Wrapping Your Mind Around Head Tremor

(This is an article from a past issue of Tremor Talk magazine. It’s just a sampling of the stories we include in each issue. Annual donors to the IETF receive Tremor Talk magazine in the mail three times per year.)

By Arif Dalvi, MD, MBA
Director of the Comprehensive Movement Disorders Program
Palm Beach Neuroscience Institute 

Dr. Arif DalviThe term tremor refers to an involuntary shaking of any part of the body. While tremor in the hands is most common, head tremor can also occur. In patients with essential tremor, head tremor can be an isolated symptom or may occur in combination with hand tremor. Essential tremor is by far the most common cause of head tremor. Another cause is cervical dystonia, also known as spasmodic torticollis. Head tremor may also occur in patients with Parkinson’s disease. Stroke, head injury, and multiple sclerosis are other causes of tremor but are less likely to cause head tremor.

Hyperexcitability and rhythmic activity in the circuits of the brain are believed to be the underlying mechanism for tremor. One such circuit includes three areas deep in the brain called the red nucleus, the inferior olivary nucleus (ION), and the dentate nucleus. This circuit is responsible for fine-tuning voluntary movements. Proper function prevents any undershoot or overshoot of movements. An abnormal response in this circuit, especially within the ION, can lead to tremor.

Approximately 95 percent of patients with essential tremor present with hand tremor. However, about 35 percent of patients have head tremor either by itself or in conjunction with hand tremor. Some patients also have voice tremor. Hand tremor occurs mostly with posture, such as when holding an object away from the body and against gravity. This contrasts with hand tremor in Parkinson’s disease that occurs when the hands are at rest. Muscle rigidity, slowness of movement, change in posture and gait also occur with Parkinson’s disease but are uncommon with essential tremor. A lip or chin tremor may also be seen in patients with Parkinson’s disease.

Cervical dystonia can be another cause of head tremor. Dystonia refers to a state of abnormal muscle tone leading to painful muscle spasms and abnormal posturing of a part of the body. When the muscle spasms and abnormal posture affect the neck it is referred to as cervical dystonia. Sustained abnormal posturing of the head is a hallmark of cervical dystonia. An enlargement of the neck muscles may be observed in cervical dystonia but is unusual in essential tremor.

Other features include an asymmetric elevation of the shoulders, excessive eye blinking or blepharospasm, and spasms of the facial muscles. Like ET, cervical dystonia can spread to one or the other arm, in long-standing cases. However, unlike essential tremor the head tremor from cervical dystonia may be associated with neck pain due to dystonic spasms.

Patients with cervical dystonia may employ sensory tricks to reduce the severity of the tremor. Touching the cheek or chin (a geste antagoniste) is a commonly employed sensory trick. Head tremor with cervical dystonia has a directional component and is usually worse when looking in one direction and reduced when looking in the opposite direction. It may be possible when examining the individual to find a head position where the tremor almost disappears. This position is referred to as a “null point”.

The diagnosis of tremor remains a clinical diagnosis. An MRI or CT scan of the brain is usually ordered to rule out structural lesions such as stroke, multiple sclerosis or a midbrain tumor. In patients where there is a question of whether the problem is essential tremor or parkinsonism, a DaTscan may be ordered. This scan is targeted towards the dopamine transporter (DaT) in the brain which is deficient in parkinsonism but normal in essential tremor. Blood tests to rule out hyperthyroidism and, in younger patients, screening tests for Wilson’s disease may also be considered.

The treatment of tremor is guided by the underlying cause. Propranolol and primidone are the mainstay of treatment for essential tremor. Other medication options that are helpful include gabapentin and topiramate. Cervical dystonia may respond to treatment with benzodiazepines. Clonazepam, which is a long-acting benzodiazepine, may be preferred in comparison to shorter acting drugs such as alprazolam or lorazepam. Baclofen can reduce dystonia by acting on GABAB receptors. Tizanidine is an alternative to baclofen. However, since tizanidine can cause liver damage (in rare cases), monitoring of liver enzymes for the first six months is recommended.

