Below, Dr. Allan Ropper and Dr. Martin Samuels answer questions about neurological disease.
Small Fiber Polyneuropathy
Question: What is the most effective treatment for severe small fiber polyneuropathy? I have been told that taking a megadose of prednisone (100mg daily for 3-4 months) can sometimes, but not always, push the disease into remission. My physicians have cautioned me that the side effects of this treatment (e.g., hypertension, weight gain, compromised bone density, etc.) make the prednisone treatment risky. I have also been told that the prednisone treatment would have to be repeated in intervals for the rest of my life, not an appealing prospect. Are there any alternative treatments or treatments that may be available in a few years?
Dr. Ropper: The treatment of small fiber neuropathy depends entirely in the underlying cause such as amyloid, Sjögren syndrome, or tumor. A few cases are due to inflammation alone, without a clear inciting cause and these may be treated with prednisone, but that is not a typical approach. It is more a matter of a very complete evaluation than it is waiting for a future therapy.
Episodic and Chronic Headache Syndromes
Question: I was diagnosed with CPH (chronic paroxysmal hemicrania) over a year ago. Can you tell me more about this headache disorder and treatment?
Dr. Samuels: CPH is a headache syndrome in which headaches occur in bouts lasting about 20 minutes each. It can be episodic (EPH) or chronic (CPH). Both types usually respond to the potent anti-inflammatory drug, indomethacin, which must be prescribed by a doctor. Everyone with the diagnosis of CPH or EPH should have one good image, preferably and MRI, of the brain, just to be certain that there is no specific cause that needs to be treated.
Ringing in the Ears
Question: I suffer from tinnitus (ringing in the ears), every waking moment. Recently, on the radio I heard about a new treatment that addresses brain waves. Can you tell me about this treatment?
Dr. Ropper: There is a deep brain stimulation method for tinnitus being done in a few centers. It involves implanting electrodes in the brain and connecting them to a pacemaker-like device under the collar bone. There has been some success, but not in everyone. Because the results have been inconclusive thus far, we at the Brigham are not doing this yet.
Speech Difficulty after Stroke
Question: My grandfather has paralysis caused by high blood pressure and is currently in the hospital. He does not have problems in moving and has full use of his arms and can walk. The only area that is paralyzed is his mouth. When he was transported to hospital a few days ago, he had very serious problems eating, drinking, and speaking. Fortunately, he can eat and drink but still cannot speak – stammering at best and not able to form words. The doctors say that he has no problem with his vocal cords but rather with the articulation of words. They have also determined that he did not have a hemorrhage of the brain so he should begin to be able to speak. Is there a name for this condition and what can be done?
Dr. Samuels: This sounds like a condition we call aphasia, which means a language problem, usually caused by damage to the part of the brain which is responsible for language formation. Usually this is associated with paralysis or weakness of the right side of the body. The aphasia usually gets better more slowly than the paralysis. Speech therapy can be helpful and, in time, his speech should improve but it could be a long time - up to two years after the stroke.
Chronic Migraine Treatment
Question: I am 43-years-old and a chronic migraine sufferer. I have tried taking magnesium, topiramate, rizatriptan, zolmitriptan, naratriptan, and sumatriptan. In the past, if all else fails, prednisone has helped to break the hold of the migraine. However, this is no longer working. My quality of life has been markedly impacted. I forget complete conversations, mix words, and forget words and memories. The pain is agonizing and so is the fear I have. What can be done for me?
Dr. Ropper: Chronic migraine has always been difficult to treat. It is important to be sure that there are not “rebound” headaches from overuse of pain medications such as ibuprofen. Antidepressants such as amitryptiline have been helpful in some cases. An ideal approach is regularization in sleep, eating, exercise, coupled with complimentary treatments such as biofeedback. A mainstream headache clinic should be able to advise about these long-term plans for treatment.
Treatment for PRES
Question:Can you give me the overall cause and treatment of PRES syndrome?
Dr. Samuels: PRES means posterior reversible encephalopathy syndrome. It is due to a defect (reversible) in the endothelium of the cerebral blood vessels. This endothelium provides the barrier to the brain from the blood. Many events may cause this to occur. Most commonly it is high blood pressure. This can occur during or right after pregnancy (toxemia). Other toxins and certain drugs – such as chemotherapy used for transplant cases - can cause it as well. With proper treatment, it is completely reversible.
Question: My 88-year-old father has had sporadic jerking and shaking five times since he was in his middle 70's. It always starts in the middle of the night, and it starts with his mouth and jaw twitching and then, over time, his entire body. He has never had the jerking longer than 24 hours. He has been given levetiracetam and lorazepam and told they could be seizures. He has had CAT scans, blood tests, and been seen by several neurologists and everything comes back looking fine. Do you have any advice on what we can do next?
