When to Suspect Lyme
By John D. Bleiweiss, M.D

Traditionally, the public has been advised to suspect Lyme (LD) if a round or oval, expanding, red rash develops 3-32 days after a deer tick bite associated with or followed by a flu-like illness. This limited description will apply to only some cases. About 50% of patients do not recall one or more of tick bite, rash or flu-like illness. The rashes associated with LD can assume a variety of morphologies including vesicular, urticarial, eczematoid or atrophic (Acrodermatitis Chronicum Atrophicans). For many patients, neurologic, cardiac, arthritic, cognitive and/or psychological complications predominate. While deer ticks and LD have a well known affiliation, other potential vectors can carry the spirochete that causes LD (Borrelia burgdorferi; Bb). These include, the lone star tick, fleas, the biting flies (e.g. green-headed fly) (and mosquitoes?). A case of suspected transmission via blood transfusion has been reported by Dr. Burrascano.

The demonstration of Bb by PCR in two museum mouse specimens dating from 1894 (Massachusetts) and in ticks collected during WW II, provides a mechanism for potential life long exposure and disease which predates the formal 1975 discovery of LD. An occasional patient will date their symptoms which resolved on antibiotic therapy for LD to early childhood. Before the diagnosis was made, patients would dismiss those symptoms with the statement: “I’ve always had those problems”. That resigned characterization implies that the longevity of the symptoms rules out a reversible cause. Subsequent resolution of the long standing symptoms on antibiotic therapy for LD belies that notion. Symptoms of LD can begin within days of inoculation with Bb or appear belatedly, but usually in the first to fourth month. Mice inoculated intraperitoneally had Bb demonstrated in the brain on biopsy 12 hours later with a peak at 48 hours (Stockholm Conference, 1990). Dr. Luft has published the detection of Bb by PCR (Polymerase Chain Reaction) in the CSF (Cerebrospinal fluid) of humans 2 weeks after the appearance of non-CNS related symptoms!

If dissemination can occur early, then staging the disease according to the temporal appearance of symptoms may be irrelevant. The absence of symptoms related to a particular organ system doesn’t necessarily exclude the presence of Bb from that organ. Conversely, due to the possibility of symptoms being engendered by chemical mediators and autoimmune reactions by the host (against non-viable but immunoreactive DNA blebs), organ dysfunction and attendant symptoms can appear at sites removed from the actual spirochetes. The diagnostic and therapeutic problems that these phenomenon entail should be obvious.

Rapid dispersion of Bb could lead to the prompt appearance of complications; e.g., meningitis. There is no absolutely predictable clinical sequence for LD. The flu-like syndrome may be absent from the initial presentation and may endure once established without treatment. Cardiac and neurologic complications can be observed sometime within the first 3 months after microbiologically contracting the disease. Arthritis (i.e., joint inflammation; distinct from arthralgias; i.e., joint pain) can also accompany the initial clinical course, but more often develops later on between the second and sixth month from inoculation. The onset of complaints can not only be subtle and desultory, but delayed for a year or more. One of my patients denied all LD related symptoms until her husband died, whereupon, a plethora of complaints cascaded into her life beginning that very day. Another had an annual flare of LD as part of an anniversary reaction centered on the date of his mother’s death.

Moreover, the early constellation of symptoms can have a paucity of findings with unidimensional presentations: the onset of solitary problems such as vertigo, or recurrent upper respiratory tract infections. Over time, as the untreated LD percolates, symptoms accrue to the burgeoning clinical picture until a multisystem presentation is created. Other patients can have their manifold symptoms complex develop in the manner of an avalanche. These patterns represent the extremes of a clinical continuum between which there are many variations on the theme ranging from mild to severe disease. Thus, The failure of a pathognomonic (unique and specific) presentation to consistently unfold causes sufficient clinical confusion, that a punctual diagnosis is problematic. Therefore, a high index of suspicion is placed at a premium. If a clinician can’t reconcile preconceived notions about how LD should announce itself with a patient’s history and physical findings, it is a disservice to the patient and an abdication of professional imperatives to presumptuously conclude that the symptoms are psychosomatic or that the patient is faking!

