Rheumatology News, April 1991 Guest Commentary
Rheumatology Research in the 90s By Allen C. Steere
Syphilis now has a competitor for the title
of most complex infection. Because of the neurologic
abnormalities it produces, Lyme disease is reminiscent
of neurosyphilis.
***Once present, the neurologic symptoms follow
a slowly progressive course, in some instances for
10 years or longer.***
Most of these patients have subtle encephalopathy
affecting the central nervous system.
They have memory difficulty, depression,
or sleep distruancesbut no seizures, myoclonus,
or changes in the level of consciousness.
They also have sensory symptoms, such as pain
in the spine, accompanied by radicular pain
in the limbs or trunk, and some havedistal
parethesias with intermittant tingling sensations
in the hands and feet.These symptoms are perilously
close to those that occur in fibromyalgia,
with the chronic fatigue syndrome, or instress-induced
syndromes-conditions that are ever so much
more common than tertiary Lyme disease.
How then does one identify the patient with chronic
neurologic abnormalities of Lyme disease?
The patients in question have characteristic
findings on laboratory evaluations as follows:
almost all were sero-positive by ELISA,
half of them had increased cerebrospinal
fluid (CSF) protein,half had evidence of
slight amounts of production of intrathecalantibody
to the spirochete, and 70% had one or more
of both abnormalities.
In addition, more than 50% had abnormal
EMGs indicating polyneuropathy affecting
both proximal and distal nerve segments,
and MRI brain scans showing areas of increased
T2 signal intensity.
In other words, many of our patients had
memory impairments on their psychological
assessments, had abnormal CSF anaysis,
frequently accompanies by EMG evidence of
an axonal neuropathy.
number of them also had intermittant
attacks of arthritis.
Combined with the evidence of immunity
to Borrelia burgdorferi, this is the clinical
picture that is most suggestive of Lyme disease.
There is some provocative information
that now suggests that Borrelia burgdorferi
infection may cause a multiple sclerosis
like picture [sic].
Dr Rudolf Ackermann in Cologne, Germany
has described 44 such patients.
So far we have seen only one: 6 years after
disease onset, the patient experienced progressive
stiffness and weakness in the muscles of his
right arm and in both legs; tendon jerks were
diffusely brisk, with bilateral ankle clonus
and Babinski sign;
and there were occasional episodes of i
ncontinence.
MRI of the brain revealed numerous small areas
of increased T2 signal intensity in the
periventricular region on the right side.
This scan is compatible with the diagnosis
of multiple sclerosis; however, in the
case of this patient, brain stem and
auditory-evoked potentials were normal,
and he did not have myelin basic protein in CSF.
What *did*suggest Lyme disease was the fact that he
had a serum IgG antibody response to B burgdorferi of
1 to 12,800 and he had evidence of intrathecal antibody
production to the spirochete.
I want to emphasize that it is not yet
proved that B burgdorferi causes this syndrome.
The patient could have two diseases-
Lyme disease and multiple sclerosis.
What we lack is the discovery of the
spirochete from the brain lesions or
the CSF, or perhaps proof of its presence
by polymerase chain reaction (PCR)
amplification of borrelial gene segments-
a technique that is not quite perfected
for use in Lyme disease.
***If B. burgdorferi does cause this syndrome,
it's absolutely amazing that this spirochete
would mimic not only rheumatoid arthritis (RA)
but also multiple sclerosis (MS), two of the most
puzzling and devastating autoimmune diseases.
*****
Now I would like to proceed to the issue of seronegative
Lymedisease. I am convinced this entity exists.***
We have evaluated approximately 200
patients with late Lyme disease in the
past 2 years, and we found that nine, or 5%,
were seronegative by ELISA. This finding
coincides with the figure from Ray Dattwyler, MD,
at Stony Brook (SUNY), who first described
seronegative Lyme disease. He stressed that
this outcome is more likely to occur in
patients who receive antibiotic therapy
during the first several weeks of infection.
Indeed, six of our nine patients (67%)
did receive antibiotic therapy during the
first month of illness, a significantly
higher percentage than in our sero-positive
patients with late Lyme disease.
***I must emphasize the subtlety of the clinical
picture in these sero-negative patients.
Two had erythema migrans followed months
later by very mild episodes of arthritis
lasting only days.
Three of the patients had a subtle
encephalopathy/polyneuropathy picture,
resulting in some memory disturbance
accompanies by slight numbness and
tingling the extremities.
They also have CSF and EMG abnormalities.
**Two of the patients with neck pain had EMGs
that revealed cervical radiculopathy.**
The two final patients had a more generalized
pain syndrome with tender points on examination-
the clinical picture of fibromyalgia.
Three of the patients (one with arthritis
and two with neurologic abnormalities)
had only a cellular immune response to
the spirochete.***