Dr. Ropper: If he is completely awake during the episodes, seizures are less likely. There are, however, many types of true seizures that can be difficult to diagnose. I assume he has had several EEGs that were normal. The next step might be a longer term EEG that can be done at home with a recorder overnight.
Question: I have recently been diagnosed with meralgia paresthetica in my right thigh. It is very numb yet extremely sensitive to touch and I often get a burning sensation. I had a nerve conduction study and an EMG both of which were normal. I feel a nerve twinge in the space between my hip and groin when I bend in a certain position. My neurologist is referring me for a nerve block hoping this will reduce swelling and provide some pain relief. I asked about surgery if more conservative measures fail and he mentioned that they sever the nerve during surgery. Is nerve decompression considered before severing the nerve?
Dr. Ropper: Meralgia paresthetica can occasionally be due to nerve compression in the pelvis or other types of nerve disturbance and it might be worth doing an imaging study and having careful abdominal and pelvic examinations by a surgeon and gynecologist. Nerve blocks are also commonly done as your neurologist has suggested. Surgery is a very last resort.
Hot Sensations on Side of Head
Question: What causes hot sensations on the right side of the head? Almost like a hot flash in the head that comes then goes after a few seconds.
Dr. Ropper: These are common and most often, do not suggest an underlying problem. It is only if the sensation persists or turns into numbness, if there is facial weakness, or other neurological symptoms that it is evaluated beyond a neurological examination. In keeping with this, a rare problem called syringomyelia can cause it but almost never as the only symptoms. No one is sure what causes them.
Quick Pains on Side of Head
Question: I have been having quick pains on the right side of my head, mainly just above the ear, with the pain traveling behind the right eye and sometimes teeth. I also have tingling in the left hand. Could this be a neurologic condition?
Dr. Ropper: Quick head pains are common and usually do not represent serious problems but hand numbness should be checked by your local physician and a neurologist if the first doctor feels that would be useful.
Writing and Thinking Ability During Dementia
Question: My father has been exhibiting some signs of dementia since his stroke six years ago and his neurologist is asking for three letters from the family. I'm wondering what the neurologist will do or can do with these family letters? I agree that my dad possibly could need to stop driving, but he is not at the point to require 24 hour care.
Dr. Ropper: The neurologist may want the letters to have objective evidence of the approximate course of your father's writing and thinking ability. In themselves, they would not be enough to guide decisions about driving and 24 hour care but may assist in diagnosis. Many other aspects of the history, examination and testing are required to for diagnosis and prognosis of dementia.
Bells Palsy and a Low Immune System
Question: My question is related to Bells palsy. I am a lupus patient and a kidney transplant patient so my immune system is low. One day I woke up and went to brush my teeth and noticed that I could not hold the water in my mouth so I went to see my doctor right away and she told me I have Bells palsy. Nothing on the left side of my face moves…I can close my eyes but not blink. I was given prednisone 60 mg for three days, 40mg for 3 days, 20mg for 2 days, 10mg for 2 days, and 1/2mg for 2 days. Now, all I can do is wait it out and see if anything changes. Is there anything else I can do? Should I be doing a facial massage or something?
Dr. Ropper: Because your immune system is not normal, you may want to ask your doctor about taking a medication to treat viruses. Otherwise, most cases of Bells palsy improve on its own over a few weeks or months but this may be different in your particular case. Massage is good if it makes your face feel better but it generally does not make a difference in recovery. Some practitioners use nerve stimulation but that is also of questionable value.
Tests for Chronic Muscle Pain
Question: What kind of tests can a neurologist do for chronic muscle pain due to either hypermobility syndrome or fibromyalgia?
Dr. Ropper: A careful neurological examination, blood tests for muscle enzymes, and an EMG (electrical test of muscle and nerves) will help to determine if there is an underlying neurological condition. These tests are normal in fibromyalgia.
Drop Seizure Evaluations
Question: Would a drop seizure or passing out without any warning (two times within a month) in a 74-year-old male likely show on an EEG? What other neurologic evaluations should be done if all cardiovascular possibilities show negative?
Dr. Samuels: Sudden loss of consciousness in a person of that age could certainly be cardiac. It could be necessary to continuously monitor the electrocardiogram to record a spell and determine its cause. A seizure is also possible. Usually after a seizure the person is confused for a little while. If this patient immediately awakens then a cardiovascular cause is more likely. If it only happens in the upright posture, it could be a drop in blood pressure, which could be due to certain medications. A careful cardiac and neurological evaluation is certainly in order.
Numbness on Side of Face
Question: I have been having some slight numbness on the right side of my face that often lasts up to an hour. Could this be a neurological condition?