Antecedent or concomitant factors which can cause symptoms and physical changes de novo or aggravate contemporary problems are reliably solicited on close questioning of LD patients. Females experience an exacerbation of their LD symptoms before or during their menses, while pregnant and with oral contraceptive hormones. Many patients have symptoms intensify or reappear with physical and emotional stress, if sleep deprived, after exercise, in a hot bath, after alcohol consumption, with fasting (hypoglycemia) or dehydration. Humidity, low barometric pressure, cold or rainy weather can elicit arthralgias, fatigue, encephalopathy or headache. A cold draft of air can precipitate the appearance of pain in exposed skin and the underlying bone, or even VII cranial nerve (Bell’s) palsy. Patients with poor control of symptoms abhor the extremes of ambient temperature. Typically, heat intolerance is revealed as irritability, headache, excessive perspiration or sleepiness. Photophobia can enhance the somnolent propensity that can occur while driving a car. Significant head trauma has incited severe symptoms that later resolved with antibiotic therapy for LD. A sudden acceleration of encephalopathy (see below), headache and dizziness, thought of as putative post-concussion syndrome, can be evoked by head trauma. Diagnostic inaccuracy will be minimized by not indolently attributing all problems following head trauma to the most obvious cause. I routinely advise my patients with LD to abstain from cigarettes, alcohol and steroids because therapeutic inadequacy or an avoidably prolonged convalescence is frequent (Dattwyler, RJ, Lancet 1:687, 1987 – on steroid use). Patients have described clinical deterioration when steroids were used fortuitously or intentionally when hypoadrenalism was absent. Another hazard attending palliative steroid use is that some symptoms will be concealed, rendering the clinical picture less interpretable. In a private communication, a physician related that one of his LD patients succumbed to fatal cardiomyopathy after receiving steroids. One helpful caveat is to avoid the use of electric blankets or sleeping in water beds with the electric current activated, otherwise you might wake up with one or more LD symptoms. Allergic and chemical hypersensitivities can enhance or cause symptoms to emerge temporarily.

Symptoms vary stereotypically during the day. Joint stiffness and “brain fog” are often reported on rising in the AM (but not solely in the AM). Fatigue can be unrelieved by sleep, or develop between noon and 4 PM, whereupon a short nap provides refreshment. “Madman Syndrome” (explosive irritability) may appear toward the end of stressful work period or late in the evening. A “mad face” can herald imminent detonation.

Prior to proper diagnosis, patients habitually report that they were assigned the following diagnoses most often: Chronic Fatigue Syndrome, Multiple Sclerosis, Fibromyalgia, Lupus, Candidiasis, Chronic mononucleosis, Hypoglycemia, and Stress-related illness. If these appear in a differential diagnosis, then LD should be considered.

On cursory inspection, many patients with LD appear deceptively well but in fact feel awful Don’t be fooled! The mien of a Lyme patient ranges from phlegmatic, sullen, staring off into space, to one of agitated anxiety and hyperkineticism. Their oral and written recitations similarly vary from cryptic to being overinclusive and circumstantial. Geschwind’s Syndrome embraces some varieties of LD encephalopathy precisely (Textbook of Internal Medicine, Ed: Kelly, 1989, p. 2509). Stuttering was reported by several patients to coincide with the onset of their LD and often proved reversible.

Patients most frequently report fatigue that varies from mild to debilitating. Usually there is a loss of interest and initiative so that lounging around becomes habitual. This derives not from laziness, but results from lassitude. Attempts to indulge avocational or vocational pursuits is frequently interdicted by either the languor of Lyme or by encephalopathy. There is a tendency to nap, sleep that is not rejuvenating, and hypersomnolence at inopportune moments; e.g., in the classroom or during a favorite pastime. Sleeping away entire days is not unknown.

Paradoxically, at usual bedtimes, patients often experience insomnia or frequent awakenings. Sleep does not always provide respite as ferocious or vivid nightmares can occur. Childhood night terrors can be due to LD or more mundane causes.

