social interaction and even develop social phobia. Sometimes, observers assume the tremor is due to drug or alcohol intoxication.

“Essential tremor can cause significant disability. In addition, tremor tends to worsen with age.

NOT THAT BAD—OR IS IT? But health care professionals actually wish more people with ET would speak up about their condition.

It's clear from studies that the majority of patients have not been to a doctor or diagnosed.The reason may be that the condition is initially mild—in fact, ET may remain mild for decades—or because a family member has ET and relatives become used to it.

“Often as patients approach retirement or late middle age, the tremors become more noticeable

COMMONLY MISDIAGNOSED Essential tremor can be easily misdiagnosed as another movement disorder, most frequently Parkinson's disease. Essential tremor actually occurs eight times more often than Parkinson's. A handwriting test is often used to distinguish between the two, says Dr. Lyons. “Essential tremor often results in large, shaky handwriting samples. Parkinson's writing typically starts out normal, then gets smaller and smaller.”

Tremors of the voice and head also do not generally occur in Parkinson's but are commonly seen with ET. A patient suspected of ET should be examined while in a reclined or seated position with complete body support.

Dystonic tremor is also often confused with ET. Patients with dystonia exhibit an abnormal, involuntary spasm or contraction of muscles in the face, neck, hand, or torso. When muscles aren't used, they normally relax. With dystonia they do not, resulting in abnormal postures.

General neurologists treating tremor have a diagnostic error rate of 25 percent to 35 percent. “There is no laboratory test for ET, no biological marker to diagnose it.

MEDICATIONS AND RESEARCH One New York-based study showed only 8 percent of patients had actually been prescribed medication for tremor. Furthermore, patients who try one or more medications for treatment usually find them to be less than satisfactory. No medication has been specifically developed to treat ET.

Experts consider the best medications to be propranolol, an FDA-approved beta blocker, and primidone, an anti-epileptic. The anti-anxiety medications clonazepam, diazepam, lorazepam, and alprazolam, and the anti-convulsants gabapentin and topiramate, have also shown some benefit for ET.

Most experts agree that primidone is the most effective treatment for ET, followed by propranolol and other beta-blockers. If tremor is not sufficiently controlled with one of these medications, the two can be used in combination. If these medications do not control tremor or cause intolerable side effects, then gabapentin or topiramate are probably the next most commonly tried medications. Benzodiazepines are also used, especially if anxiety is also an issue, but the potential for addiction should be considered.

Meanwhile, current research focuses on finding the gene that causes ET. In many large families, the condition appears to be dominantly inherited—that is, there's a 50–50 chance each child has it.

Finding a dominantly inherited gene is viewed by experts as crucial to finding a cure.

Essential Tremors is when you have uncontrolled shaking movements in parts of your body - most commonly the arms and hands. It is more common with increasing age. It tends to occur in families. It is mild in some people but can become severe and disruptive to daily activities in others. There is good treatment available in the form of medication and occasionally surgery.

What is tremor?A tremor is a repetitive movement of a part of the body. It is involuntary. This means that it is generally not controllable and happens without you deciding to move that body part. It is often felt as a trembling or shaking sensation.

Action tremor: A voluntary movement such as lifting a cup to one's mouth.
Postural tremor: A voluntary holding of a position against gravity such as reaching or extending one's hand or arm. Most people with ET experience both postural and action tremor.

Essential Tremor is the result of abnormal communication between certain areas of the brain, including the cerebellum, thalamus, and brain stem.

The cause of ET is unknown, but there is evidence that for some people the disorder is genetic. However, people wit no family history of tremor can also develop ET.

A slight tremor is present in all people. That is called physiological tremor. It may not be noticeable. Certain things will make a physiological tremor more noticeable such as caffeine (in coffee, tea and cola), anxiety or tiredness.

What is essential tremor? Essential tremor is also called familial essential tremor. It is different from physiological tremor described above. It usually starts in the hands and arms. It can sometimes become quite severe so that everyday activities like holding a cup can be difficult. The tremor is usually not there at rest but becomes noticeable when the affected body part is held in a position, or with movement.

The term 'essential' means that there is no associated disease that causes the tremor.

What causes essential tremor?
Essential tremor is known to be familial condition, meaning that it runs in families. At least 5-7 out of 10 people with essential tremor have other members of the family with the same condition. Genes are passed on to a child from each parent and determine what we look like, how our body functions and even what diseases we get. Particular genes have been shown to have certain changes present in families with essential tremor.

