Sarasota Neurology

Parkinson’s disease is the second most common neurodegenerative disorder, just behind Alzheimer’s disease. Parkinson’s disease is characterized by specific clinical symptoms including rigidity (stiffness), slowness of movement, unsteadiness (gait imbalance) and tremor. For the accurate diagnosis of Parkinson’s disease to be made, a patient needs to have 3 of the 4 major symptoms of the disorder. Each patient with Parkinson’s disease is different and may have differing degrees of each component of Parkinsonism. Not all patients with Parkinson’s disease have tremor. Some may have more instability of gait, shuffling or slowness of movement. There are several medications available that neurologists can use to treat Parkinson patients to alleviate their Parkinson symptoms and improve their overall quality of life. Unfortunately, there is a down side to this treatment. Patients who have been on Sinemet for a few years tend to develop motor fluctuations. Motor fluctuations include end-of-dose wearing off, where their functional abilities deteriorate before the next dose of medication is due. Other motor fluctuations include freezing and off time.
Parkinson freezing is simply when a patient becomes “stuck” meaning they cannot move. This occurs more frequently when going through doorways, stepping up onto a curb or stair or when getting up to start walking. Freezing can also occur first thing in the morning, just when getting up out of bed. Freezing episodes can last for a second up to a few minutes. It is the goal of every Parkinson’s disease neurologist to minimize a patient’s amount of freezing, through various medications and dosing schedule changes. Off time can occur in two settings: one is predictable, usually at the end of the dosing interval but the other occurs randomly, without warning. These sudden off time events are more problematic as they tend not to respond as well to medication changes. Off time is troublesome for the patient and caregiver. Affected patients become virtually immobile, essentially frozen in place. There are different degrees of off time, but in all cases, the patient’s mobility and ability to function are severely impaired. Off time may last minutes to hours. For those patients with short duration off time, additional medication or shorter dosing intervals usually will help. Off time may also occur first thing in the morning when waking up. Even if Parkinson patients take their medications, it may be an hour or more before they are functioning normally. For patients with prolonged off times, usually greater than 45 minutes, there is treatment.
Apokyn (apomorphine) is a self administered injectable medication that rapidly relieves off time. Its duration of action is generally less than 2 hours. This is an ideal medication for patients with one or multiple daily freezing episodes. For those affected patients, Apokyn can literally give them their lives back, particularly when more waking hours are spent in the “off time” than in “on time.” For a patient or caregiver to administer Apokyn, some training is required. This is covered by the drug manufacturer and by Medicare. Side effects can include a drop in blood pressure, lightheadedness, nausea or vomiting. When initially starting a patient on Apokyn, medication to prevent nausea is given first. After being on the Apokyn for a few weeks, patients frequently can stop the antinausea medication.
If you are a patient or caregiver and feel that Apokyn may be of benefit, contact your neurologist or Parkinson disease specialist for more information. An excellent information package, with DVD, is available at no cost. The first step is to make the call to improve your quality of life.  For more information, visit the website for Dr. Kassicieh at: www.DrKassicieh.com.

Recent studies have suggested that qualifying Parkinson patients benefit from earlier treatment with deep brain stimulation, as reported in Clinical Neurology News. The study indicates that younger Parkinson disease patients are more likely to benefit from early brain stimulator treatment. There is information that may suggest that this therapy may have a protective effect in delaying the progression of Parkinson’s disease. Deep brain stimulation (DBS) was FDA approved in 2002 for treatment of Parkinson’s disease. Symptoms that are best controlled include tremor and dyskinesias although brain stimulation can also help reduce freezing and off time. Younger Parkinson patients develop motor complications such as dyskinesias, off time and freezing much earlier than older patients with Parkinson’s disease. As reported by Dr. David Charles, a Vanderbilt University Medical Center Parkinson neurologist, “No therapy…has bee shown to slow the progression of Parkinson’s.” The previous thinking was to wait until a patient had severe motor complications that could not be controlled with medications prior to considering DBS therapy. The new thinking, and research, is exploring benefits of DBS in earlier stages of Parkinson’s disease. In various reported cases, patients not only benefited from better control of their Parkinson motor symptoms but also had improved quality of life. Added advantages is that Parkinson patients treated earlier with DBS used less medications over an 18 month period, as shown in one small study. There are two studies currently looking at the benefits of early DBS therapy in Parkinson patients: EARLYSTIM is a French study and a smaller study at Vanderbilt University are in progress. It should be noted that Parkinson’s disease is a progressive neurodegenerative disorder. Even patients with DBS therapy do have progression of their symptoms. Memory loss can be a part of the Parkinson syndrome and is not helped by DBS therapy. DBS is not a substitute for optimal neurological and medication management of Parkinson symptoms. Dr. Kassicieh, at Sarasota Neurology, provides medical and neurological management for patients with Parkinson’s disease and brain stimulators. For more information click here.