Intermittent fevers range from low grade to 104.5 degrees F. The typical context for high fevers is in the first week of antibiotic therapy especially if multiple agents and/or IV drugs are used. While fever is the hallmark of the classic Jarisch-Herxheimer (J-H) reaction, its appearance is inconstant. Compared with most causes of high fever, the patient can look and feel their best during or shortly after the fever with a relatively non-toxic demeanor. This can sometimes be diagnostically helpful. The differential diagnosis of febrile seizures in infants should include LD. Many LD patients have routinely subnormal body temperatures so that the appearance of a temperature of 98.6 degrees F may be compatible with a low grade fever analogous to diabetics.

Very often, the pinna and ear lobes are varying shades of red. Less commonly, a similar erythema can be observed on the hands or malar (upper cheeks) areas. A malar rash is not pathognomonic of Lupus, if in fact SLE is distinct from LD (Abstract 55A, V LD Symposium). Fifth Disease (slapped face) is suspected of being due to LD. Lymphocytoma of the ear lobes has been encountered more often in Europe. Cold hands and feet even in warm environments occurs and some patients have Raynaud’s phenomenon. Potentially contributing to this vasoconstriction are excessive levels of vasoconstricting hormones, magnesium and potassium deficiency, limbic or hypothalamic dysfunction due to CNS infection, local inflammation of capillary sphincter or hypothyroidism. Eczema and psoriasis can appear in conjunction with LD. A female LD patient had generalized psoriasis covering 40% of her body. Antibiotics for LD gave total relief.

Alterations of cutaneous sensation are very common. Most often there is numbness and tingling (paraesthesias)of the central face, fingertips, scalp and in the extremities. Muscle twitching usually occurs in the eyelids and extremities. Tremors, myoclonic jerking of entire extremities or truncal shudders can suggest pseudoseizures but is attributed to neuritis. Patients also report electric shocks, dysesthesias (abnormal sensory responses to stimuli), painful or itchy skin and flushing. An inordinate amount of exertional or non-exertional sweating may be described in the absence of hyperthyroidism. One of my patients experience anhydrosis (inability to sweat) for 27 years until antibiotics were given for LD.

Dizziness, imbalance and clumsiness can become very frustrating as patients drop objects or knock them over, trip a lot, turn into the wall when rounding corners, and develop sloppy and slower handwriting.

Many use the phrase “a vibration in my head”. Others remark that they feel “toxic”. Along with the “brain fog”, these colloquialisms connote LD until proven otherwise.

Eventually the majority, but not all, complain of one or more of “foggy brain”, forgetfulness, anxiety, mood swings, loss of initiative, depression, impairment of concentration, inattention, easy confusion or disorientation when attempting intellectual tasks. Paper shuffling can be the end result when patients attempt to organize or assimilate even limited amounts of information. These problems, and the others described below, constitute the salient features of Lyme encephalopathy.

Short term memory impairment causes patients to forget what they were going to say, why they entered a room, where objects were placed, the previous sentence or plot content, calendar dates, their schedules, names and faces of familiar people, even family members. Cognitive neglect caused one patient to wander around the room looking for the room looking for the pencil clenched between his teeth. A mother left her infant and baby carriage in my office parking lot and went home. Others forgot how to spell even simple words, how to read or must re-read with varying degrees of comprehension. One patient drove to Philadelphia instead of the desired Princeton destination because the initial letters were identical and confused him. After shopping for groceries, another patient placed her shoes in the refrigerator and stored the food in the clothes closet. Lyme patients can lose their way home or on the way to work, bypassing otherwise familiar exits or plain forgetting where they are in time and space or how they got there. This is known as topographical disorientation or environmental agnosia. Elementary math problems may prove insurmountable. numerical errors are common. Sequential task performance is compromised in Lyme. Lyme patients have a penchant for saying, “Wait a minute”, 2-3 times rapidly when the only demand on them is to record a phone number, which also speaks of perseveration.

Inattention frequently characterizes the way patients relate to the world. Some patients participate passively, unable to initiate or engage in the usual forms of social and intellectual exchange. Verbal and written forms of expression have a typical Lyme flavor. The content demonstrates disorganization, an inability to follow a train of thought and there is a proclivity to ramble on and on in great detail which propels further confusion amongst the forest of details. Ubiquitous among the myriad cognitive flaws are the frequent errors of word selection or pronunciation and the consistent word and number reversals.

