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Bacteria or virus -- what caused the Black Death?
The new `Great Imitator'
Sheep tick
can pass rare skin cancer to people
NEWS ARTICLE from Desert News, 7-28-01, By Sharon Haddock
``PROVO -- Utah County officials are asking the public to be on the alert after two local men were definitively diagnosed with tularemia or "rabbit fever."
Both men had been hunting recently and were probably exposed to the rare bacterial disease the first part of July [2001], said Dr. Joseph Miner, Utah County Health Department executive director. One was bitten by deer flies while camping in Pinedale, Wyoming, and the other reported handling a rabbit that may have been ill.
The disease, with fewer than 300 cases reported in the United States each year, is potentially fatal and is usually spread through contact with infected wild rabbits, ticks or deer flies. Muskrats and beavers also can contract and spread tularemia.
Symptoms include the development of an ulcer at the site of the bite within 3 to 10 days followed by swelling of the lymph nodes nearest the site and headache, fever, malaise, and/or pneumonia ...
Miner said the 2 men who became ill went for several weeks without being properly diagnosed because the disease is so rare. Both are now responding to the antibiotic treatment ...
Tularemia, along with plague, botulism, brucella and anthrax, is one of the 5 infections considered most likely to be used as a bioterrorism weapon through the air or water supply. "In light of this, it's extremely important that health-care professionals and the public notify us as soon as possible if any of these are suspected," Miner said ... ''
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NEWS ARTICLE from Reuters Health, 7-24-01
``Authors Challenge Established Theory on 'Black Death'
LONDON -- A virus similar to the deadly Ebola virus might have caused the "Black Death" that ravaged Europe between 1347 and 1670, UK-based researchers say.
Their findings, which challenge the established theory that a bacterium caused the plague, suggest that a similar outbreak could happen again, with devastating consequences.
The Black Death arrived in Sicily in 1347 and during the next 3 years it spread relentlessly through Europe. More than 25 million people are thought to have died as infected people travelled to Britain and elsewhere, initiating epidemics that continued in Europe for over 300 years. The disease mysteriously disappeared in 1670.
Since the end of the 19th century, when scientists found that the bubonic plague was caused by a bacterium called Yersinia pestis, it has been universally thought that bubonic plague was the disease that had beset Europe.
In their new book, Biology of Plagues, published by Cambridge University Press, Dr. Susan Scott and Dr. Christopher Duncan from University of Liverpool in England provide evidence that this view may not be correct.
"Bubonic plague was given its name because of the appearance of buboes (swollen lymph glands) in the victims. Presumably, historians were struck by the name plague and recognised that some victims displayed buboes. They automatically assumed that bubonic plague caused the Black Death and subsequent plagues, which we refer to as hemorrhagic plague," Dr. Scott told Reuters Health.
After examining reports of symptoms, symptom timing, and autopsy evidence that victims' internal organs had disintegrated, Dr. Scott and Dr. Duncan suggest that hemorrhagic plague may have been caused by a virus that is distantly related to Ebola.
As further evidence, they note that the Black Death was quickly recognized as an infectious disease. An effective 40-day quarantine period was established by the late 14th century and was maintained throughout Europe for 300 years. But quarantine measures are completely ineffective against bubonic plague ...
[Dr. Scott] cautioned: "If a similar virus were to re-emerge with a long infectious but symptomless period, modern transport would mean the infection could spread rapidly throughout the world. The mortality would be catastrophic." ''
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FEATURE ARTICLE from The Washingtonian, 1-91, By Neil Raven
``Bicycle Boy -- His Behavior Was Compulsive, It's Origins Unknown; Then a Good Doctor Seemed to Make a Miracle Happen
He was 12 years old, and every day he pedaled furiously on his stationary bicycle for as many hours as they would allow him. He was so absorbed in his effort that it was all they could do to get him to stop for meals.
In fact, before he was hospitalized at a psychiatric institution he had been unwilling to stop for meals, for school work, for the simple exchanges of ordinary life. At age 12, he had lost almost 30 pounds. He looked, in the language of the ward, cachetic, or in the language of his friends, as if he had been an inmate in a concentration camp.
