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$Unique_ID{BRK03948}
$Pretitle{}
$Title{Lyme Disease}
$Subject{Lyme Disease LD Lyme Arthritis Lyme Borreliosis Rheumatoid Arthritis
Brachial Neuritis Parsonnage-Turner Syndrome Bell's Palsy Babesiosis}
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Copyright (C) 1986, 1986, 1987, 1989, 1990, 1991, 1992, 1993 National
Organization for Rare Disorders, Inc.

238:
Lyme Disease

** IMPORTANT **
It is possible the main title of the article (Lyme Disease) is not the
name you expected.  Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.

Synonyms

     LD
     Lyme Arthritis
     Lyme Borreliosis

Information about the following diseases can be found in the Related
Disorders section of this report.

     Rheumatoid Arthritis
     Brachial Neuritis (Parsonnage-Turner Syndrome)
     Bell's Palsy
     Babesiosis

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Lyme disease is an infectious tick-transmitted inflammatory disorder
characterized by an early focal skin lesion, and subsequently a growing red
area on the skin (erythema chronicum migrans or ECM).  The disorder may be
followed weeks later by neurological, heart or joint abnormalities.

Symptoms

The first symptom of Lyme disease is a skin lesion.  Known as erythema
chronicum migrans, or ECM, this usually begins as a red discoloration
(macule) or as an elevated round spot (papule).  The skin lesion usually
appears on an extremity or on the trunk, especially the thigh, buttock or the
under arm.  This spot expands, often with central clearing, to a diameter as
large as 50 cm (c. 12 in.).  Approximately 25% of patients with Lyme disease
report having been bitten at that site by a tiny tick 3 to 32 days before
onset of ECM.  The lesion may be warm to touch.  Soon after onset nearly half
the patients develop multiple smaller lesions without hardened centers.  ECM
generally lasts for a few weeks.  Other types of lesions may subsequently
appear during resolution.  Former skin lesions may reappear faintly,
sometimes before recurrent attacks of arthritis.  Lesions of the mucous
membranes do not occur in Lyme disease.

The most common symptoms accompanying ECM, or preceding it by a few days,
may include malaise, fatigue, chills, fever, headache and stiff neck.  Less
commonly, backache, muscle aches (myalgias), nausea, vomiting, sore throat,
swollen lymph glands, and an enlarged spleen may also be present.

Most symptoms are characteristically intermittent and changing, but
malaise and fatigue may linger for weeks.

Arthritis is present in about half of the patients with ECM, occuring
within weeks to months following onset and lasting as long as 2 years.  Early
in the illness, migratory inflammation of many joints (polyarthritis) without
joint swelling may occur.  Later, longer attacks of swelling and pain in
several large joints, especially the knees, typically recur for several
years.  The knees commonly are much more swollen than painful; they are often
hot, but rarely red.  Baker's cysts (a cyst in the knee) may form and
rupture.

Those symptoms accompanying ECM, especially malaise, fatigue and low-grade
fever, may also precede or accompany recurrent attacks of arthritis.  About
10% of patients develop chronic knee involvement (i.e. unremittent for 6
months or longer).

Neurological abnormalities may develop in about 15% of patients with Lyme
disease within weeks to months following onset of ECM, often before arthritis
occurs.  These abnormalities commonly last for months, and usually resolve
completely.  They include:

     1. lymphocytic meningitis or meningoencephalitis
     2. jerky involuntary movements (chorea)
     3. failure of muscle coordination due to dysfunction of the cerebellum
(cerebellar ataxia)
     4. cranial neuritis including Bell's palsy (a form of facial paralysis)
     5. motor and sensory radiculo-neuritis (symmetric weakness, pain,
strange sensations in the extremities, usually occurring first in the legs)
     6. injury to single nerves causing diminished nerve response
(mononeuritis multiplex)
     7. inflammation of the spinal cord (myelitis).

Abnormalities in the heart muscle (myocardium) occur in approximately 8%
of patients with Lyme disease within weeks of ECM.  They may include
fluctuating degrees of atrioventricular block and, less commonly,
inflammation of the heart sack and heart muscle (myopericarditis) with
reduced blood volume ejected from the left ventricle and an enlarged heart
(cardiomegaly).

When Lyme Disease is contracted during pregnancy, the fetus may or may
not be adversely affected, or may contract congenital Lyme Disease.  In a
study of nineteen pregnant women with Lyme Disease, fourteen had normal
pregnancies and normal babies.

If Lyme Disease is contracted during pregnancy, possible fetal
abnormalities and premature birth can occur.

Causes

Lyme disease is caused by a spirochete bacterium (Borrelia Burgdorferi)
transmitted by a small tick called Ixodes dammini.  The spirochete is
probably injected into the victim's skin or bloodstream at the time of the
insect bite.  After an incubation period of 3 to 32 days, the organism
migrates outward in the skin, is spread through the lymphatic system or is
disseminated by the blood to different body organs or other skin sites.

Lyme Disease was first described in 1909 in European medical journals.
The first outbreak in the United States occurred in the early 1970's in Old
Lyme, Connecticut.  An unusually high incidence of juvenile arthritis in the
area led scientists to investigate and identify the disorder.  In 1981, Dr.
Willy Burgdorfer identified the bacterial spirochete organism (Borrelia
Burgdorferi) which causes this disorder.

Some researchers believe that genetic factors may determine whether a
person with Lyme Disease will be cured with antibiotics, or if they will not
respond to antibiotics and consequently suffer from chronic arthritis.  Their
response is determined by their human leukocyte antigen (HLA) genes located
on the 6th chromosome.