Concentration on a task can be problematic because attention span is abbreviated. As increasing amounts of information have to be processed, the Lyme patient becomes proportionally lost, disoriented, frustrated, fatigued and finally must desist from further intellectual activity. The desire to initiate projects and social interaction is often blunted if not absent altogether. Thus a deterioration in academic and vocational performance is a frequent manifestation of LD in children and adults..

Miklossy (NeuroReport 4:841-848, 1993) reported the detection of Bb spirochetes on dark-field microscopic examination of post-mortem brain biopsy specimens FROM PATIENTS WITH ALZHEIMER’S DISEASE! CSF and blood cultures grew out Bb from those cases. In my view, a child assigned a diagnosis of Attention Deficit Hyperactivity Syndrome (ADH) or PNI (Perceptual Neurologic Impairment) should be evaluated for LD. A 16 year old boy whose Tourette’s Syndrome began at age 5, had Osp A antigen detected in his CSF. LD treatment resolved the Tourette manifestations. Another patient of mine with ADH had a positive IgM Lyme antibody in the serum. The manifestations of ADH were eradicated while on antibiotics. Distinguishing causality from mere exacerbation of ADH or Tourette by Lyme is moot, and therefore, I suggest an evaluation LD for these patients. Parenthetically, the boy with Tourette also had cognitive impairment, familial nephritis with early renal insufficiency and OCD (Obsessive Compulsive Disorder). These clinical features all remitted with antibiotics! A real estate agent’s prominent encephalopathy resolved with LD treatment whereupon his commercial output jumped to a record zenith and became the recipient of numerous corporate awards. The benefits of arresting LD are self evident to him.

Personality changes are nearly universal in Lyme encephalopathy with emotional and expressive incontinence being typical. Usually there is a baseline irritability which fluctuates. Patients with LD encephalopathy react to even mild degrees of stress with frustration, anger or crying spells out of proportion to the situation. Emotions can reach escape velocity and rages can become volcanic with a momentum beyond volitional control. Unpleasantness is inevitable due to volatile tempers, super critical dispositions, and impatience with themselves or others. Lyme patients can be easily irritated by anyone just walking into the same room even though eye contact is never made or words exchanged. Low threshold exasperation in unexpected circumstances is not uncommon. Thus a parent responds to an infant’s needs with anger and frustration. Perpetrators of “shaken baby syndrome” recapitulate an emotional response indistinguishable from that of a Lyme patient whose encephalopathy is out of control.

Many express morbid fears of occult illness, impending death and can be generally pessimistic or maudlin. Some develop intricate paranoid theories regarding imagined conspiracies against them. Lyme patients often evince a tendency for being overly sentimental. Hyperbolic thought finds expression in obstinacy, self-righteousness, being contentious, speaking in categoricals, and inappropriate and atypical vulgarity. Internalized anxiety results in the perception of being hurried even without a deadline or the inability to remain calm when there is no reason for not feeling calm. Panic attacks are the extreme of this anxious state and should arouse a suspicion of LD. I suspect that in addition to CNS infection of the limbic system, these phenomenon could also be the result of elevated adrenaline levels, Mg++ deficiency or hypoglycemia. A rare LD patient will admit to agoraphobia or claustrophobia.

Depression alternating with anxiety is very common in LD. Psychiatrists should routinely evaluate a depressed patient for LD before/when initiating psychotropic medication. A patient with LD may have a plausible reason to be depressed, but their emotional response can not only be incongruous with their usual coping style but also the depression can be ablated or ameliorated with control of the LD infection. Lyme encephalopathy typically vitiates an otherwise mature and functional emotional repertoire.

With a loss of voluntary and subconscious editorial control of emotions and expression in word or behavior, a patient with encephalopathy gives the general impression of being erratic, inappropriate, if not dysfunctional. Less frequently, mania, obsessive-compulsiveness, schizoaffective disorders and homicidal/suicidal ideation is encountered and has been reported. Fatal attractions are consistent with the LD style.