Neupro patches were approved by the FDA for Parkinson’s disease treatment in September 2007. They proved to be very effective in the control of Parkinson symptoms, as compared to the effects of other dopamine agonists including Mirapex and Requip. Unfortunately, in March, the FDA recalled Neupro due to problem with the patch delivery of the medications. What they found posed no imminent danger to patients. Rather what was happening was that the active drug, rotigotine, was crystallizing in the patch therefore not delivering the full dosage of medication to the patient. What would happen is that affected patients’ Parkinson symptoms would not be as well controlled. It is not clear if Neupro patches will be brought back to market as reported on Emaxhealth.

Dopamine agonists remain one of the main Parkinson treatment medication groups available to control Parkinson symptoms. These can be used as first line medications for early Parkinson’s disease, showing as good as an effect as Sinemet - the gold standard for treatment of Parkinson’s disease. Many feel that it is beneficial and studies have shown that starting early treatment with dopamine agonists can limit the long term side effects of starting Sinemet early. This is particularly true for delaying development of dyskinesia, which are involuntary movements of arms, legs and head. Dopamine agonists can also help to suppress tremor associated with Parkinson disease.

If you are still using Neupro patches, you should contact your treating neurologist or Parkinson specialist to get the weaning patches and titrate off this drug. Many other excellent treatments for Parkinson’s disease are available. For more information visit: Parkinson Doctor.

Normal pressure hydrocephalus (NPH) is a rare disorder that is characterized by progressive gait difficulty, urinary incontinence and memory loss. Although the press has covered this topic extensively in both the written and video media, true normal pressure hydrocephalus remains quite uncommon. The underlying problem is actually an excessive build up of spinal fluid in the brain. The areas of the brain that stores this fluid are known as the ventricles. In NPH, the spinal fluid flows out of the brain but, due to reasons that are not entirely clear, there is a build up of excessive fluid in the brain. This results in enlarged ventricles causing a condition called communicating hydrocephalus.

Normal pressure hydrocephalus develops very slowly, over months to years. It is usually seen in individuals over the age of 65. As the ventricles slowly increase in size, affected patients begin to show signs of slowed, wide based, unsteady gait. Urinary incontinence may also develop during this time. Later during the disease process memory loss begins. All of the symptoms are very slowly progressive. Patients can be diagnosed incorrectly with Parkinson’s disease, Alzheimer’s disease, depression or just dementia.

The gait difficulty comes from the fact the the nerve fibers that control walking and balance become stretched as the ventricles enlarge. With this comes progressively worsening gait imbalance and falling. Patients may complain of weakness and fatigue. Patients will actually will tell you that their feet feel stuck to the ground, giving rise to the term magnetic gait. Memory loss seen in normal pressure involves mainly recall and slowness of thinking. Recognition of objects, tasks and individuals is better preserved. Without careful testing however, one can easily make the mistake of making an erroneous diagnosis of Alzheimer’s disease versus normal pressure hydrocephalus associated dementia. Urinary incontinence is a later finding in the disease process. There is an increasing need to urinate more frequently and urgently. If the dementia progresses too far, patients will become indifferent to their incontinence.

Diagnosis is made by obtaining an MRI or CT brain scan. The ventricles appear enlarged in the absence of brain atrophy (shrinkage.) As a normal process of aging, there is a certain amount of atrophy. It other conditions such as Alzheimer’s disease, alcoholism or in patient’s who have received chemotherapy, brain atrophy can be more prominent. The key in diagnosing NPH is that the degree of ventricular enlargement is out of proportion to the expected degree of atrophy. The degree of ventricular enlargement can be measured as a ratio to the degree of atrophy. The second step in diagnosis, after a complete neurological exam, is to do a diagnostic spinal tap (lumbar puncture.) During this procedure, 1-2 ounces of spinal fluid is drained off. The patient is then tested to see if their gait improves.

