Lois Pope LIFE Center
Miami, Florida 33136
Toll Free 800-996-3783
Tel: 305-243-6946
Fax: 305-243-3337
Movement Disorders
Parkinson’s Disease:
Between 1-1 ½ million Americans are affected by Parkinson's disease, a disorder of the central nervous system. "Parkinson's" disease usually affects people age 50 or older but about 10-20% of people with Parkinson's are diagnosed before the age of 50. The four primary symptoms include rigidity of the limbs, tremors, slow movement (bradykinesia) and postural instability (which is perceived as a loss of balance and unsteadiness sometimes with falls). There is no definitive lab test and the diagnosis is made by a neurologist who evaluates the patient.
What causes this disease?
People with Parkinson's disease have a shortage of a chemical that affects movement (called dopamine) in their brains. This happens because the cells that make dopamine in an area called the substantia nigra are no longer functioning properly. We do not know exactly how these changes happen. Many theories include accelerated aging and cell death, a genetic susceptibility, and environmental factors, among others. It is probable that the disease is a combination of many things.
Medical Treatment
Standard treatment of Parkinson's disease in the 21st century is with medications. These medications attempt to replace the missing dopamine in the brain (Sinemet®), or by drugs that seem to affect the use of dopamine in the brain (Symmetrel®, Eldepryl®). Newer drugs, called dopamine agonists (Parlodel®, Permax®, Mirapex®, Requip®), make the dopamine-sensitive brain cells work better. Yet newer drugs are under development.
Surgical treatments
One surgical technique for treating Parkinson’s disease is thalamotomy and pallidotomy, which destroy the "trouble-causing" cells in the brain using an electrode. Newer techniques for this are available. Another exciting new technique is high-frequency stimulation of a part of the brain called the subthalamic nucleus. This is known as Deep Brain Stimulation (DBS). An experimental surgical technique to treat Parkinson’s disease is brain tissue transplants using genetically-altered human cells, human fetal tissue, human stem cells, or pig cells induced to produce dopamine.
The setting of the DBS neurostimulator is easily adjustable by an external magnetic control —without further surgery—if the patient’s condition changes. Unlike previous surgeries for Parkinson’s disease DBS does not damage healthy brain tissue by destroying nerve cells. DBS blocks electrical signals from targeted areas in the brain. Thus, if newer, more promising treatments develop in the future, the fine DBS electrodes can be removed without resulting injury to the brain.
Do I need medicines after DBS surgery for Parkinson’s disease?
Although most patients still need to take medication after undergoing DBS, many patients experience considerable reduction of their Parkinson’s disease symptoms and a some patients are able to reduce the dose of medications. The reduction in dose of medication leads to a significant improvement in side effects such as dyskinesias (involuntary movements caused by long-term use of levodopa). In some cases, the stimulation itself can suppress dyskinesias without a reduction in medication.
Essential Tremor
Essential Tremor (ET) is not a life-threatening disease. It is sometimes called benign essential tremor for that reason, but it can be a life-altering condition. People with ET often report changes to their lives because of the disease. Many activities might be disabled as a result of the tremors.
Embarrassment is a major problem because people stop eating in restaurants, going to social or family functions, shopping and pursuing hobbies alone or with friends. The resulting isolation may cause problems and strain family relationships.
Some people with ET no longer drive—which can be a severe hardship in the U.S.A. People with ET may be forced to leave their jobs if the tremors disable them or they may not accept promotions that would entail more personal contact.
ET’s effects extend to the most ordinary daily tasks: women may have difficulty applying makeup and men may have difficulty shaving. With ET, simple tasks become daily struggles and if this happens then treatment is merited.
Dystonia
Causes:
Dystonia affects people of all ages and ethnic backgrounds. Some forms of it can be inherited; researchers have identified more than 10 genes or chromosome locations associated with the disorder. It produces painful, prolonged muscle contractions that can deform a limb or the spine.
What is affected by dystonia?
Muscle spasms can affect any part of the body including eyelids (blepharospasm), face (oromandibular), vocal cords (dysphonia) and/or arms, legs and trunk. "Writer's cramp" is a dystonia in the hand or arm muscles.
Dystonia may also occur due to birth injury, traumatic injury, toxins (such as manganese), certain medications (such as haloperidol), stroke, or be part of another disorder such as Parkinson’s disease. It develops gradually, and symptoms can appear at any age. There is no specific test for dystonia, so it is diagnosed by a neurologist based on the symptoms and signs of the disease.