Adolescent hormonal surges and the emotional turmoil wrought by LD at once camouflage and exacerbate each other mutually. Thus, children tend to be unruly, hard to please and prone to atypical emotional reactions. A child who is misbehaving in class should not be dismissed as a “bad kid”. Lyme can catalyze inappropriate behavior and commentary. Many patients retrospectively realize that they were out of control but in the event were unable to intercept their behavior. Misattribution as to the origin of behavioral perturbations is the rule. The development of aberrant personality traits can be gradual or even situational, further obscuring the medical etiology. An acute break from normal behavior can serve to highlight the abnormalities and suggest the need for evaluation. thus, dysfunctional behavior and intellectual incapacitation are bitterly recalled by LD patients when they finally realize how their interpersonal relationships, school and vocational conduct were negatively impacted.

Clinically, there is considerable overlap between LD and Chronic Fatigue Syndrome (CFS). In their masterful review of the pathophysiology of CFS, Drs. Rosenbaum and Susser (Solving the Puzzle of CFS, 1992) describe SPECT scan findings of brain perfusion (blood) in CFS patients. Hypoperfusion of the left temporal, right parietal and left frontal lobes were consistently seen. These regions control the very areas of functioning which are abnormal in both LD and CFS, namely verbal capacity, memory, emotions and higher order information processing. Dr. Jay Goldstein has proposed the term “limbic encephalopathy” to describe the disorders of memory, appetite, temperature and appetite regulation, libido and hormonal homeostasis seen in CFS. He advances the concept of an “agent X” which incites CFS and causes dysregulation of the immune system and the limbic components of the CNS. I feel “agent X” is Bb. I am encouraged in this view by anecdotal experience whereby CFS responded to antibiotics given either fortuitously or by way of Lyme treatment, due to positive serologies (antibody tests) for LD in some CFS patients and the development of a J-H reaction in CFS patients who take antibiotics. Crimson crescents, portrayed as diagnostic for CFS, I have detected in LD where the diagnosis was secure.

Limbic encephalopathy elegantly embraces the preceding observations in Lyme encephalopathy. It is a potential mechanism to explain my suggestion that LD can be responsible for anorexia nervosa/bulimia. Anorexia was documented in earlier studies on LD. Uncinate fits and are characterized by hypersexuality, rage states and vulgarity, are compatible with LD. Indeed, disinhibition, the release of the usual brakes on behavior, would be part of limbic dysfunction.

LD could cause reversible disturbances in brain physiology through cytokine mediators, direct infection; e.g., encephalitis and perivasculitis, demyelination, metabolic aberrations within the CNS such as regional hypoperfusion, altered rheologic (flow) characteristics of blood, intracellular acidosis and the depletion of ATP. Permanent changes may include demyelination or loss of neurons leading to atrophy.

Neurologic complications in earlier reports were said to occur in 20% of LD cases. In my experience, and as published by Dr. Logigian, 90% of patients have one or more of encephalopathy, cranial neuritis or psychiatric changes. Early in the course of LD, these problems may be absent or muted, but eventually intrude and can become dominant aspects of LD.

Many patients are told that they have Multiple Sclerosis (MS) because of brain MRI findings or a spinal tap was positive for oligoclonal bands (OCB) or myelin basic protein (MBP). The medical literature is quite emphatic that MRI does not reliably distinguish between MS and LD because there is too much overlap in their supposedly distinct appearance and location of plaques. Plaques have been detected with both disorders in the brain and spinal cord. OCB’s and MBP are non-specific markers for demyelination (loss of sheath around nerves) and do not signify a cause of the demyelination. In Miklossy’s study above, senile plaques stained avidly for Bb spirochetes. Vincent Marshall reviewed the MD literature in Medical Hypothesis (Vol 25: 89-92, 1988) and advances the notion that LD is causing MS! His survey revealed that multiple studies prior to 1951 were able to demonstrate spirochetes in the spinal fluid of MS patients (by inoculation into animals and on silver stain of CNS tissues). Dr. Coyle has documented the presence of antibodies to Bb in MS patients (Neurology Vol. 39:760-763, 1989). The encephalopathy attributed to MS is very reminiscent of LD. Both MS and LD are associated with sinusitis (Lancet, 1986). Dr. Leigner has reported a case of LD which fulfilled all criteria for MS. The epidemiology of MS and the geographic distribution parallels that of LD. The symptoms of both LD and MS can be aggravated if the patient takes a hot bath. Anecdotally, patients with LD, who previously had been identified as MS, responded to antibiotic therapy.