It is not clearly understood how this genetic change leads to essential tremor. However, it is likely that it somehow affects some parts of the brain that are responsible for controlling movement.

Who has essential tremor?Studies have shown different rates of essential tremor. Some have shown it to be present in 3 in 1,000 people, whereas other studies have shown it may affect as many as 5 in 100 people. It is equally common in men and women and is more common with increasing age. Most people who develop essential tremor are aged over 35, but it can occur in younger people.

What are the symptoms?The only symptom in essential tremor is tremor. If you have other symptoms, then you may have a different condition. (For example, tremor can be a symptom of various conditions such as Parkinson's disease. In these other conditions, tremor is just one of several other symptoms.)

In essential tremor, the tremor usually begins in one arm or hand. Within 1-2 years, the other arm is likely to be affected. Very occasionally, it may also spread to involve the legs. Three in ten people with essential tremor have a tremor of the head. The voice, jaw or face may also be involved.

At first, the tremor may not be present all the time. Eventually it will be present all the time when the affected body part is held in a position or with certain movements. It may be worse with stress, tiredness, hunger or certain emotions such as anger. Extremes in temperature may also make the tremor more severe.

You may be able to control the tremor to an extent. It may be less noticeable when you are working with the affected body part. For example, when you use your hand the tremor may ease off. It is not present when you are resting or sleeping.

Up to 7 in 10 people with essential tremor find that the tremor reduces after drinking some alcohol.

How is essential tremor diagnosed? There is no test to diagnose essential tremor. Your doctor can usually diagnose essential tremor based on your explanation of the tremor and an examination. It is important for the doctor to make sure that there are no other conditions present that are causing tremor. In some cases, this may mean that you need to have some tests to rule out other conditions. For example, blood tests or a brain scan. You may also be referred to a neurologist (a doctor with a special interest and expertise in the brain and nerves).

While the diagnosis of ET remains a visual one, certain brain scans Magnetic Resonance Imaging (MRI) and Computerized Tomography (CT) may be helpful in eliminating any other conditions which also produce tremor as a symptom. For example growths such as tumours or damage to the brain can be seen on certain brain scans. Blood samples may also be taken to rule out thyroid or copper metabolism problems, both of which can cause tremor. DATScan a diagnostic test can distinguish between ET and tremors of Parkinson's disease.

Other conditions that can cause tremor and need to be ruled out include: a side-effect from some prescribed medicines, anxiety, caffeine, some poisons, kidney and liver disease, thyroid disease, Parkinson's disease and other movement disorders.

What is the treatment for essential tremor?Essential tremor cannot be cured. Treatment reduces the severity of the tremor, sometimes greatly. There are various treatments that are used.

No treatment is an option If your tremor is mild, you may not need any treatment.

Medication There are two medicines used initially for essential tremor - propranolol and primidone. These medicines have been shown to ease the tremor in up to 8 in 10 affected people.

Propranolol - this is a medicine that is usually used in heart disease. It is in a class of medicines called beta-blockers. It has also been shown to be effective in essential tremor. This medicine should be used with care if you have a heart conduction problem or a lung disease such as asthma. The most common side-effects with propranolol are dizziness, tiredness and nausea (feeling sick).

Primidone - this is a medicine that is primarily used for epilepsy, but it also works very well in essential tremor. The most common side-effects are sleepiness, dizziness and nausea. These may improve if you continue to take this medicine.

When the diagnosis of essential tremor is made, you may be offered one of these medicines. A low dose is usually started at first, and gradually increased until your tremor is eased. If you reach the maximum dose without a satisfactory improvement, then the other medicine can be tried. If that also doesn't work, you can try them together. Other medicines can be tried if these two are not effective. A wide range of medicines have been shown to have some effect on reducing the severity of the tremor.

Surgery If medicine treatment is not effective, and the tremor is severe, then a surgical procedure may be an option. There are two main surgical procedures that may be considered - thalamotomy and thalamic deep brain stimulation. They both involve the thalamus. This is a deep part of the brain that organises messages travelling between the body and brain.

