Lyme Disease of the Nervous System: Tracking the Elusive Spirochete - An Interview with Dr. Bass by Dr. Paget

Interviews with Experts

Anne R. Bass, MD

Associate Attending Physician, Hospital for Special Surgery
Program Director Rheumatology Fellowship Program, Hospital for Special Surgery
Associate Professor of Clinical Medicine, Weill Cornell Medical College

Stephen Paget, MD: My name is Dr. Stephen Paget, Chief of Rheumatology at Hospital for Special Surgery and it is a pleasure today to introduce Dr. Anne Bass, an Assistant Professor of Clinical Medicine. She will focus on Lyme disease and neurologic manifestations of Lyme disease. Anne what is our current concept of how Lyme disease develops?

Anne R. Bass, MD: Well, we do think that Lyme disease is an infectious process; that is number one -- that it is antibiotic responsive. Clearly many of the manifestations of Lyme disease stem from the body's own immune response to the organism and that applies to neurological Lyme disease just as it does to arthritic manifestations of the disease.

Stephen Paget, MD: Is it an eminently treatable disease?

Anne R. Bass, MD: It is extremely treatable. It is best treated early. If you see a patient with erythema migrans and other symptoms and treat them promptly with antibiotics, the vast majority will not go on to have later complications and, even those patients who do have later complications, will be by and large curable with antibiotics.

Stephen Paget, MD: Is it less common today? Are we picking it up earlier because its more known?

Anne R. Bass, MD: I don't think it's less common. I think that we just have a very educated public and medical treatment complex. I think this is a disease that is picked up very early. Most patients who have erythema migrans rash recognize it and go to the doctor promptly, and physicians have come to recognize that flu-like symptoms and headache in the summer months might be Lyme disease. So I think that we are seeing a lot of the disease, but we are treating it early.

Stephen Paget, MD: Tell me about the manifestations in the neurologic system that patients can get with Lyme disease.

Anne R. Bass, MD: Well, early on about 15% of patients who are not treated with antibiotics will go on to have often a facial palsy, with weakness on one side of their face, which will generally resolve even if they don't receive treatment. The minority will develop a mild meningitis picture or what we call radiculitis with pain in nerve roots going down the arm or the leg. Those manifestations again are quite antibiotic responsive.

The later neurological disease is quite rare. In this country, we sometimes see what we call a subtle encephalopathy where patients have cognitive and sometimes mood abnormalities. These patients also respond to antibiotics although much more slowly. The more severe central nervous system disease, encephalomyelitis, is seen almost exclusively in Europe. Peripheral neuropathy can be seen here but, again, is much more common in Europe.

Stephen Paget, MD: Why does there seem to be such a difference in Europe versus the United States regarding the incidence of these problems?

Anne R. Bass, MD: The Borrelia burgdorferi, the cause of Lyme disease, comes in several substrains, and the strains differ in the United States from those that we see in Europe. So in this country, we see Borrelia Sensu stricto which causes, in untreated patients, very commonly an arthritis and much less commonly neurological disease. In Europe, they see other organisms: Borrelia Afzelii, which is responsible for the acrodermatitis skin lesion. They also see Borrelia Garinii and some of the sensu stricto strains as well. And those strain differences probably explain the differences in the manifestations that we see there.

Stephen Paget, MD: In Lyme disease in genera,l what roles do genes, the infectious organism itself, and the immune system play in the way we see it clinically?

Anne R. Bass, MD: I think all of those things play an important role. In this country, there have been rare case reports of patients with refractory arthritis. Those rare cases probably have an immunologic basis. There are HLA association HLA-DR-4 that may make some rare patients susceptible to this chronic arthritis that is not antibiotic responsive. We don't know much about host factors regarding neurological disease, which is far less common. We think that, with regard to neurological disease, there may be more importance in the differences between strains of Borrelia. But as we learn more about the immune response to the organism, we may find different host responses that explain differences in disease manifestations.