LD has been documented to cause strokes, paralysis, a variety of seizures, transient or permanent blindness, Parkinsonian-like movement disorders, motor and/or sensory neuropathies, mononeuritis multiplex, radiculoneuritic pains, meningitis and encephalitis. It has been affiliated with Lou Gehrig’s disease and the Guillain-Barre Syndrome.

Recent reports suggest that the spectrum of neurologic LD is actually similar in both Europe and the USA (Jacqueline, MS; Surv Opthalmol 35:191-204, 1990) and belies the assertion that European LD research holds no relevance for LD in the USA.

The more commonly noticed neurologic deficits involve one or more cranial nerves (I thru XII), most often the sensory divisions of the trigeminal (V) and the motor components of the facial (VII) nerves in my patients. In declining order, deficits to pain sense are detected in V2, V3, and V1. V2 neuritis appears as paraesthesias or dullness in the central face and cheeks. Gum and tooth pain can be another manifestation of trigeminal neuritis. Rule out dental abscess or sinusitis which can present with similar tooth pain.

The most common cranial neuritis I see is that of the VII nerve. Abnormalities of the VII nerve can be varied. Usually there is symmetry of the central facial creases, the lips at rest or in motion, or overt deviation of the mouth or smile to one side. Colleagues have dismissed these asymmetries as normal findings, saying “well, everyone has those”. I feel there is significance when antibiotics cause these so-called innate or normal findings to resolve.

When Bell’s palsy is present, there are the facial defects described above for VII neuritis plus a wider eye on the same side as an elevated eyebrow, often attended by complaints of tearing and drooling (usually at night) on the affected side. 10.6% of 951 LD cases were found with Bell’s palsy and 25% of those have had bilateral Bell’s palsy (Clark, JR et al. Laryngoscope 1985: 95: 1341-45). Bilateral Bell’s promulgated as pathognomonic for LD, actually can be associated with intrapontine lesions, diabetes mellitus, syphilis, sarcoid, leukemia, Guillain-Barre, viruses or diphtheria. Considering the incidence of Bell’s palsy in LD, it is improper to treat it as viral in origin without a work-up for LD.

Incidentally, hyperaccusis (sound sensitivity) can be a feature of VII neuritis. Olfactory neuritis (I) is attended by dysosmia (unusual smells). Neuritis of the III, IV and VI cranial nerves will show up as double vision. When the VIII nerve is involved, vertigo and impaired hearing can result. I have had at least two cases of Meniere’s Disease respond to treatment for LD. Dysphagia (difficulty swallowing) can be associated with X neuritis but not invariably. More often in my experience, a deviated uvula or soft palate is perceived. Dysphonia (altered voice) can occur with X neuritis when the branches that serve the larynx are affected. Recurrent laryngeal nerve paralysis has been seen with LD (Schroeter, V. et al. Lancet 2:1245, 1988). IX neuritis (glossopharyngeal) can cause a unilateral sore throat which was reported by 3 of my patients. XI neuritis (spinal accessory) presents as trapezial or sternocleidomastoid muscle weakness resulting in a drooped shoulder or weakness on resisted head rotation respectively. Do not confuse this with a unilateral dystonia where the affected shoulder girdle will be elevated with preserved motor strength on both sides. Hypoglossal (XII) neuritis can be associated with a heaped up tongue on the unaffected side or deviation of the tongue on protrusion toward the abnormal side.

LD related headaches can have a wide variety of patterns and can broadcast the early onset of LD or a flare of LD. The headaches incorporate the characteristics of migraines, muscle tension or cervical/radicular headaches. Pseudotumor cerebri (elevated CSF pressure with normal CSF analysis in the absence of intracranial masses) can complicate the course of LD and cause headaches. Papilledema (swelling) of the optic disc is usually present when CSF pressures are elevated, but not invariable. A spinal tap will have high opening pressures and be therapeutic as well. Depending on the characteristics of the headaches, sinusitis and brain tumors may have to be ruled out. Cluster headaches have characteristics compatible with some LD headaches including responsiveness to 100% oxygen.

