Harvard Doc Answers Neurology Questions

Whitehouse, N.J.: My dad died of Parkinson's and Alzheimer's. What is the chance that I would be diagnosed with one or both of these diseases? Is there anything I can do in middle age to cut my risk?
Dr. Martin A. Samuels:
Both Parkinson's disease and Alzheimer's disease may be rarely familial, but more often we believe that there is a genetic predisposition acted upon by as yet unknown factors, some of which a person may be able to control. At the moment, we have no scientific proof that you can do anything to reduce your risk of these illnesses, but it makes good sense to keep your blood pressure and cholesterol under good control. And minimize excessive drinking of alcohol, and smoking. Let me add that when I refer to "unknown factors," I mean an incalculable and unavoidable array of stimuli, such as innocent viral infections, which might give one person a common cold but another person, with the correct genetic predisposition of the immune system, an immune-mediated disease such as MS or lupus.

Calgary, Alberta, Canada: I am a partial paraplegic. I fell in 1979 and partially severed my spinal cord. I have been walking with braces and crutches. About two years ago (26 years after the accident) I suddenly developed new feelings, muscles and strength in my legs. What's going on? Why did it take so long? Will it continue?
Recovery from spinal-cord injury depends on the extent of the injury and the amount of retained function. In most cases, the spinal cord is not completely severed but rather traumatized by crush or vascular injury. There are two phases of recovery; the first is rapid, within days or weeks. The second is very prolonged, measured in years. It is believed that partially damaged fibers in the spinal cord may reconnect after long delays. It is also possible that some nerves and muscles that were not completely damaged have taken over the functions of those that suffered irrevocable damage, thus allowing for a very delayed recovery of some functions.

Jacksonville, Fla.: I have several medical problems involving my immune and nervous systems. My family has a history of Alzheimer's. I worked in pest control for several years and dealt with multiple chemicals. Could these have caused my medical symptoms?
It is very unlikely that chemicals encountered in the workplace would cause disorders that are as diverse as immune disorders and Alzheimer's disease. In general, stimuli from the environment, including some toxins, trigger just certain diseases, and do so mainly when there is a genetic predisposition. Many toxins are known to damage the nervous system in specific ways. For example, lead can cause cognitive delay in children exposed to high levels, as in lead paint. Mercury, often found in fish, can also cause nerve damage at high levels. Stimulant drugs, such as cocaine and methamphetamine, may cause strokes and seizures. Some patent medicines may contain potentially toxic levels of minerals that can damage the nervous system. Usually, when a toxin has damaged the nervous system it is possible to demonstrate very high levels of the offending drug or toxin in tissues, such as blood, hair or nails. Most concerns about microscopic levels of minerals (iron, lead, mercury) or minor trauma (head injury, occupational injuries) as the sole cause of diseases are grossly exaggerated and lead to an enormous amount of hypochondriasis, obsessional dietary habits and other behaviors, which serve only to reduce the richness of life.

Madison, W.Va.: My husband is 57. He was diagnosed with Alzheimer's in 2003. He has been to the University of Pittsburgh Alzheimer Disease Research Center. Is there any place in the United States where stem-cell therapy is being used as a treatment?
Stem-cell therapy is not yet an approved treatment for any human neurological disease. It is one of many avenues being explored in the effort to develop effective therapies. The ADRC at Pittsburgh is one of the best places in the country for the care of dementia. I recommend following its instructions and possibly enrolling in approved clinical studies that are underway.

Dresden, Ohio: I had two siblings with MS. My brother passed away 14 years ago, at 34, from complications of the disease. My sister, who is now 36, is struggling with it. I also have a first cousin who was diagnosed with MS 30 years ago. He has a brother who has been hit with the disease. We have been told time and again that MS is not genetic. If that is so, how could so many members of the same family be victims? We were born and raised in a Minnesota farming community. My cousins lived on a dairy farm their entire lives. It makes me wonder if there might be some kind of environmental link.
MS can be genetically determined, in that there is clearly a genetic predisposition to the disease. Having a close relative with MS definitely increases the risk of acquiring the disease, but it doesn't mean you are going to get it. Recent research has shown that most of the geographic variation seen in MS is due to genetic rather than environmental factors, so it is unlikely that growing up in a rural environment has much to do with it.

About four years ago I began having strong tremors in my right hand, leg cramps, balance problems, disorganization and other neurological symptoms. The worst was bumping into my wife of 30 years at work and not being able to remember her name or who she was. A neurologist diagnosed Parkinson's disease. I also had recurring sinus problems. An MRI revealed only a massive sinus infection, which was treated with surgery. The day after the operation, all of my neurological symptoms vanished and have not returned. Is there a possible connection?
There is no evidence that sinus infections could cause a disease that looks clinically like Parkinson disease. Chronic infection, could, however, bring out the symptoms of a neurological disorder. Conversely, treating the sinus disease would not directly treat the neurological disease, but could ease the symptoms in an indirect fashion. I would recommend another neurological opinion about the possibility of Parkinson disease. A good general internist should be able to further evaluate the sinus problem. Also, there are some rheumatic conditions that can cause sinus inflammation and neurological problems. These should be easily diagnosed with the proper blood studies ordered by an internist or rheumatologist.