Thalamotomy - in this procedure, the thalamus on one side of the brain is destroyed. It has been shown to be very effective. It stops or greatly reduces the tremor in up to 9 out of 10 people with essential tremor. There are risks involved such as a bleed into the brain. Potential side-effects include muscle weakness, speech problems and memory loss. If the thalamus on both sides of the brain is destroyed, there is a higher chance of side-effects. This is not usually recommended.

Thalamic deep brain stimulation - this procedure involves placing an electrode (fine wire) into the thalamus on one or both sides of the brain. The electrode is connected to a device called a stimulator. The electrode and stimulator stay in the body. (The stimulator is placed under the skin at the top of the chest.) The simulator sends electrical impulses down the electrode to the thalamus. It is not known exactly why this device works. It seems to interrupt or block the nerve signals coming through the thalamus that cause the tremor. If you have this procedure, you will need to have regular reviews to make sure that the stimulator setting is correct. This aims to minimise side-effects and maximise benefit. It may produce a good response in up to 9 out of 10 affected people. Again, there is a small risk that the procedure may cause a bleed into the brain. Side-effects include loss of sensation, speech problems and weakness. These usually resolve when the stimulator settings are adjusted.

Botulinum toxin injections (Botox®)There is some evidence that Botox® injections are helpful in reducing certain tremors. Unfortunately, a Botox® injection into the arm also produces weakness of the arm. This is usually not tolerated. It is mainly useful when essential tremor affects the head and neck.

Alcohol Many people find that alcohol is helpful in reducing their tremor. It needs to be used with caution to avoid developing an alcohol problem. It is not advisable to drink more than the normal recommended amount of alcohol. That is: men should drink no more than 21 units of alcohol per week, no more than four units in any one day, and have at least two alcohol-free days a week. Women should drink no more than 14 units of alcohol per week, no more than three units in any one day, and have at least two alcohol-free days a week. Pregnant women, and women trying to become pregnant, should not drink alcohol at all. One unit is in about half a pint of normal strength beer, or two thirds of a small glass of wine, or one small pub measure of spirits.

Intermittent treatment Some people with essential tremor only wish to have treatment for specific times. For example, prior to going to a social engagement or before a particularly important meeting. In these situations a single dose of propranolol or an alcoholic drink may ease the tremor satisfactorily for the occasion.

What is the outlook? Essential tremor is called a progressive disease. This means that it tends to gets worse over time. It does not shorten expected lifespan and does not lead on to any more serious brain disorders. Some people have a mild tremor which does not affect daily life very much. If your tremor is more severe, it may significantly disrupt your ability to carry out normal activities such as drinking from a cup.

However, treatments work well to ease the severity of the tremor in most people with essential tremor.

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Life with Essential Tremors
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Essential Tremor in Childhood
Essential tremor (ET) may occur in children and has a prevalence of 4.6%-5.3% of the childhood population. There is often a family history of tremor. In my study of hereditary essential tremor 25% of people with ET developed tremor in their first decade and 60% in their second decade. No tremor related disability was present before the age of ten. However, 55% of young people with ET had disability and 25% reported social handicap by the age of 20 years old.

ET inevitably affects the hands in childhood but head tremor is relatively rare, being apparent in about 5% of children. The types of hand tremor present are:

1.    20% have kinetic tremor only (i.e. tremor present only when the hands are moving).

2.    5% have postural tremor only (i.e. tremor is visible when the hands are held outstretched).

3.    75% have both postural and kinetic tremor.

The functional impact of ET in children includes difficulty with:

1.    Holding a hot drink

2.    Drinking from a cup

3.    Using a spoon

4.    Writing

5.    Typing

6.    Drawing

7.    Using tweezers

8.    Painting nails

9.    Playing a musical instrument

The frequency (the number of tremor cycles per second, measured in Hz from the fingers) of ET in children is interesting, having a lower frequency in the 7-12 years than the 14-16 years age range (average 5.3Hz compared to 9.0Hz respectively). Curiously putting a 300 gram weight on the fingers of young (7-12 years old) children increases the tremor frequency from 5.3Hz to 8.2Hz, whilst in teenagers and adults it has no effect on tremor frequency. When electromyographic (EMG) recordings of tremor are made from children with ET, the tremor has an alternating pattern between the flexor and extensor muscles of the forearm (i.e. the tremor is caused by bursts activity in the muscles of the finger flexors, then finger extensors, then flexors then extensors and so on).