Immunosuppression due to LD has been reported. Therefore, it is not surprising that recurrent or intractable upper respiratory tract infections (URI’s) have been noted. LD can cause or worsen pre-existing sinusitis, asthma, bronchitis, otitis, mastoiditis. Frequently, the pediatric history of LD contains a pattern of repetitive URI’s. Mastoiditis can also be associated with a Bell’s Palsy. LD can be affiliated with the appearance of new onset allergies for the first time in a patient’s life or magnify an atopic predisposition. The usual medications for sinusitis and allergies will have a predictably diminished effect, when LD is operant.

Another concomitant reported by an incidental patient is the occurrence of motion sickness which can be reversed with LD treatment.

Eye related problems in LD are commonplace and can include conjunctivitis, ocular myalgias, keratitis, episcleritis, optic neuritis, pars planitis, uveitis, iritis, transient or permanent blindness, temporal arteritis, vitritis and periorbital edema (Jacqueline MS; Ibid). Horner’s syndrome, ocular myasthenia gravis, and an Argyll-Robertson pupil are also reported. Optic neuritis has been observed to become recurrent or intractable after treatment with steroids. Given the earlier remarks about the detrimental effects of steroids on LD, recidivous optic neuritis may be due to occult LD.

Lyme hepatitis occurs in approximately 15-20% of patients. Liver tenderness is inconstant and the elevated liver enzymes respond to antibiotics. Sometimes, the hepatitis appears temporarily in the early phases of treatment with subsequent resolution.

In many of my patients, cysts are found not uncommonly in various locations: thyroid, breast, liver, bone, ovary, skin, pineal gland, and kidney. Some forms of Polycystic Kidney and Fibrocystic Breast Disease may be LD manifestations.

LD can cause an interstitial cystis leading to bladder pain relieved by urination. A neurogenic bladder can develop with either hesitancy, frequency, loss of bladder awareness, urinary retention, incontinence or the symptoms of UTI (urinary tract infection). I suspect that some cases of chronic pyelonephritis are actually LD. Pediatricians may want to consider that nocturnal enuresis (bedwetting) is secondary to LD.

Constipation severe enough to cause fecal impaction can occur. Many LD patients will experience a spastic (irritable) colon and that diagnosis should spark a search for LD. I have treated LD attended by ulcerative colitis with substantial remission of the colitis when antibiotics were inaugurated. Fecal incontinence due to impaired rectal sphincter tone can occur. Dr. Martin Fried has demonstrated Bb spirochete in the gastric and duodenal mucosa of children with LD who complained of abdominal pain and who were documented to have gastritis and/or duodenitis. It is appropriate to work up LD when confronted by these clinical entities.

Among untreated patients with LD, arthritis can ultimately develop in up to 60%. The joint swelling, which may or may not be painful, frequently is episodic, recurrent and migratory if multiple joints are involved. Any joint can be affected including the TMJ (temporomandibular) and small joints of the fingers (contrary to earlier reports). Up to 10% of untreated LD arthritis can develop into destructive/deforming synovitis almost identical to Rheumatoid Arthritis (RA). Dr. Lavoie has published the coincident findings of LD with RA, and SLE (lupus) with LD. The SLE was associated with positive DS-DNA (double stranded DNA) which is considered diagnostic for lupus. This marker improved with antibiotic treatment for the LD. The author felt that the LD might be causing/aggravating the SLE.