Dystonia can come and go, so some people who experience it may have a hard time convincing health care providers that it exists. The spasms may change significantly with different actions. For example, hand dystonia may occur when trying to write but not during other activities. It does not shorten life but like essential tremor is has life-altering effects including pain and discomfort. In severe cases it may interfere with basic activities such as walking, dressing, or eating.
There are a number of medications that can treat dystonia, either alone or in combination. One of the most common treatments is Botox (botulinum toxin) injections. Another formulation of botulinum toxin used is called Myobloc® (U.S.) or Neurobloc® (Europe).
Botox injections
Although most people in the U.S. know Botox as a cosmetic treatment for facial lines, it was originally developed in 1989 as an orphan drug to treat strabismus (eye squints), and was later used for dystonia and muscle spasms. Injection of the drug causes weakness in the targeted muscle, thus relaxing the spasm and relieving the dystonia.
SURGERY:
Deep Brain Stimulation (DBS): Pacemakers for the Brain
What is Deep Brain Stimulation?
Deep Brain Stimulation (DBS) is a surgical option for patients with Parkinson’s disease (PD), Essential Tremor (ET) or dystonia. During DBS surgery electrodes are implanted within the brain to deliver electrical impulses. The stimulation offers patients relief from the tremors, rigidity, slowness of movement, stiffness and may help balance problems associated with their conditions. What makes this treatment so unique is that stimulation can be and usually is adjusted as a patient’s condition changes over time.
Deep brain stimulation is a new and improved variation of an old surgery. The old surgery involved destroying small parts of the brain within structures called the thalamus or globus pallidus with heat. Today, it is no longer necessary to destroy even small parts of the brain.
How does Deep Brain Stimulation work?
An electrode implanted in the brain emits pulses of energy to block the abnormal activity in the brain neurons which causes the symptoms. The success of deep brain stimulation surgery is directly related to finding the specific area in the brain for stimulation. See surgical technique below.
Who is a candidate for this surgery and when is it right for me?
• Any patient who is dissatisfied with his/her increasing loss of control of movement
• ANy patient who exhibits symptoms causing a decline in the quality of life
• Has had an adequate and reasonable trial of medications
How is Deep Brain Stimulation surgery done?
It is important to note that surgical techniques can vary among centers and surgeons. What follows is the technique used here at the University of Miami Miller School of Medicine.
Deep brain stimulation involves the implantation of a very thin lead containing four electrode contacts into a specific target area in the brain. The lead extends through a small opening in the skull below the skin and is connected to an extension wire with a connection behind the ear. The extension wire is connected to an impulse generator or “pacemaker” that is implanted under the skin just below the collarbone. Programming of the stimulation is easy and painless using an external magnetic control box.
The surgeon is aided by computerized brain-mapping technology to find the precise location in the brain where nerve signals generate the tremors and other symptoms. Highly sophisticated imaging and microelectrode recording equipment are used to map both the physical structure and the functioning of the brain by the surgeon and neurologist in the operating room.
At the Miller School of Medicine the procedure starts with an MRI image of the brain. On the day of the procedure, the patient undergoes placement of a temporary head frame. A CT scan of the brain is then obtained with the frame in place. The MRI and CT of the brain are loaded into a computer which fuses them into one picture. Accuracy is of paramount importance and this fusion along with microelectrode recordings of the brain neurons permits pinpoint accuracy. This is not performed routinely at all institutions in the United States.
After careful targeting of the pertinent area of the brain is achieved, the surgery begins. This consists of four portions. A local anesthetic is administered and the surgeon makes the small opening in the skull.
Then comes the placement of a microelectrode allowing the surgeon and the neurologist to confirm that the target chosen on the preoperative imaging studies is accurate. Once accuracy is confirmed, the next step is placement of the actual stimulator electrode. After the stimulator is in place, the patient is examined by the neurologist. This examination ensures that under stimulation the patient’s symptoms are improved and that side effects do not occur. The final part of the procedure is performed with the patient fully asleep. This is the placement of the programmable generator under the skin of the chest wall under the collarbone. The entire procedure takes approximately 2 ½ -3 hours. On average, the hospital stay is 24 hours.
The patient is awake during surgery to allow the neurologist to assess the patient’s clinical status along with the detailed brain recording. While the electrode is being advanced through the brain, the patient does not feel any pain because of the unique nature of the human brain and its inability to generate pain signals.
The stimulators are turned on for the first time three weeks after implantation.
How effective is Deep Brain Stimulation?