Botulinum toxins can play a role in the treatment of head tremor, particularly in dystonic head tremor. Botulinum toxins block the release of neurotransmitters. This results in decreased transmission of the signal from nerve ending to the muscle, thus reducing the tremor. Repeat injections are required every three to four months.

Deep brain stimulation (DBS) surgery was approved by the FDA in 1997 for the treatment of tremor. However, the target in the brain varies based on the condition being treated. DBS surgery carries an approximately two percent risk of bleeding in the brain, hence it is only offered to patients with advanced tremor that is disabling and not controlled by medications. Head tremor can be more difficult to control than hand tremor and may require DBS surgery to be done on both sides of the brain.

Non-pharmacological methods to reduce head tremor rarely provide sustained benefit. Physical therapy is generally not useful, however, relaxation techniques can help reduce tremor as anxiety is often an exacerbating factor. There is no specific diet that is helpful but reducing caffeine intake can help reduce tremor.

Not every person with ET will be affected by head tremor. But if you are, it is important to talk to your physician so you understand what it is and what treatment options are best for you.

 

 

 

Surgical Treatment of Essential Tremor

(This is an article that Dr. Arif Dalvi wrote for our May issue of Tremor Talk magazine. It’s just a sampling of the stories we include in each issue. Annual donors to the IETF receive Tremor Talk magazine in the mail three times per year.)

By Arif Dalvi, MD, MBA
Director of the Comprehensive Movement Disorders Program
Palm Beach Neuroscience Institute 

Background
Many patients with essential tremor (ET) get relief with medications. However, some patients, despite trying multiple medications, have a disabling tremor affecting activities such as eating, writing or using tools. Severe tremor also leads to social embarrassment and isolation. Surgical options can significantly improve quality of life in such patients.

Surgical treatment for ET goes back many decades. Abnormal circuits in a deep brain structure called the thalamus misfire sending signals to the muscles causing a tremor. In the 1970s, Irving Cooper, a neurosurgeon from Columbia University in New York, introduced the idea of making a lesion (similar to a small stroke) in the thalamus to suppress these tremor circuits. However, long term experience shows tremor relief from this method called thalamotomy may wear off in a few years. Patients with tremor in both hands need a thalamotomy on both sides of the brain, leading to higher risk of complications including difficulty with speech compared with a lesion only on one side.

The Birth of DBS
To find the best target the patient undergoes brain mapping while awake. The area within the thalamus is given a test dose of electrical stimulation to see if the tremor subsides. Alim Benabid, a neurosurgeon from Grenoble in France, realized stimulation on a constant basis could provide long-term control of tremor. He developed a brain pacemaker connected to a wire in the brain targeting the thalamus and the idea of deep brain stimulation (DBS) was born. This is the most established surgical technique for control of tremor. DBS was approved by the FDA in 1997 for ET and is covered by Medicare and many private insurers for appropriate patients.

DBS has the advantage of not requiring a stroke-like lesion in the brain. Unlike with a misplaced thalamotomy, side effects can usually be reversed by turning the pacemaker off. Both sides of the brain can be targeted without inducing the kind of complications seen when thalamotomy is done on both sides. DBS settings can be gradually increased over the years if the tremor gets worse. The battery for the DBS pacemaker requires replacement every three to five years. It must be kept in mind that there is approximately a two percent risk of a brain bleed with initial electrode placement.

DBS results depend on accurate placement of the electrode. New types of electrodes allow electrical stimulus to be directed in different directions. These directional electrodes allow for good tremor control while minimizing side effects even without perfect placement. DBS technology continues to improve with directional electrodes, smaller and longer lasting pacemakers, and rechargeable batteries being some of the innovations.

