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His Behavior Was Compulsive

JWissmille jwissmille at aol.com
Fri Aug 3 14:24:14 EST 2001


"I still had all the chronological snobbery of my period...the assumption that
whatever has gone out of date is on that account discredited."--C.S. Lewis"

" Scientific reasoning is a kind of dialogue between the possible and the
actual, between what might be and what is in fact the case." 

Sir Peter Medawar

[Sir Peter Medawar shared the Nobel Prize for Medicine in 1960 for his work on
the immune system and the theory that the immmune system was based  self 
versus non
self -a theory that is coming into question now with the work of  several
immunologists (3 different trials). the immunologists  can't reproduce 
Medawar's results. results] 
_________________________  

Dr. Pachner is now at Robert Wood Johnson with Leonard $igal. 

"...... BUT THE RESPONSE WAS DRAMATIC.
     Within days of the intitiation 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 ."

 ".....Pachner thinks about the bicycle boy and says he was just one of many 
cases. His eyes widen: 'There are so many ways it can present.  And there are 
so many ways it presents that look like bad diseases, that when you identify 
it and your reverse it - YOU FEEL LIKE GOD!'......"

 ".....  He 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...."

source:   The Washingtonian  Jan. 1991

Medicine by Neil Raven

title:  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 sationary 
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 instituition he had 
been unwilling to stop for meals, for school work, for the simple exchnages 
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 sketal, 
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 tallking 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.  Pachner smiles.
     The photo is one of the few clues that this the office of a physician.  
It is a tiny office, crowded with a large ficus tree. More wall space is 
devoted to photographs of his four children than to his diplomas from Yale 
College.  Yale School of Medicine, and Johns Hopkins.  There are photos of 
his wife, scenes of His family cavorting among the goats and cattle on the 
Maryland farm where he lives and from which he commutes daily.  There are 
bookcases jammed with textbooks, but it could be the office of a professor of 
philosophy.
     He 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 psychatists 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 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 clebrated moments in medicine -  
the identification of a new disease.  An arthritis specialist, Allen Steere,  
had become  interested in a group of children in Old Lyme, Connecticut, not 
far from Yale.  The children had  a curious form of arthritis that followed 
the apprearance of a peculiar and characteristic skin rash called erythema 
chronicum migrans, or ECM.
[first described in this contry by Dr. Scrimenti in Wisconsin, in 1970.  -  
georgia]
    Steere had described the condition in 1975.  In 1982, Drs. Willy 
Burgdorfer and Alan Barbour, working at the Rocky Mountain Laboratory in 
Montana, pinpointed the cause of the disease.

    It turned out that Steere's 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 , whcih 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 bessels - 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 Connnecticut 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 behaviour 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.
   Working in Steere's clinic, 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, bizzare 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 bessels 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 unchartered 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 similaiar 
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 penecillin 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 
intitial 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 intitiation 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.  A 21-year-old man 
with a history of violent outbursts, confusion, and wild laughing was thought 
to have a herpes-virus infection of his brain; treated for Lyme disease, he 
returned to normal.  A 55-year-old woman who had gone to her doctor with a 
facial droop was cured after a diagnosis of Lyme disease led to early 
tratment with intravenous penicillin.  A 37-year-old man with fatigue, a sore 
throat, joint and muscle pains, and facial-muscle paralysis who was thought 
to have multiple sclerosis was found to have Lyme disease, and all symptoms 
resolved .  A 61-year-old man with double vision who was thought to have a 
brain tumor was treated for Lyme disease with only partial improvement, 
probably because his disease was too advanced to be cured.  And a 
6-year-old girl suffering from headaches, knee pain, and tingling in her toes 
- and later from vertigo and staggering - was apparently cured after 
treatment for Lyme disease followed positive studies of her blood and 
cerebrospinal fluid.

    Pachner thinks about the bicycle boy and says he was just one of many 
cases. His eyes widen: "There are so many ways it can present.  And there are 
so many ways it presents that look like bad diseases, that when you identify 
it and your reverse it - YOU FEEL LIKE GOD!"
    Pachner finds all this humbling.  He is quick to say that his insight was 
built upon bricks laid by others:  by Allen Steere, who identified the 
disease in those children of Old Lyme; by Louis Reik, the Yale neurology 
resident who preceded Pachner in the arthritis clinic and who convinced  
Pachner that patients with Lyme arthritis actually had neurological problems: 
by the whole stucture of the Yale School of Medicine, which fostered clinical 
investigation and which alllowed neurologists to haunt the arthritis clinic.
   He 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 geting 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.  It comes back to 
the immune system.
    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.
    He never expected financial rewards from his work.  Pachner's father, a 
Czech diplomat before the Second World War, had fought Hitler, and after the 
war he had come to America, but the best job he could get was in a factory.  
The family was not wealthy. Yet they managed to send Andrew to Yale.  His 
widowed mother still doesn't understand what it is that Andrew does at 
Georgetown.  She wishes he would "be a real doctor," which is to say, she 
wishes he would go into private practice.  But that isn't what Pachner has in 
mind.
    There are still too many questions to answer. 

    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 physicans, 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 usnuspecting 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 Georgetwown, 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 
antiseums, only about half the patients are positive for the antibody to the 
B. burgdorferi spirochete early in the couse of the disease.  And, if the 
patient happens to be treated with an antibioitc 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 
postive 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 abscence 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 wil 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. 

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