Dr. Ropper: Depending on your age and other conditions, this could be an inflammation in the nerve in your face, which has several causes that can be detected by an examination and blood tests. You should be seen by your general doctor soon and referred to a neurologist if he or she feels that is appropriate.
Prophylactic Dose of Aricept for AD
Question: My grandmother, mother and 63-year-old old sister have Alzheimer’s disease. I am 58, should I be on a prophylactic dose of Aricept?
Dr. Samuels: There is no reason to take Aricept prophylactically. The vast majority of people with AD do not have a familial illness. You can have a neurological evaluation if you think that will reassure you better.
Question: Is there an effective treatment for Meniere's disease?
Dr. Samuels: Meniere’s disease [a disorder of the inner ear which causes episodes of vertigo, ringing in the ears, a feeling of fullness or pressure in the ear, and fluctuating hearing loss] causes attacks of dizziness and hearing loss. Over time, the hearing in the effected ear becomes worse and there is often tinnitus (ringing in the ear). It is difficult to treat, but sometimes diuretics can be helpful. Ultimately an otologist can inject an antibiotic into the ear that will cause damage to the nerve and reduce the symptom of dizziness though the hearing in that ear will be worsened. Meniere's disease is usually treated by otorhinolaryngologists.
Muscle Twitching is Fairly Common
Question: I have this muscle twitching that has been going on for more than a year now. It is generalized, mostly my right eye starts twitching randomly, sometimes my thigh muscles may twitch or my arms may twitch when I stretch it out. I have had an MRI that ruled out multiple sclerosis last year. I also have had lab work done to rule out vitamin deficiencies. I am still having the twitching almost on a daily basis, it lasts for five to 10 minutes but comes back again without warning. What do you think may be going on here?
Dr. Ropper: Most of the time, these moving muscle twitches, or fasciculations, are benign and no cause is found. They tend to go away. This is a fairly common problem in normal people. Reducing coffee and similar stimulants may help. There also are few diseases of blood electrolytes, nerves and muscles that can cause twitches and these can usually be separated from the benign ones by a neurological examination, routine chemistry tests and an EMG.
Sympathetic Nervous System Can Cause Sweating
Question: Is there any connection between a neurological problem and hyperhidrosis?
Dr. Samuels: Yes, hyperhidrosis [a condition characterized by abnormally increased perspiration] can be the result of overreaction of the sympathetic nervous system. The sweat glands can be injected with botulinum toxin to reduce the sweating if it is a cosmetic problem.
Question: My sister's husband, 55, has developed a drooping left eyelid. He has had difficulty with enunciation for a few years now, although he can speak slower and more distinctly if he thinks about it. Sometimes he sort of chokes on his food, has a bit of a shuffle, and says his left arm feels as though "it just hangs there." I have two questions: Is there a definitive test for myasthenia gravis? How serious a disease is it?
Dr. Ropper: There are several tests that taken together, are almost certain for myasthenia gravis [an autoimmune neuromuscular disorder that leads to fluctuating muscle weakness and fatigue]. Sometimes, the tests must be repeated several times and even then, a very small number of cases remain uncertain. Most neuromuscular clinics can perform these tests - some are blood tests, others related to EMG, and it can be useful to inject a drug called neostigmine, which improves myasthenic symptoms. There are several diseases that can imitate myasthenia so a careful examination is required.
Health History Could be Key to Neurological Condition
Question: My mother has a progressive neurological disorder with no diagnosis for 20 years. She has seen many specialists and was even a case study at a neurolgical conference, but she was still unable to get diagnosed. She is currently paralyzed, on a vent. She cannot speak or eat, but she is alert. Genetic testing was done - but no link. We have recently found out that in 1946, when she was 11, there was a worldwide flu pandemic and she contracted the H1 Influenza B virus. She was temporarily paralyzed for several days and received her first tracheotomy. I found out that this flu was a strain of the World War 1 flu that killed millions. Most of the people who survived were diagnosed with Parkinson disease years later. At a recent hospital stay, a doctor said that it was a "real possibility" that her 1946 flu caused her neurological disorder and we needed to speak to a neurologist. Could the flu that my mother caught in 1946 have caused her neurological disorder today?
Dr. Samuels: There is a post-encephalitic form of Parkinson disease that occurred after the early 20th century epidemics of the flu. This type of Parkinsonism has occurred occasionally since the epidemics ended about 1920, so acquiring a similar illness in 1944 is remotely possible. A neurologist certainly should see her, as there are some features of post-encephalitic Parkinsonism that can be recognized by an expert. It is more likely, however, that she suffers from an unrelated neurodegenerative disease. An expert neurological consultation is nonetheless advisable.