Is it possible for a physician to mistake Lyme disease for multiple sclerosis, since white lesions show on the MRI film for both diseases?
Most experts will exclude neuroborreliosis (Lyme disease) when they are evaluating patients for possible multiple sclerosis. There are widely available laboratory tests that help make this distinction. By and large, the symptoms of neuroborreliosis affect the peripheral nervous system, mainly the nerves, whereas the symptoms of multiple sclerosis exclusively affect the central nervous system. For example, Bell's palsy (facial weakness) affecting both sides of the face, often occurs in Lyme disease, but very rarely in authentic multiple sclerosis. Sometimes, the MRI of the brain shows some small white lesions in patients with a history of Lyme disease, but there is no good evidence that these are similar to the kinds of lesions seen in MS. Of course, a rare person might have both MS and Lyme disease, but this would be relatively rare and there are no data suggesting a causative relationship.

Is Lewy body disease hereditary? Why doesn't it get much publicity?
Lewy body disease is the second most common cause of Parkinsonism after Parkinson disease. It is relatively rare but is seen regularly by specialists in Parkinson-like diseases. It is usually not hereditary, but, as is the case with many human diseases, likely the result of a genetic predisposition combining with environmental factors to trigger the clinical onset of the disease. There is no direct treatment for Lewy body disease, but the symptoms may sometimes be helped with medications that are used for conventional Parkinson's disease and various psychiatric disorders.

I have primary progressive multiple sclerosis. Balance is my main problem. Are there any medications to treat this?
Primary progressive multiple sclerosis should be managed by an MS expert. There are two interlocking problems; one is inflammation in the nervous system and the other is loss of nervous tissue (neurodegeneration). The inflammation problem is the one that is treatable, but it may require a fairly aggressive regimen of drug therapy in people with primary progressive MS, so it is generally believed that such patients should be managed in an experienced MS center. At the moment, we have no evidence that any other form of therapy (e.g., physical therapy) is beneficial is slowing the progression of this type of MS.

I was diagnosed with multiple sclerosis a few months ago. Since I have several symptoms that are bothersome, I would like to know if there are any improved medications for fatigue and spasticity (stiffness).
Fatigue in MS is a common and sometimes debilitating problem. Amantadine is one of the best-proved treatments for the fatigue of MS, but some newer drugs, such as modafanil (which is used to treat sleepiness) may sometimes help, as well. Stiffness is usually due to spasticity, which can often be treated with pills, such as baclofen or tizanadine, or sometimes with local injections of botulinum toxin.

I had a right lobectomy surgery in 1990, which has been successful in controlling my seizures. However, I have daily headaches and some severe "brain pain"--deeper than a migraine or headache. My concern is scar tissue. Do you ever recommend gamma knife or some other procedure to break up the post-op scar-tissue problems?
Head pain this far post-operatively is probably not due to scar tissue in the brain. Also, the brain itself is not pain sensitive, so breaking up scar tissue there would probably not treat the head pain. It is more likely that these are more conventional headaches, like migraine headaches. If so, there are many treatments that could help. A neurologist who specializes in headache could be of great help.

I am 63 years old and was diagnosed with multiple sclerosis in 2000. I have been reading about a possible DNA serum vaccine, among other potential treatments. Since I am on my own, I would want to aggressively treat my disease in order to better be able to remain independent.
There are many new therapies that are being tested as a possible treatment for multiple sclerosis, but a vaccine is not yet one of them. The best approach is to become associated with one of the major MS clinics around the country, where you can have an expert advise you about the new therapies and chances to enroll in legitimate clinical trials.

In 2004, my wife suffered a subarachnoid hemorrhage. Numerous MRIs and arteriograms were unable to pinpoint the exact cause or location. A VP shunt was installed due to increasing intracranial pressure. There has been slight short-term memory loss since the event. What is the likelihood of another occurrence and what is the longer-term prognosis?
When angiograms do not find the cause of subarachnoid hemorrhage, that usually means that the chance of a recurrence is very low. Unfortunately, the memory problem could have been caused by damage from the original hemorrhage. Your wife should consult a cognitive and behavioral neurologist about possible treatments.

I heard that there may be a new target for deep brain stimulation (DBS) for patients with progressive supranuclear palsy--the part of the brain called the pedunculopontine. Can you tell me about this target and how DBS would work on it?
Stimulation of the peduculopontine nucleus in the pons is a promising target for deep brain stimulation for people with gait disorders of various kinds. These include Parkinson's disease, syndromes similar to Parkinson's disease and a disease called progressive supranuclear palsy. This form of therapy is still experimental. Deep brain stimulation can work by two separate mechanisms. The first is by stimulating a brain nucleus so rapidly that it cannot recover between stimuli, thus simulating a destructive lesion. It was discovered many years ago that the biochemical changes of Parkinson's disease result in overactivity of certain nuclei in the brain, particularly the globus pallidus and the subthalamic nuclei. The first surgical treatments for Parkinson's disease involved destroying these structures, thereby reducing the overactivity. This is less than optimal therapy: destroying brain structures can lead to some unavoidable side effects. By stimulating these structures very rapidly, their overactivity is reduced but they are not destroyed. This allows the doctors to turn off the stimulator or even remove the stimulating electrodes if the treatment does not work or is causing unacceptable side effects. As far as we know, most deep brain stimulation works by this mechanism. It is also possible that one could use the stimulator to make an abnormally silent structure become active. As of this moment, the precise mechanism of deep brain stimulation in the pons to restore gait has not been discovered. But it could be this "activation" effect.

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