MRI-Focused Ultrasound
MRI-Focused Ultrasound (MRI-FUS) is the most recent surgical option. High energy ultrasound waves are targeted to the thalamus with high-quality MRI imaging. The ultrasound beam makes a lesion like a thalamotomy. The procedure is done on an awake patient in an MRI suite. A lighter test dose is applied to see if tremor improves. If there are no side effects, a full intensity dose is applied. MRI-FUS does not require a burr hole in the skull or electrodes and pacemakers within the body. In this sense, it is “noninvasive,” but a misplaced lesion can still result in permanent side effects. Small numbers of patients with ET have undergone this procedure, usually with favorable results. How these patients will fare in the longer term remains to be seen.

Surgical option choices for severe tremor should be made under the guidance of a movement disorders neurologist highly experienced with these procedures.

 

ET Has Affected 5 Generations in My Family

By Shari Finsilver,
VP of the IETF Board of Directors

Increasing awareness about essential tremor (ET) has been my passion for almost 20 years.  But, I must confess … I was not very open about my tremors prior to this. In fact, I did everything imaginable to hide them, even from my parents!

My courage came from all the members of my support group. It is often said that when we volunteer, we get much more back than we give. Well, that was so true in my case when I decided to start a support group in Michigan in 2000. I marveled at the candor, courage, and self-pride in many of those members and, eventually, decided to follow their lead to become more open about my own ET.

Joining the IETF Board
When I was invited to join the IETF Board of Directors in 2001, I found another platform that I could use to further raise awareness, while also getting involved in education programs, fundraising, and research. I am committed to expanding the work of the IETF, the most recognized organization helping ET patients and families.

ET began challenging my life at 11 years old. I’ve heard it said that those of us with ET are much more intelligent and creative. Well, I’m not sure I would go so far as to say that … but I did become very creative in developing ways to cope with these troubling tremors! When the IETF compiled a list of coping tips, I was able to share all of mine with all of you.

The Choice to Have DBS Surgery
After having lived with increasingly challenging ET for almost 40 years, I decided to have deep-brain stimulation (DBS) surgery in 1999. DBS changed my life.

ET has, so far as I know, affected five generations in my family. I will continue to do everything in my power to help those of us with ET get more effective medical treatments, increase awareness so the public doesn’t misjudge and label us, better educate the medical community, and fund research that will discover a cure.

Join Us to Help Raise ET Awareness
I hope I can count on each of you to do something that helps the ET community. So many great ideas are listed on the IETF website. If all of us raise our voices about ET, we can educate the world.

 

 

New, exciting changes for DBS patients

Activa PC+SResearchers at the University of Washington are developing a new device to help monitor and record tremor changes in Deep Brain Stimulation (DBS) patients. With the new Activa PC+S device, developed by Medtronic, stimulation can be turned on and off, leading to battery conservation of the device — an issue for current DBS technology. A person with essential tremor could detect their tremor and adjust the stimulation within clinician set limits.

The Medtronic Activa PC+S sits in the chest with electrodes, electrical conductors that make contact with a nonmetallic part of the circuit, wired into the brain. The electrode doesn’t have to be used to stimulate but since it is present, it can still be used to record tremor to later analyze for adjustments, if needed.

Researchers are also developing an Android smartwatch app to communicate with the piece of hardware from smartphones and smartwatches. When the patient senses the tremor, they can enable stimulation and modify within parameters in real time without a computer or without visiting their physician.

This device is not yet FDA-approved and still in early research stages. For more information visit, The Daily.

Choosing the Right Doctor: IETF ET Specialist Directory

Selecting the right doctor for you isn’t always easy, and it shouldn’t be. When you put your life and health in someone else’s care, you should feel confident that this individual has the right blend of qualifications, skills and personality traits to give you the care you need. But shopping for a doctor can be overwhelming. The International Essential Tremor Foundation can help make the process easier. The IETF has an international directory of movement disorders neurologists and ET specialists who are credible, professional physicians approved by the IETF Medical Advisory Board.