His parents, after all the agonizing, had coaxed him into a car and driven him out to the facility , where they had carried his suitcase as they walked him to the ward. And they had handed their son over to the care of others, out of desperation, convinced that he was now beyond their help - their son who wanted only to pedal, to exert himself and withdraw from the world he had once embraced with such sunny exuberance.
The psychiatrists questioned the parents and the boy - the skeletal, restless boy, who not so long before had been a good student, a healthy, happy son. He had been a wonderful athlete, an exciting soccer player, but he had had some knee problems. Over two years he had had four episodes in which his right knee swelled enough to require treatment.
It was after the last episode that he had withdrawn. He spent most of his time alone in his room, fiddling with a ham radio, not talking to his friends or his parents. He stopped doing his homework. And then came the exercising, the disinterest in food, the weight loss.
At a glance, the boy reminded the psychiatrists of the young women who suffered from that dreaded and potentially lethal psychiatric condition, anorexia nervosa. He had that bony look, that restless hyperactivity.
But he was male, which is unusual for anorexia nervosa patients. And he was only 12 -- most patients with anorexia nervosa are older. It could be a working diagnosis. But when things don't quite fit the pattern, you ask questions. You call in more opinions. They called in Andrew Pachner.
Andrew Pachner looks over to the framed photograph on the wall of his office at the Georgetown university Hospital's neurology department. The photo is a blowup of a single Borrelia burgdorferi spirochete - a microorganism that bears a striking resemblance to the organism that causes syphilis ...
[Dr. Pachner] recalls the day he first laid eyes on the 12-year-old bicycle boy. Pachner was then a junior faculty member in the Department of Neurology at the Yale School of Medicine, living on a salary that didn't even approach subsistence level. While the university looked the other way, all the junior faculty members moonlighted to pay the rent. Among Pachner's stints was a job evaluating patients at the psychiatric institute.
Not all patients were selected by the psychiatrists for Pachner's review. But the bicycle boy was. For one thing, there were those swelling episodes and the probable history of arthritis.
While he was still in training, Pachner had drifted down to the Yale arthritis clinic. Diseases of the joints might seem an unlikely source of fascination for a doctor specializing in diseases of the nervous system, but there was a vital connection. Diseases of both are often [sometimes?] caused by mistakes that cause the immune system to turn against itself - autoimmune diseases.
The doctors studying arthritis were happy to have Pachner around. Many of their arthritis patients were suffering from autoimmune diseases, such as systemic lupus erythematosus, which have neurologic complications. Pachner's neurology expertise was welcomed.
While Pachner was examining patients in the arthritis clinic, he became an interested bystander to one of the most celebrated moments in medicine - the identification of a new disease.
... a group of children in Old Lyme, Connecticut, not far from Yale ... had a curious form of arthritis that followed the appearance of a peculiar and characteristic skin rash called erythema chronicum migrans, or ECM. [first described in this country by Dr. Scrimenti in Wisconsin, in 1970.]
... In 1982, Drs. Willy Burgdorfer and Alan Barbour, working at the Rocky Mountain Laboratory in Montana, pinpointed the cause of the disease.
... [The] young patients had an arthritis caused by a spirochete. Unlike bacteria, spirochetes are not easily grown in the laboratory. The standard way to study a microorganism is to grow it on a special broth, a culture plate.
But spirochetes, like exotic zoo animals, do not live long outside their native habitats. Once outside the body, they die. The human body makes antibodies to the organism , which makes diagnosis possible, but the antibody tests can be tricky, and occasionally misleading.
The world's best-known spirochete is Treponema pallidum, which causes syphilis. The one that causes Lyme disease would prove to be an even bigger problem than syphilis in some ways, because people could not avoid it by abstemious behavior.
It was a spirochete that awaited children as they ran through the Connecticut woods, doing what their parents thought was healthy and good. The spirochete was carried by forest animals, and it waited for the unsuspecting, anyone who cared to enjoy the great outdoors: hikers, pregnant women toting little kids, fishermen, gardeners, and farm workers. It was the tick-borne spirochete that causes Lyme disease.
The bicycle boy had had his first attack of Lyme arthritis in 1982, two years before Pachner discovered him pedaling away on the psychiatric ward.