In properly selected patients, deep brain stimulation is remarkably safe and effective, although the surgery is not completely without risk. Beneficial effects have been demonstrated to last for several years. DBS in patients who initially responded well to medications, but over time have developed side effects, can experience between 50 to 80 percent improvement in such symptoms as tremor and slowness of movement with the stimulator. Patients on average report a 50 percent improvement in their walking. Similarly, patients with involuntary movements (dyskinesia) due to their medications, experience over 80 percent reduction in their severity. Some patients may be able to reduce their medications following deep brain stimulation.
Regarding Parkinson’s disease in particular, an important indicator of the effectiveness of any treatment is the duration of “on-time” without dyskinesia. This means the patient is mobile and can perform everyday tasks without experiencing the involuntary movements.
What risks are associated with Deep Brain Stimulation?
This is brain surgery and is not without risk. There is approximately a two to three percent chance of brain hemorrhage that may be of no significance, but may rarely cause paralysis, stroke, speech impairment or other major problems. This means that for every 100 patients who undergo surgery, two or three will experience a permanent or severe complication. However, this also means that most patients will have no complications. There is a 15 percent chance of a minor or temporary problem. Infections can occur. While treatment of infection may require removal of the electrode, the infections themselves have not caused lasting damage and are treated with antibiotics.
Who can be evaluated for Deep Brain Stimulation?
Patients with Parkinson’s disease, essential tremor, dystonia or tremor due to multiple sclerosis, with movement-related symptoms that cannot be controlled by medications can be evaluated as possible candidates for deep brain stimulation. In addition, patients who experience intolerable side effects from medication may also be candidates.
Deep brain stimulation has been successful in treating patients as young as 13 years of age. In general, surgery is performed on those under 75 years of age but we have implanted patients as old as 84 years of age. Each patient must be assessed individually concerning his or her stamina and overall health.
Has Deep Brain Stimulation been approved by the Food and Drug Administration?
Yes. In January of 2002 the FDA approved deep brain stimulation for the treatment of Parkinson’s disease. It had previously been approved for the treatment of Essential Tremor in 1997.
What happens after Deep Brain Stimulation surgery?
A series of adjustments in the electrical pulse will be made over weeks or months. It is necessary for patients to be able to travel to a location where the stimulation of their implanted pacemakers can be adjusted. The first few follow-up visits should be to the center where the surgery was performed, but subsequent electrical programming can take place at another medical center that has the necessary equipment and expertize.
Should I have Deep Brain Stimulation surgery or wait for Stem Cells?
As research progresses in the area of movement disorders and particularly Parkinson’s disease further treatment options may become available. One area of interest is that of stem cell transplantation. Although stem cell therapy is not available now, researchers are feverishly working to make this option a reality. This procedure would consist of implanting stem cells instead of a stimulator using the same techniques described above. These cells would eventually differentiate into neurons that would take the place of those missing in Parkinson’s disease, thus producing dopamine and reversing the further progression and symptoms of Parkinson’s disease. Due to the fact that deep brain stimulation is not destroying brain cells it most likely would not prevent you from future surgical therapeutic options.
The UM Team:
Jonathan Jagid, MD is an Assistant Professor of Neurological Surgery and a Board Certified Neurosurgeon at the University of Miami Miller School of Medicine. Dr. Jagid specializes in the field of stereotactic neurosurgery and movement disorders. He trained at the University of Miami under the mentorships of Dr. Howard Landy and Dr. James Schumacher.
Bruno V. Gallo, M.D. is an Assistant Professor of Neurology at the University of Miami Miller School of Medicine. Dr. Gallo is a specialist in movement disorders, epilepsy and migraine. He has been the principle investigator on phase I and Phase II clinical research trials and a national and international instructor for Deep Brain Stimulation programming. He has been involved in over 400 DBS implants.
Carlos Singer, M.D. is an Associate Professor of Neurology at the University of Miami Miller School of Medicine and is Director of the Movement Disorders Division. He is board certified in both Internal Medicine and Neurology. Dr. Singer has been a member of the Department of Neurology faculty since 1989.
Bonnie Levin, Ph.D. is an Associate Professor of Neurology at the University of Miami Miller School of Medicine and serves as the neuropsychologist for the Deep Brain Stimulation program at the Miller School of Medicine. She has been actively involved with DBS for many years and has reviewed many protocols involving research and DBS at a national level. She is very experienced in the screening of patients from a neuropsychological standpoint.
Appointments
For appointments or more information, call (305) 243-3245 between 9 a.m. and 5 p.m. Eastern time, Monday through Friday.