Treatment for confirmed cases of normal pressure hydrocephalus is a brain surgery procedure know as a ventriculoperitoneal shunt placement. In this procedure, a tube is placed in the ventricles and the other end drains into the abdomen. The tube is run under the skin. Spinal fluid is then absorbed in the abdomen. There is no known effective medical treatment for NPH. Early diagnosis and treatment is important as the gait disorder and urinary symptoms can be alleviated. Once the memory loss has begun, this cannot be reversed.

In order to have the accurate diagnosis of normal pressure hydrocephalus, a patient should be seen by a neurologist or neurosurgeon familiar with the condition. It is not necessarily easily diagnosed, even by experienced physicians. Nonspecific gait disorder is common with advancing age. Dementia is also common, particularly over the age of 70. Stroke, Parkinson’s disease and low thyroid can mimic the symptoms of normal pressure hydrocephalus. The main point is that of all these conditions, true normal pressure hydrocephalus occurs very rarely and is generally considered a diagnosis of exclusion of every other problem plus meet the diagnostic criteria listed above.

Myasthenia gravis is a rare disorder of muscle weakness, affecting approximately 70,000 individuals in the United States. Many confuse this with multiple sclerosis. Multiple sclerosis is a central nervous system disorder affecting the insulation (myelin) on nerve cells in the brain and spinal cord. In contrast myasthenia gravis is a muscle disease where transmission of electrical impulses to the muscle fail. This results in the muscle not contracting fully, resulting in weakness. This condition can selectively affect the eye muscles, muscles of the head and neck or be generalized affecting all muscle, including the diaphragm. If the diaphragm is involved, patients can have varying degrees of breathing problems, including respiratory failure.

Myasthenia gravis (MG) is the standard model of a neurological autoimmune disorder. It is the the best understood of all autoimmune diseases. In autoimmune diseases, the body’s immune system fails to recognize a particular body system as being part of “itself.” In MG, the immune system does not recognize the transmitter receptors for acetylcholine (the muscle communication transmitter) as being part of the body’s own system. The immune system for antibodies against the receptors and attacks them. With fewer receptors on the muscle membrane, the muscle does not get the full chemical message to contract. This causes affected individuals to have weakness in the muscles affected by MG. These antibodies can be detected by a blood test for acetylcholine receptor antibodies. It is important to note, however, that not all patients with myasthenia will have a positive antibody test, known as a false negative (as high as 25%.) Particularly patients with ocular myasthenia frequently have negative tests. This does not mean that they do not have the condition.

Diagnosis of myasthenia is based on a careful medical history and detailed neurological exam. All patients with MG have some degree of visual symptoms including double vision or eyelid drooping. The presence of variable eyelid drooping, particularly if it shifts from side to side, is virtually diagnostic of myasthenia gravis. Confirmatory tests can include EMG testing and a chemical test known as the Tensilon test. Tensilon (edrophonium) is a chemical that temporarily blocks the enzyme that stops the action of acetylcholine. This has the effect of making the acetylcholine receptors that are “on” stay on longer, thereby combating weakness. Patient’s with suspected myasthenia can be given Tensilon. This short acting drug will temporarily reverse the weakness that myasthenic patients have. Other lab work should be performed to exclude other muscle diseases, thyroid problems or other metabolic problems that could cause muscle weakness.

Once the diagnosis is made, patients can be treated with Mestinon ( pyridostigmine.) Mestinon is a long acting version of Tensilon and works by inhibiting cholinesterase, the chemical that breaks down acetylcholine. If we did not have this enzyme, we could not relax our muscles. When acetylcholine is removed from the muscle receptor the muscle no longer contracts. Mestinon works well for symptomatic relief of the muscle weakness and associated symptoms of MG. Other symptoms associated with weakness can include chewing and swallowing difficulties, weakness of breathing with shortness of breath and generalized weakness. Double vision and drooping eyelids, conditions made worse by being in bright sun light, are frequent complaints.

For patients that have persistent symptoms, despite Mestinon therapy, clinicians will frequently employ the use of immunosuppressant therapy that will dampen the response of the immune system. This has the effect of limiting the destruction of the acetylcholine receptors on the muscle membrane surface. The most commonly used drug for this is Prednisone, a steroid drug, frequently used to treat autoimmune disorders. Although Prednisone works very well, it has its downside. Prednisone side effects can include weight gain, elevated blood sugar, cataracts, accelerated osteoporosis and other serious medical problems. Other immunosuppressants have been used with success. These include Imuran (azathioprine), cyclosporine, Cytoxan (cyclophosphamide) and CellCept (mycophenolate.) While being helpful in treating MG, these powerful immunosuppresants have their own lists of potential side effects. The purpose of immunosuppressant therapy is to cause myasthenia to go into remission. This is not always successful.