Pachner was aware that syndromes similar to Lyme arthritis, syndromes suspected to be caused by an infectious agent, had been described in Europe, and he knew these syndromes often included some neurological features, usually a form of radicular pain , which radiates down an arm or a leg. Radiculitis meant the trouble was in peripheral nerves, which flow to and from the spinal cord out to the extremities.
But none of the these arthritis-related European syndromes involved the central nervous system. None of these European syndromes caused complex behavioral changes, and no connection had ever been drawn between an infectious arthritis and any sort of neurological disease that might affect a person's behavior.
In order to cause a behavioral change, a disease has to affect the brain directly and in a widespread fashion. Various forms of vasculitis - inflammation of the small blood vessels - can do this. Autoimmune diseases can do this.
But none of the infectious - arthritis group of diseases were known to be capable of involving the whole brain. Focal lesions can "stroke out" particular functions, causing paralysis, speech deficits, or sensory loss, but the entire brain must be involved for memory deficits, disorientation, or obsessive behavior to occur.
Clearly, what was going on in the bicycle boy was amore than a simple radiculitis: in which only a single nerve root would be affected.
By 1982, physicians in Connecticut had been alerted to the possibility of Lyme arthritis, and the boy's first attack of knee pain had been treated with a form of tetracycline. But two years later, when the boy started to withdraw from life, started to become a behavior problem, his physicians made no connection between his psychiatric symptoms and his earlier episodes of arthritis.
"Lyme arthritis" was a disease of the joints or, at most, of the skin and the joints: nobody had any basis for suspecting a connection between the knee and brain disease - except perhaps for Andrew Pachner.
... Pachner had begun to uncover neurological symptoms and findings in his Lyme arthritis patients. Another neurologist, Louis Reik, who had preceded Pachner in the arthritis clinic, had passed on his suspicions that the Lyme patients might have more than simple radiculitis complaints. But it was up to Pachner to push ahead with his observations.
Pachner connected the symptoms of the European patients to the new, more diverse symptoms he was seeing in the Yale clinic. Reading through the chart of that 12-year-old boy, Pachner began to get excited.
Could this boy have an infection that affected not just his knee but his brain as well? The organism identified as causing Lyme arthritis was a spirochete. Syphilis was a spirochete, and what syphilis could do to a brain was well-known. It could cause dementia, bizarre pain syndromes, a whole variety of symptoms so diverse that medical students are taught to think of syphilis as the "great imitator".
Syphilis mimics many diseases because it can affect so many organs: heart, brain, joints, nerve, eye. Wherever blood goes, syphilis can go. Syphilis can cause a vasculitis of the small blood vessels in the brain, the eye, almost anywhere. Could this new spirochete, this borrelia burgdorferi, be as strange and protean in its manifestations as the "great imitator" itself?
Could it be, thought Pachner, that this bicycle boy has Borrelia in his brain?
If the spirochete that causes syphilis can enter the body through genital tissues, multiply, migrate to small branches of the vascular tree, migrate through the thin blood-vessel walls, and set up house in the brain and nervous tissue, and in heart tissue and aorta, was it so farfetched to believe that the Lyme spirochete might do something similar?
Might it enter the body through a break in the skin caused by an insect bite, the way malaria does, enter the blood stream, and multiply first in a knee joint causing arthritis, and then wreak havoc years later in the brain, as syphilis has been known to do?
Not having an answer, not having solid evidence or similar cases, Pachner could not voice his suspicions to the boy's parents. He spoke instead to the psychiatrists and asked them to transfer the boy to Yale - New Haven Hospital. The parents were told simply that there was a chance the therapy at Yale could help their son. They were willing to try anything.
When the boy arrived at the hospital, he was taken to the neurological ward . Pachner met his parents and explained that he believed there might be a connection between their son's previous bouts of arthritis and the problems that had landed him on the psychiatric ward. But Pachner could make no promises--they were in uncharted waters.
The boy's parents did not know what to say. Their son's strange course had been so baffling, their odyssey through the psychiatric wards so bizarre, they could accept anything. They had no choice but to hope that Andrew Pachner was correct.
On the neurology ward, Pachner did a lumbar puncture on the boy, inserting a needle into the midline of his back, passing it between the vertebral bones to the fluid-filled sac called in which the spinal cord floats. Examining the fluid, called cerebrospinal fluid, or CSF, Pachner noted a profusion of immune cells called lymphocytes.