Stephen Paget, MD: There are new vaccines for Lyme disease and, given the fact that some patients will get this and no longer develop problems with Lyme disease, is there any possibility that the vaccine itself may trigger off the disorder given potential cross reactivities?

Anne R. Bass, MD: Originally there was great concern that the vaccine might be associated in particular with a chronic arthritis because it is know that the patients who develop a chronic oligo- or monoarthritis tend to have antibodies against the Osp-A protein, which you don't see in early Lyme disease. (Borrelia down-regulates Osp-A when patients are initially infected and it only seems to be up-regulated much later in the disease process.) So antibodies against Osp-A are seen in patients with chronic arthritis and the vaccine is, in fact, the Osp-A protein. So there was a concern that if you vaccinate patients with Osp-A, they might in fact develop a chronic arthritis of, if they had a chronic arthritis, it might worsen. To date, that has not been seen. So it appears to be safe.

Stephen Paget, MD: So to whom do you give the vaccine?

Anne R. Bass, MD: The vaccine really should be given only to patients who live or spend a lot of time in an endemic area (which is largely Long Island, Connecticut, the New Jersey shore, and upstate New York) where they are frequently in areas where they might come in contact with ticks. They spend time outdoors. Those patients, if they choose, will benefit from the vaccine. The vaccine doesn't last forever. The patients will probably need boosters every two years, maybe even one year, to maintain immunity, and the patients need to recognize that. It is also not 100% effective. It is only about 90% effective after the first three doses. So it is not a cure-all, but I think for patients who do live in these areas and spend a lot of time outdoors, it can be helpful.

Stephen Paget, MD: So if you have patients who have been fine all of their lives and they develop Lyme disease that you would agree is clear Lyme disease, both clinically and supported by serological information, and then the Lyme disease manifestation is over with and, from then on, they develop aches and pains and cognitive abnormalities, how do you treat those patients? What is that entity?

Anne R. Bass, MD: Right. With some of those patients, it will depend on how much antibiotic therapy they have had and what really is the nature of their illness. So in the case of a patient who has gotten three weeks of oral antibiotics for early Lyme disease but later presents with actual objective cognitive abnormalities, especially if there is positive PCR in the CSF, that patient may have Lyme encephalopathy and benefit from intravenous antibiotics, ceftriaxone in particular, for a month.

On the other hand, if you have a patient who had oligoarthritis and was treated with a month or maybe even two months of intravenous antibiotics and down the road has myalgia and arthralgia and subjective complaints of cognitive difficulties, but normal testing when it is done objectively, and negative PCR (if the patient is sick enough neurologically to have an LP done), that patient does not benefit from further antibiotic therapy. I think that has been shown very clearly. That patient is treated supportively much in the way we treat a patient with fibromyalgia.

Stephen Paget, MD: What is your concept about the entities of chronic fatigue syndrome of fibromyalgia after eosinophilia myalgia after obvious Lyme disease and the "Gulf War Syndrome"? What is that entity and what does it tell us about the disease?

Anne R. Bass, MD: I don't think we really know the answer to that question. I think possibilities include alterations in the balance of cytokines in the immune system that was somehow changed at the time of an infection. There may be psychological sequelae from being ill. I think that there is a tremendous mind-body connection, and it is not that the patient is not having symptoms; it's just that there are major psychological influences on the experience of pain. There is also the anesthesia literature on chronic pain syndromes, where they have shown that pain that is initially initiated in one area, for example an injury to an arm, can later, because of chronic pain, spread to other areas. There can be activation of the nerves that are responsible for pain in the spinal column, so there can be neuropathic spreading of pain. So as we come to understand these processes better, we may have better treatments for these patients. They are certainly symptomatic. In patients who have post-Lyme syndrome, analyses of how they are functioning in their lives show that they are functioning poorly, just like the patients with fibromyalgia. It's not that these patients are not ill. It is just that we haven't come up with the best way to make them feel better.

Stephen Paget, MD: Thank you very much.

Dr. Bass was interviewed by Dr. Stephen A. Paget, Physician-in-Chief, Hospital for Special Surgery.

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