Arshia Sadreddin webA highlighted example of an expert physician found through the directory is Dr. Arshia Sadreddin. Dr. Sadreddin recently relocated her practice from Barrow Neurological Institute in Phoenix to California Pacific Medical Center in San Francisco. Dr. Sadreddin is a board certified neurologist who completed her medical education at Ross University School of Medicine and her internship, residency and fellowship at St. Joseph’s Hospital & Medical Center in Phoenix, AZ. She is a member of the IETF Medical Advisory Board. Dr. Sadreddin specializes in the treatment of patients with Parkinson’s disease, Huntington’s Disease, various tremors including essential tremor, dystonia, and facial spasms. Her philosophy of care is proactivity and partnership in care through human kindness.

To find a highly-skilled ET specialist like Dr. Sadreddin in your community, visit the IETF website. Make your association with your doctor a confident and comfortable relationship worth the extra effort.

Contact Info:
Arshia Sadreddin, MD
Movement Disorders Specialist
California Pacific Medical Center-Pacific campus
2100 Webster St. Suite 115
San Francisco, CA 94115
Telephone: (415) 600-7886

Researchers prove resistance training benefits dexterity in ET patients

hand_weights_on_workout_matA recent IETF-funded study shows resistance training to be a possible therapy for individuals with ET. A team of researchers from Griffith University and Bond University in Australia identified that a generalized resistance training program for the upper limb is capable of improving manual dexterity in individuals with ET, and to a lesser degree, reduce abduction force tremor.

“Given that resistance training (RT) can reduce tremor amplitude and improve upper limb fine motor control in older adults, it is surprising that few studies have explored RT as a therapy for older adults with ET,” said Dr. Justin Keogh, Faculty of Health Sciences and Medicine of Bond University.

The lack of existing research inspired Keogh and his research team to compare healthy, older adults living with ET to those without ET through function tests. The function tests were used to assess activities common to everyday life. After a six-week resistance training program involving dumbbell bicep curls, wrist flexion and wrist extension exercises, functions test results significantly improved.

Results show that a simple dumbbell-based resistance training program had many significant benefits for older adults, with and without essential tremor. This indicated that both groups of older adults can significantly improve many real-world measures of manual dexterity. The greatest benefits following resistance training were gained for the limb most affected due to the disorder. This study is great news for individuals with ET to further explore the use resistance training as a viable therapy for improving upper
limb-function and ultimately, improving their quality of life.

To learn more about other IETF-funded research, please visit: http://essentialtremor.org/research/ietf-funded-research/.

Study seeks DBS advancement

DBSDeep Brain Stimulation (DBS) has been around for many years and is one of the most common surgical options for the treatment of essential tremor.  Recently, a new system has been developed that takes DBS to the next level. The new device actually senses and records the brain signals that cause the symptoms of essential tremor and other movement disorders, allowing researchers the opportunity to see exactly what signals are related to abnormal movements.

Although approved for use in the European Union in January, Medtronic’s Activa PC+S system has not been approved by the Food and Drug Administration (FDA) for use in the United States. However, the new device is currently cleared for study in the U.S. and two patients with advanced Parkinson’s disease have already undergone the surgical implantation of the new device.

The hope is that in the near future, this technology will develop to a level where the device itself will monitor the patient’s brain activity and automatically adjust therapy based on the individual’s needs– just as a pacemaker does for heart patients today. This would be a big advancement in DBS if this technology can be developed. Instead of DBS sending a constant, unchanging signal to cancel out tremor symptoms, the device itself would automatically make adjustments and changes to offer patients optimum benefit.

Read more about this study here or learn more about surgical options for essential tremor in this webinar.

DARPA Focuses on DBS

DBS

Deep brain stimulation (DBS) is a surgical treatment involving the implantation of a medical device called a brain pacemaker, which sends electrical impulses to specific parts of the brain.

The Defense Advanced Research Projects Agency (DARPA) is the agency of the United States Department of Defense responsible for the development of new, advanced technologies in order to maintain the technological superiority of the U.S. military. DARPA recently announced that it will commit $70 million over the next five years to the Brain Research through Advancing Innovative Neurotechnologies (BRAIN) initiative. More specifically, to further investigate Deep Brain Stimulation (DBS).