Now he knew he had something. Patients in Europe who had neurological symptoms following arthritic disease showed similar findings in their cerebrospinal fluid.
Those lymphocytes might be the marker for the presence of the Borrelia spirochete. Pachner ordered an intravenous line started on the boy and 20 million units of penicillin to be infused daily for fourteen days.
There was no reason to expect sudden response or improvement. If Pachner was right, if the boy's current depression and compulsive behavior were attributable to a brain infection with the spirochete Borrelia, then the initial infection dated back two years, to his first episode of arthritis. A long standing, deep-seated infection like that could not be expected to be resolved overnight.
But the response was dramatic. Within days of the initiation of therapy, Pachner recalls, "his behavior changed."
The parents were speechless . Even now, Pachner finds it difficult to describe the sensation of watching those first changes in the boy.
"It was like-" Pachner searches for a word, shakes his head, then finally says, "a fairy tale. That's all you can say ."
The boy was discharged. Pachner watched him leave with his parents. Two weeks later, the boy arrived with his parents at Pachner's clinic. He had gained weight, but more important, he was talking again, was more outgoing, and had gone back to school.
Within months the boy was back playing soccer and he was doing his homework. The transformation, or the reclamation, was complete. He was back to normal.
In the process, the understanding of the disease that had been called Lyme arthritis had expanded. The disease was no longer limited to the joints. It would henceforth be called Lyme disease, a disease of many organs, including the brain. IT WAS THE NEW GEAT IMITATOR.
Pachner has reported this new disease in many guises:
[Dr. Pachner] speaks of the subtle differences among the various strains of the spirochete that may cause subtle differences in the damage, the signs, and symptoms of the disease. In his laboratory, he is getting to know the spirochete, or the "bug," as he calls it. He is fascinated by the mysteries:
Deer, for example, do not get sick, although they harbor large numbers of Borrelia organisms. Why? "Host defenses," Pachner says. [How do we know that the deer do not have long-term problems, if they live long enough?] ...
He seems driven by the will to know. He was working on his studies of the Lyme disease patients while he was living the impoverished life of a neurology resident, moonlighting like mad ...
Pachner left Yale for Georgetown in 1987, following Johnathan Pincus, the Yale professor of neurology who had been appointed chairman of the neurology department at Georgetown. Pinicus, author of the classic textbook Behavioral Neurology, was able to attract Pachner offering lab space and freedom to pursue his research interests.
Pachner shows me around his laboratory, of which he is proud. "I remember how scarce lab space was at Yale, how people doubled up and scraped by."
The lab Pachner has at Georgetown would have been considered a land of milk and honey at Yale. Several technicians work for him, and they are busy with lab chores. He has set up an assay for the Lyme antibodies, and a technician shows him some "runs."
The blood samples are sent in from local physicians, and some test positive: There is Lyme disease in the Washington area.
Although Lyme disease is known to occur in may countries, particularly in Europe, and in 45 states in this county, the Mid-Atlantic and New England states have an especially high infestation rate.
The tick that carries the disease, Ixodes dammini, [Ixodes scapularis] clings to deer, field mice, and even dogs. Because the ticks are so small, their human victims are often unaware of having played host to this blood sucker, which may cling for four to six days to an unsuspecting body.
In endemic areas such as certain parts of New England and Washington, any patient who walks into the doctor's office with one side of his face drooping in the classic manner of Bell's palsy should be suspected of Lyme disease. And Bell's palsy is only one common neurologic complication.
Since Pachner's studies called attention to the many sites that may be inhabited by the spirochete, attention has also been focused on heart lesions, which vary from direct attack on the heart-muscle wall - myocarditis - to an attack penetrating every layer of the heart from the inner lining through the heart walls to its coverings -pancarditis.
Patients with Lyme disease can show up at the doctor's office with anything from severe chronic fatigue to arm pain to a variety of palsies to arthritis and skin rashes. Erroneous diagnoses of dementia, multiple sclerosis, psychiatric disease, and arthritis are common, so closely can the great imitator mimic the symptoms of other illnesses.
