                                Lyme Disease
                                Lyme Arthritis
                                ~~~~~~~~~~~~~~
          Lyme disease is a 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.
 
 
                                  Symptomatology
                                  ~~~~~~~~~~~~~~
          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,
     occurring 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.
 
 
                                   Etiology
                                   ~~~~~~~~
          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.
 
 
                             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.
 
                              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.
 
          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.
 
                             Therapies:  Standard
                             ~~~~~~~~~~~~~~~~~~~~
          For adults with Lyme disease the antibiotic tetracycline 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.
 
          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.
 
          When Lyme Disease is contracted during pregnancy, treatment with
     penicillin should begin immediately to avoid the possibility of fetal
     abnormalities.
 
          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.
 
 
                         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 July 1989.  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.
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