For patient’s with a severe worsening of their myasthenia symptoms, hospitalization may be necessary. Higher dosage, intravenous steroids can be given in combination with a blood filtering technique, known as plasmaphersis. The latter is used to filter out the acetylcholine receptor antibodies. A surgical technique, thymectomy, can be used to remove the thymus gland in the chest. This gland is responsible for making the cells that make the acetylcholine receptor antibodies.

Myasthenia gravis is a serious neurological condition and autoimmune disease characterized by muscle weakness that can affect vision, swallowing, breathing and general activities. Serious complications, including respiratory failure, can develop for untreated patients. Affected individuals should seek out care from a board certified neurologist, preferably one specializing in movement disorders or neuromuscular disease.

One of the most frustrating neurological conditions is restless legs syndrome (RLS.) It is characterized by an intense overwhelming need to move your legs at night or when sitting or resting. This can be so severe that affected individuals have difficulty sitting through a movie or driving in a car for any distance. Occasionally RLS can be associated with abnormal sensation, particularly in the feet and lower legs. The abnormal sensory symptoms are a form of peripheral neuropathy that is associated with RLS. Symptoms can be so severe as to be an impairment to sleep and feeling of well being. RLS is consider to be a form of sleep disorder, even though individuals can have symptoms during the day, while awake. New research findings that were reported in the New England Journal of Medicine indicates that there is a genetic component to RLS. There are many who doubt the actual existence of RLS but those affected with this condition would disagree. As a movement disorder specialist, having seen many patients with this condition, there is no question that it is real. To ignore the medical literature and scientific evidence on the existence of RLS is foolish.

RLS can markedly impair the quality of life of affected individuals. They typically have chronic sleep deficit with a higher incidence of depression. The recent report on the genetic linkage of families with members affected with RLS is fascinating. Another common sleep condition, periodic limb movements of sleep, shows a stronger genetic correlation to run in families due to a specific genetic sequence variant at the 6p chromosome. Many of these individuals have RLS. If one isolates out the patients with RLS only, the genetic linkage becomes less clear. It is perhaps that RLS represents a subset of individuals with some chromosomal variation. This may serve as a marker for potential development of RLS in patients who have a history of RLS or periodic limb movements of sleep. These individuals can also have trouble with elevation in their blood pressure as well as other conditions associated with long standing sleep deprivation. There are several excellent treatment options for RLS including the dopamine agonists, Klonopin, low dose mild narcotics and other medications. If you are affected with this condition, it is important to get medical help as excellent treatment exists to control symptoms.

Essential tremor is a common disorder than is characterized by involuntary shaking of the head, jaw, hands or legs. It is estimated that there are 5-10 million affected individuals in the United States. Unfortunately, many patients are incorrectly diagnosed as having Parkinson’s disease when they actually have a form of essential tremor (ET). ET tends to run in families and can have onset anywhere from 20-60. Parkinson’s disease most commonly starts after the age of 60 and is generally thought not to be hereditary. The characteristics of essential tremor and Parkinson related tremor are quite different. Typically the tremor seen in Parkinson’s disease occurs in the hands and is seen with the hand at rest. The tremor has a unique “pill-rolling” quality to it. The Parkinson tremor stops when the individual moves or uses their affected hand. Essential tremor is not present when the affected limb is at rest. When an individual moves their arm or tries to grasp or hold an object, then their tremor becomes apparent. This is most readily seen when trying to write, hold a beverage container or use eating utensils. Writing is affected in both conditions. In essential tremor, the writing becomes jerky with a zig-zag appearance to letters whereas with Parkinson patients, their writing becomes progressively smaller, trailing off into a non-legible line. It is uncommon for Parkinson patients to have head, jaw or leg tremor. These are much more commonly seen in the ET population. Patients with Parkinson’s disease have other clinical features that benign tremor patients do not have.