Deep brain stimulation (DBS) surgery is an FDA-approved treatment that has been proven to significantly reduce the tremor associated with ET. In DBS surgery, a wire (electrode or lead) is placed in the ventral intermediate nucleus (VIM) nucleus of the thalamus, located deep in the brain. The wire connects under the skin to a pacemaker-like device in the chest, which provides mild electrical currents to control symptoms. In ET, DBS of the VIM nucleus of the thalamus is the most commonly used surgical procedure to control tremor.

Advances in technology have now opened up this option for other complex conditions such as depression, which is precisely why DARPA is so interested in the technology.  According to the U.S. Department of Veterans Affairs, 10% to 18% of Operation Enduring Freedom/Operation Iraqi Freedom (OEF/OIF) troops are likely to have PTSD after they return from service.  That is a significant number of men and women who may require treatment.

So what does this initiative mean for those affected by essential tremor? DARPA would like to see DBS go further than just treat symptoms. “There is no technology that can acquire signals that can tell them precisely what is going on with the brain,” says Justin Sanchez, DARPA program manager, to The New York Times. He explained that DARPA is “trying to change the game on how we approach these problems.”

DARPA hopes to develop DBS to the point that the device will be able to monitor brain signals in real time, treat illness accordingly and measure the success of that treatment. This would be a real games changer for ET patients suffering from severe tremor symptoms. Imagine never having to turn the DBS device on or off, or have it calibrated. It would be programed to know exactly how to manage individual tremor symptoms, then evaluate the results and make adjustments accordingly.

Only time will tell if DARPA is successful with this ambitious project. But even if all their goals are not realized, they are bound to discover an abundance of new information about how the brain works.

Learn more about BRAIN.

Phase III of Focused Ultrasound Trial Begins

 

Dr. W. Jeffrey Elias photo

Dr. Jeffrey Elias and the ExAblate

The first patient has been treated as part of a Phase III trial evaluating the success and safety of treatment using the ExAblate Neuro on essential tremor patients. The study builds on promising pilot studies demonstrating the preliminary safety and effectiveness of MR guided focused ultrasound technology. Read about Phase I of the trial here.

The results of this trial are expected to support a submission of the ExAblate Neuro to the FDA for Pre-Market Approval.

InSightec, makers of the ExAblate Neuro, will be partnering with BIRD (US-Israel Binational Industry R&D) and the Focused Ultrasound Foundation for this trial.

Find information on registering for this and other essential tremor studies at clinicaltrials.gov.

Results From Focused Ultrasound Study

 

ultrasoundstudy_patients

Dr. Jeff Elias (center) and the patients who participated in the essential tremor study at UVA

The New England Journal of Medicine published the results of the pilot trial for the use of focused ultrasound to treat patients with essential tremor. These Phase I results indicate that focused ultrasound can safely and effectively treat targeted areas deep in the brain.  In focused ultrasound, more than 1,000 ultrasound waves are focused to a single site in the thalamus for the treatment.

The study included 15 patients with essential tremor that could not be managed by medication. Jeffrey Elias, MD, neurosurgeon at the University of Virginia and IETF Medical Advisory Board member, is the lead investigator of the study.

Phase I findings:

  • Dominant hand tremor improved by 75 percent.
  • Substantial improvements in daily disabilities (85 percent) and quality of life as assessed by clinicians and patients.
  • Outcomes and complications were comparable to surgical procedures for tremor, including radio frequency thalamotomy and deep brain stimulation.

Phase III of this study will begin soon. For information on how to register, visit clinicaltrials.gov.

The IETF will continue to watch as results of focused ultrasound studies are posted. Large, randomized controlled trials will be required to assess the procedure’s efficacy and safety.

Watch a video featuring Billy Williams, the first patient treated with focused ultrasound for essential tremor.