Managing Post-Spinal Injury
Question: After his spinal cord injury (T-12 burst fracture) was repaired by spinal fusion surgery in 2002, my husband has since suffered intense chronic pain. All non-surgical treatment options have been tried, unfortunately without success. Even with all the current medications, he averages - on a daily basis – a 5 to 7 level of pain. Are there any surgical (even if radical), or other interventions that can decrease this chronic pain?
Dr. Ropper: There are several good surgical options for post-spinal injury pain. The two main ones have a reasonably good track record: 1) spinal cord stimulator and 2) pain pump - both can be inserted temporarily as a test before committing to more permanent placement.
Allergic Reaction Can Cause Neurological Complications
Question: Two months ago I was at a hospitalized for angiodema [a rapid swelling of the skin and tissues] that was the result of an allergic reaction to a high blood pressure medication. I received an injection of a form of Benadryl intravenously. Unfortunately, it was injected too fast and a feeling of being struck by lightening and burning went from my brain to the pelvic area to my feet. Since that moment of injection, my entire body vibrates nonstop. Some days are much worse than others - it can even wake me up at night. I have had numerous blood tests with my own physician with nothing showing up. Now I am thinking it is time to see a neurologist, but there are so many different neurology specialties. Do you think a neurologist may be able to help me?
Dr. Samuels: Some people do get prolonged symptoms after an allergic reaction of this kind. Often such patients have a history of migraine. It seems that people with migraine have a heightened sensitivity to pain and this can be stirred up by an acute trauma of this type. An appointment with a general neurologist could be useful in evaluating your symptoms.
Signs of stroke
Question: What are the signs of stroke?
Dr. Ropper: Most strokes start suddenly with either paralysis or numbness on one side of the body, slurred or difficult to understand speech, and/or loss of vision in one eye. Any one of these symptoms can mean that you are having a stroke. The symptoms may clear in minutes or hours (a transient ischemic attack, or TIA) but reversible symptoms represent as big a risk as a stroke that does not improve. Some strokes are due to bleeding in the brain and, in addition to the symptoms noted above, there is usually severe headache and there may be vomiting and loss of consciousness. If you are experiencing any of these symptoms, it is important to seek medical help immediately.
Question: Can a stroke be prevented?
Dr. Ropper: Atrial fibrillation, especially in people over the age of 55, and heart valve disease increase your risk of stroke. By using anticoagulants such as warfarin (Coumadin®) the risk of stroke can be greatly reduced. The amount of warfarin that is taken by a pill every day must be carefully monitored by blood tests in order to avoid bleeding in the body. If you have diabetes, high blood pressure, or high cholesterol you also are at increased risk for stroke. Control of these risk factors by medications reduces your risk of stroke over time. Smoking also is a well documented risk factor for stroke and you should make every effort to quit. If you have had a transient ischemic attack (TIA) in the previous days and weeks, you are at risk of stroke and should be examined as soon as possible. TIAs are a warning of a stroke to come. In some cases, a partially blocked artery is found to be the cause of the TIA and opening of the artery with surgery or placement of a stent may prevent a stroke. In patients who have already suffered a stroke, the addition of an aspirin or an anticoagulant and possibly high doses of “statin” drugs to reduce cholesterol have all been shown to reduce the risk of a second stroke.
Headache versus migraine
Question: What is the difference between a headache and migraine?
Dr. Samuels: Headache is one of the most common afflictions of human beings. Nearly everyone has experienced headache and many people have recurrent headaches. Migraine is the most common type of recurrent headache, but other causes of headaches exist as well. It is important to see a doctor about headaches so the doctor can hear about the nature of the problem and carry out a neurological examination. Other causes of headache can be excluded based on a skilled physician's evaluation, sometime with some tests, including brain images. In the end, most recurrent headaches will be determined to be migraine. Migraines run in families and show a huge spectrum of manifestations across the entire life span. Contrary to common belief, all migraines are not necessarily severe. They may be preceded by a period of neurological impairment, call the aura, which is usually a visual experience, such as flashing lights or holes in the visual experience. The headache itself may be mild or severe. If severe, it can be associated with nausea, vomiting, sensitivity to loud sounds and bright lights. A severe migraine headache can last several days, but most are relieved by sleep and therefore disappear after a few hours. Many relatively new treatments are available to abort a migraine headache and other treatments are available to prevent the migraines if they are occurring frequently enough to interfere with a person's lifestyle. Primary care doctors are skilled in determining whether a headache is serious or not and in initiating appropriate treatment. Neurologists are specialists in diseases of the brain and the rest of the nervous system and may be consulted by the primary care doctor if a particular patient’s headache problem is unusually severe, unusual or resistant to treatment. Within the specialty of neurology, there are some physicians who specialize only in headache. These doctors are consulted for difficult or unusual headache problems.
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This page was last modified on 7/12/2013