Treatment for patients with essential tremor (benign tremor, familial tremor) is readily available. Many patients have already discovered that small amounts of an alcoholic beverage will temporarily stop their tremor. Medications used to reduce tremor severity may include primidone, clonazepam, propanolol, mirtazepine or Topamax. All of these medications can be helpful but there is a potential for side effects, as with any treatment. For patients with severe, disabling tremor that is not responsive to medical treatment, surgical treatment with deep brain stimulation can be very effective. This procedure is highly effective in minimizing or completely controlling tremor. It has been approved for use since 1997. This procedure can dramatically improve a patient’s quality of life.
In summary, if you or one of your family is affected by obvious tremor, it is in their best interest to see a movement disorder specialist to get the correct diagnosis and treatment. The treatment and prognosis of essential tremor and Parkinson’s disease is quite different. The medications used for one will not help the other. Significant problems with unwanted side effects can develop if each condition is not treated properly.

Botox (botulinum toxin Type A) has been available in the United States for clinical use since 1989. At that time it was approved by the FDA for treatment of eye and facial muscle spasm disorders, blepharospasm and hemifacial spasm respectively. Then in 2000 the FDA approved Myobloc (botulinum toxin Type B) for treatment of cervical dystonia, a condition of involuntary neck muscle spasm. The dystonias, as a class of muscle spasm disorders, are characterized by involuntary muscle spasms involving the muscles in the neck, face and extremities. The cause of the majority of these conditions is unknown. In some individuals, spasticity (tight muscles which cannot be relaxed, a form of dystonia) can result from stroke, traumatic brain or spinal cord injury or cerebral palsy.

Prior to the use of Botox, it was very difficult to treat muscle spasm disorders. Medications had side effects and surgery had limited benefit associated with the risk of complications. Botox opened an entirely new avenue to treat spasticity. The drug works by causing a chemical relaxation of muscles that are injected. Botox is highly selective in that it remains in the muscles that it is injected into. Patients with cervical dystonia have difficulty with their head pulling to one side of the other. They may also have their head pulling backward or forward. Not only is this condition painful, it also causes patients to have functional difficulty with activities such as driving, playing sports or even eating. In patients with limb dystonia, there is involuntary spasm of an arm, leg or both. This can cause difficulty with dressing, walking or even personal hygiene (if their hand is fisted up.) Botox (or Myobloc) can provide excellent relief of these symptoms thereby improving patients’ quality of life. For patients with severe muscle spasticity from stroke, Botox provides relief of the tight muscles allowing for greater ease in certain activities. It is important to note that Botox (or Myobloc) will not restore function of any limb affected by the stroke. What the treatment will do is provide increased comfort due to reducing pain from spasm and allow for improved ease in doing some daily activities.

Blepharospasm is characterized by involuntary blinking which can result in forced eye closure and functional blindness. Affected individuals may have difficulty driving or watching a movie because of this. Botox has been shown to be the single most effective treatment for this condition. Of all the muscle conditions treated with Botox, none respond as well as those patients affected with hemifacial spasm. This condition affects one side of the face and is characterized by spasm the facial muscles on that side. This can also result in a degree of functional blindness. While most conditions treated with Botox (or Myobloc) have a therapeutic effect for 2-3 months before requiring retreatment, patients with hemifacial spasm may not need retreatment for anywhere from 3-6 months.

For any patient considering receiving Botox or Myobloc, it is important to see a physician familiar with diagnosing and treating these uncommon movement disorders. These individuals are familiar with administration of Botox or Myobloc which will help to obtain optimum results from each treatment

Restless legs syndrome is a movement disorder that affects approximately 10% of the American population. Affected individuals have the uncontrollable urge to move their legs, particularly at night when sleeping. Symptoms can also occur with sitting for prolonged periods of time such as when driving, flying or watching a movie. If the condition worsens, the arms can also be affected. Patients with RLS can have a numbness, burning or tingling sensation in their extremities. RLS causes significant sleep difficulties and over time, chronic sleep deprivation. Patients can find it difficult to fall asleep or are awakened repeatedly at night from their legs moving. This will also disturb their bed partner’s sleep. Another movement disorder called Periodic Limb Movements frequently accompanies RLS, affects patients. PLM occur only when a patient is asleep.
RLS and PLMs can be effectively treated with readily available medications that are effective for most individuals. Requip was approved by the FDA one year ago and Mirapex November 2006 for treatment of RLS. Improved quality of sleep results in lessening of a risk of medical problems. Job security and quality of life are also dramatically improved. Physicians specializing in sleep or movement disorders should be consulted for diagnosis and treatment.