Tremor in children may be associated with dystonia (sustained muscle spasms) or tics. In addition an over-active thyroid gland may cause tremor and so a blood sample for thyroid function tests may be required.

Very little is known about the treatment of ET in childhood, although small doses of propranolol (20-60mg) have been reported to help small numbers of children. There are no proper randomized placebo controlled studies of any treatment for ET in children.

The psychology of children is a very complex field. However, one way of preparing a child with ET for the almost inevitable school playground comments, is where possible, to be open about the condition.

Conclusion Essential tremor affects about 5% of children. It predominantly involves the hands and may produce disability and handicap by the second decade. When necessary small doses of propranolol may be helpful, although there are no controlled trials to prove that it is effective for ET in children (unlike adults where there is class A evidence to support its use). A common sense open approach about the condition is probably wise.Dr Peter G Bain MA MD FRCP Reader & Consultant in Clinical Neurology Division of Neurosciences, Charing Cross Hospital, Imperial College London

What is Essential Tremor?

Tremor is an unintentional, somewhat rhythmic, muscle movement involving to-and-fro movements (oscillations) of one or more parts of the body. Essential tremor (previously called benign essential tremor) is the most common form of abnormal tremor. (In some people, tremor is a symptom of a neurological disorder or appears as a side effect of certain drugs.) Although it may be mild and nonprogressive in some people, in others the tremor is slowly progressive, starting on one side of the body but eventually affecting both sides. Hand tremor is most common but the head, arms, voice, tongue, legs, and trunk may also be involved. Hand tremor may cause problems with purposeful movements such as eating, writing, sewing, or shaving. Head tremor may be seen as a "yes-yes" or "no-no" motion. Essential tremor may be accompanied by mild gait disturbance. Heightened emotion, stress, fever, physical exhaustion, or low blood sugar may trigger tremors or increase their severity. There may be mild degeneration in the certain parts of the cerebellum in persons with essential tremor. Onset is most common after age 40, although symptoms can appear at any age. Children of a parent who has essential tremor have up to a 50 percent chance of inheriting the condition. Essential tremor is not associated with any known pathology.

Is there any treatment?

There is no definitive cure for essential tremor. Symptomatic drug therapy may include propranolol or other beta blockers and primidone, an anticonvulsant drug. Eliminating tremor "triggers" such as caffeine and other stimulants from the diet is often recommended. Physical and occupational therapy may help to reduce tremor and improve coordination and muscle control for some individuals. Deep brain stimulation uses a surgically implanted, battery-operated medical device called a neurostimulator to delivery electrical stimulation to targeted areas of the brain that control movement, temporarily blocking the nerve signals that cause tremor. Other surgical intervention is effective but may have side effects.

What is the prognosis?

Although essential tremor is not life-threatening, it can make it harder to perform daily tasks and is embarrassing to some people. Tremor frequency may decrease as the person ages, but the severity may increase, affecting the person's ability to perform certain tasks or activities of daily living. In many people the tremor may be mild throughout life.

What research is being done?

The National Institute of Neurological Disorders and Stroke, a unit of the National Institutes of Health (NIH) within the U.S. Department of Health and Human Services, is the nation's leading federal funder of research on disorders of the brain and nervous system. The NINDS sponsors research on tremor both at its facilities at the NIH and through grants to medical centers.

Scientists at the NINDS are evaluating the effectiveness of 1-octanol, a substance similar to alcohol but less intoxicating, for treating essential tremor. Results of two previous NIH studies have shown this agent to be promising as a potential new treatment.

Scientists are also studying the effectiveness of botulinum toxin as a treatment for a variety of involuntary movement disorders, including essential tremor of the hand.

Essential Tremor in Children
Essential tremor (ET) is often thought of as an adult onset condition. However, it can start in early childhood affecting about 5% of children, predominantly involving the hands and there is often a family history of tremor. There are two kinds of hand tremor which are: Kinetic tremor only (i.e. tremor present only when the hands are moving) and Postural tremor only (i.e. tremor is visible when the hands are held outstretched) with some children experiencing both. The hand tremor leads to difficulty holding drinks, eating, writing, coloring, playing a musical instrument and sports.

Very little is known about the treatment of ET in childhood but it is helpful to be open about the condition, to discuss it and to have a common sense open approach. Children will often cope better than adults until school age when often a variety of problems such as writing, school meal times and comments from peers, may cause anxiety and embarrassment.