The diagnosis can be difficult even when the physician suspects Lyme disease. In Pachner's laboratory at Georgetown, blood, spinal fluid, or joint fluid from patients with Lyme disease often fails to yield positive cultures for the spirochete, which is difficult to keep alive outside the body.
While Pachner's laboratory has the highest-quality technicians and antiserums, only about half the patients are positive for the antibody to the B. burgdorferi spirochete early in the course of the disease. And, if the patient happens to be treated with an antibiotic before the diagnosis is made, the antibody test may turn negative while living spirochetes are still reproducing inside the body.
Making matters worse, antibody tests for Lyme disease may be falsely positive in patients who have no Lyme spirochetes but who have instead syphilis or other disease. Special antibody tests have to be done to be sure the doctor is not dealing with a "false positive," in which the test is positive but the patient has no Lyme disease.
Questions have been raised about the wisdom of any pregnant woman in an endemic area such as Washington venturing into wooded areas during tick season. Late spring and early summer are the peak times for the bites that leave the hallmark skin rash, but patients can be infected on any warm day of any month.
There is still no clear evidence about how much risk Lyme disease poses to a developing fetus, but in the absence of hard data, may physicians point to the concept that Andrew Pachner's studies implied: This spirochete behaves in many ways like syphilis, infiltrating along blood vessels. With syphilis as a model, few physicians feel comfortable about the risks for mother and child infected with Lyme disease.
With its many parks running through the heart of the city, with the C&O-Canal running into the heart of Georgetown, Washington is an area in which the country laps up to the front door of suburban and urban dwellers. Deer are common along the canals far into town as Glen Echo and Brookmont on the Maryland-District line and, in Virginia, along the George Washinton Parkway almost to Rosslyn.
Over the coming years, as Washington physicians become more aware of its many guises, more and more cases of Bell's palsy, dementia, fatigue, and arthritis will prove to be Lyme disease.
And there may even be a few boys who have withdrawn from friends and families-boys who are languishing on psychiatric wards-whose blood or spinal fluid will wind up in Andrew Pachner's lab, registering positive. [ What about those who "register" negative on the ELISA or Western Blot?]''
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NEWS ARTICLE from SCOTLAND ON SUNDAY, 9-25-00, By Tom Peterkin, Health Correspondent
``Sheep tick can pass rare skin cancer to people
SCOTTISH doctors have discovered that the humble sheep tick is responsible for passing a rare but deadly form of skin cancer to humans.
The new research has heralded the prospect of the cancer being tackled by antibiotics instead of more aggressive therapies.
Researchers at Raigmore Hospital in Inverness have identified a link between a B-cell lymphoma skin cancer and bacteria transmitted by the parasitic blood-suckers.
It has been known for some time that the bacterium Borrelia burgdorferi is responsible for passing on Lyme disease to humans. But doctors in Inverness are the first to identify a significant relationship between the tick-borne bacterium, which is a distant cousin of syphilis, and cancer.
Dr John Goodlad of the department of pathology at Raigmore Hospital said: "We knew that because there was a high incidence of Lyme disease in the Highlands if there was going to be a relationship between B-cell lymphoma and cancer, we were going to find it here.
"We did a large number of controls, so we know that it was not just a chance result. Our findings showed a statistically significant association and gave us evidence that the organism was present. This is the first time that the link has been shown in the UK and it is certainly the first British study of this."
It is estimated that the Scottish Highlands has one of the highest rates of Lyme disease in Northern Europe. In the north of Scotland, there are 16 cases per 100,000 people each year.
"But that is probably a gross underestimation," said Goodlad. "It is probably much higher than that, because it is not a notifiable disease. Not all cases are reported and no one has done an epidemiological study to look at it thoroughly."
Borrelia burgdorferi triggers the unpleasant symptoms of Lyme disease such as arthritis, inflammation of the heart and brain, and skin rashes.
The disease took its name from the town of Lyme in Connecticut, where it was first recognised in 1975. Since then it has been recorded all over Europe and North America.
But the new link with skin cancer was only revealed when 20 B-cell lymphoma patients were compared with other skin cancer victims at Raigmore.
The researchers found that a significant number of those with lymphoma carried burgdorferi-specific DNA.
The research ... has been published in this month's edition of the American Journal of Surgical Pathology ... ''
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