Affected Population

Lyme Disease occurs in wooded areas with populations of mice and deer which
carry ticks, and can be contracted during any season of the year.  Since
first identified in 1975, Lyme Disease has become more common.  In 1989, 7400
cases were reported.  Lyme disease has spread to at least 45 states.  New
York accounts for at least 50 percent of the reported cases.

Related Disorders

Rheumatoid Arthritis is a disorder similar in appearance to Lyme disease.
However, the pain in rheumatoid arthritis is usually more pronounced.
Morning stiffness and symmetric joint swelling more commonly occur in
rheumatoid arthritis, and knotty lumps under the skin may be present over
bony prominences.  Bony decalcification which can be prominent in Rheumatoid
Arthritis is detected on X-rays.  (For more information on Rheumatoid
Arthritis, please see articles in the Prevalent Health Conditions/Concerns
area of NORD Services).

Brachial Neuritis, also known as Parsonnage-Turner Syndrome, is a common
inflammation of a group of nerves that supply the arm, forearm, and hand
(brachial plexus).  It is characterized by severe neck pain in the area above
the collarbone (supraclavicular) that may radiate down the arm and into the
hand.  There also may be weakness and numbness (hyperesthesia) of the fingers
and hands.  Although many cases have no apparent cause, this syndrome may
occur following an immunization (tetanus or diptheria), surgery, or infection
with Lyme Disease.  (For more information on these disorders, choose
"Parsonnage-Turner" as your search term in the Rare Disease Database.

Bell's Palsy is characterized by sudden onset of facial paralysis
resulting from a decreased blood supply to part of the head and compression
of the facial nerve.  It occurs rapidly over several hours, sometimes
following exposure to cold or draft.  A slight fever, pain behind the ear, a
stiff neck, and unilateral facial weakness and stiffness are among the
earliest symptoms.

Babesiosis is an infection carried and transmitted by deer ticks.  It can
cause disease when the tick attaches to humans.  Symptoms include a
malaria-like illness, fever, lack of appetite, headache, chills, stomach pain,
vomiting, and diarrhea.  In most people the diseases causes mild symptoms or
no symptoms at all.  However, in very young children, the elderly and
immunosuppressed persons the disease can be life-threatening if left
untreated.  (For more information on this disorder, choose "Babesiosis" as
your search term in the Rare Disease Database.)

Therapies:  Standard

For adults with Lyme disease the antibiotic tetracycline Doxycycline and
minocycline is the drug of choice.  Penicillin V and erythromycin have also
been used.  In children penicillin V is recommended rather than tetracycline.
Penicillin V is now recommended for neurological abnormalities.  It is not
yet clear whether antibiotic treatment is helpful later in the illness when
arthritis is the most predominant symptom.  Treatment should be started as
soon as the rash appears, even before the Enzyme Linked Immunoabsorbent Assay
(ELISA) test is completed.  Results of this test may be inaccurate if
patients have had antibiotics soon after contracting Lyme Disease, or in
those who have weakened immune systems.

If Lyme Disease is contracted during pregnancy, careful monitoring by
physicians is highly recommended to avoid possible fetal abnormalities and/or
complications.  Treatment with penicillin should begin immediately to avoid
the possibility of fetal abnormalities.

For tense knee joints due to increased fluid flowing in the joint spaces
(effusions), the use of crutches is often helpful.  Aspiration of fluid and
injection of a corticosteroid may be beneficial.  If the patient with Lyme
disease has marked functional limitation, excision of the membrane lining the
joint (synovectomy) may be performed for chronic (6 months or more despite
therapy) knee effusions, but spontaneous remission can occur after more than
a year of continuous knee involvement.

In 1989 a new Lyme Disease antibody test, manufactured by Cambridge
Biosciences Corp., was approved by the FDA.  This test is being used by local
laboratories throughout the nation, making tests more available to the
general population.  However, it is 97% specific for antibodies to Lyme
disease when compared to Western blot tests, but it cannot identify the live
bacteria in patients who have not yet developed the antibodies.

Lyme Disease may reoccur in some patients resulting in chronic neurologic
disorders such as Polyneuropathy and Encephalopathy.  These after-effects are
treated with antibiotics.

Therapies:  Investigational

Researchers are trying to develop a test that will identify the Lyme disease
bacteria in patients who have not yet developed the antibodies.  This would
enable doctors to diagnose Lyme disease very early in the course of the
illness.

This disease entry is based upon medical information available through
September 1992.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Lyme Disease, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     Lyme Disease Foundation, Inc.
     P.O. Box 462
     384 Merrow Rd.
     Tolland, CT  06084-0462
     [phone]

     American Lyme Disease Foundation, Inc.
     Royal Executive Park, 3 International Dr.
     Rye Brook, NY  10573
     [phone]
     (800) 876-LYME

     The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
     Box AMS
     Bethesda, MD  20892
     [phone]

     Lyme Disease Clinic
     Yale New Haven Hospital
     333 Cedar St.
     New Haven, CT  06510

     Lyme Disease Clinic
     Marshfield Clinic
     1000 North Oak Ave.
     Marshfield, WI  54449

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA  30333
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  P. 1251.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 1726-9.

ASSOCIATION OF CHRONIC LYME ARTHRITIS WITH HLA-DR4 AND HLA-DR2, Allen C.
Steere, et al.; N. Eng. J. Med, (July 26, 1990, issue 323 (4)).  Pp. 219-223.

CHRONIC NEUROLOGIC MANIFESTATIONS OF LYME DISEASE, Eric L. Logigian, M.D., et
al.; N Eng J Med, (November 22, 1990, issue 323 (21)).  Pp. 1438-1444.