I would like to present a case of a 38 y.o. white female who, 24 days postpartum, presented with Seropositive LD. She had a prior history of a round rash 6″ diameter in 6/90, DS-DNA =320 in 12/90 and previously treated Lyme (seronegative) by various physicians (including myself) from 12/90 to 12/92 with eradication of all symptoms. The patient became pregnant 7/93 and her former Lyme symptoms were revived. She declined to take amoxil prescribed by her OB-GYN until 11/93. this was followed by 10 days of Zithromax with minimal results. The exacerbation accelerated about 2 weeks prior to her 3/28/94 visit to me. In addition to Lyme, her contemporary problems included SLE, episodic Sjogren’s Syndrome (dry mouth/eyes, parotiditis), anticardiolipin antibodies without thrombotic events, polyarthritis (in ankles, knees, hips, shoulders and PIP’s of the fingers), a left IX neuritis, a right Bell’s Palsy, ptosis of the left upper eyelid, myositis of the rectus abdominus muscle, generalized fibrositis and periostitis, degenerative changes in several finger joints and a few costochondral joints, and fibrocystic breast disease. Encephalopathy was absent! The lab findings included: Lyme Elisa IgG = 1.4 (+), Lyme IgM = 320 (++), Lyme LgM by IFA = 640 (+++), a NEGATIVE Western Blot (only 41 KDA) and IgG by IFA for LD, positive Lyme IgG Antibody in synovial fluid (Class II exudate) obtained from the right knee = 1.94, DS-DNA = 5, 120 (less than 10 is normal), ANA = 2,560 (+++), positive Sjogren antibody (SSB = 33), positive anticardiolipin antibodies (IgG = 66, IgM = 44.9), leukopenia (wbc = 2,800), and anemia (Hgb = 11.4), 10 bands and 1736 PMN’s on CBC differential, ESR = 32, slightly depressed complement levels: C3 = 55, C4 = 14. Lyme PCR (a test for the DNA of Bb) in synovial fluid was negative. Concurrent vaginal bleeding prevented us from obtaining an immediate U/A.

Other lab tests : Fe = 34, Ferritin = 64, transferrin saturation = 9.36%, Bun = 18, Creatine – 0.9, Rheumatoid Factor and SSA negative; thyroid and coagulation profiles, TSH and PTH normal; CH50 = 222, CXR and EKG noncontributory.

The patient acutely developed (after using Motrin on her own, instead of the prescribed Percocet for relief of arthralgias while the work up was in progress) leukopenia (wbc = 1,700),neutropenia (PMN’s = 1,074), and an acute hepatitis with abnormal liver enzymes (LFT’s) : Total Bilirubin = 1.58, alkaline phosphatase = 328, LDH = 344, GGTP = 113, SGOT = 246, SGPT = 285.

Within 72 hours of stopping Motrin use, the wbc rebounded to 4,700 but by then the patient had developed an acute partial paralysis of her legs (3/5), with deltoid, biceps and vastus lateralis myositis, a new left VII neuritis, and the polyarthritis was more ebullient and painful. A fever of 101 degrees F had appeared. Subsequently, hypertension (BP = 154/94), a small posterior pericardial effusion and hypokalemia (K+ = 3.3 mmol/L) were appreciated. Urine and Blood Cultures were negative. Urine analysis (U/A) contained 30-35 wbc, but proteinuria and cylindruria were absent. Elevated LFT’s were again encountered. The patient declined, at various times: an MRI of brain, a bone marrow aspiration, spinal tap, EEG, and EMG.

On admission to the hospital, IV Claforan was initiated. At 41 hours, a crescendo J-H reaction attained a fever apex of 103 degrees F, but the wbc count again fell to a nadir of 1,800 with neutropenia (67.2% = 1200 PMN’s). As the patient gradually defervesced (temp = 100 degrees F at 75 hours), the wbc count slowly ascended to 2,700 (PMN’s = 2100) in spite of continued Claforan, The paralysis remitted over the initial 48 hours. concomitantly, the LFT’s normalized rapidly, the Sjogren manifestations and cranial neuropathies disappeared, the BP became 120/70, muscle tenderness was reduced and the U/A was devoid of abnormalities.

By discharge to home on the 6th hospital day, the polyarthritis (as judged by joint swelling) had resolved in 75% of the involved joints and was ameliorated in the rest, and the fever = 99.6 degrees F. However, the knees were still painful and hobbled the patient, and range of motion was impaired in the knees, shoulders and hips. Biaxin, begun day 6, caused a transient acute memory loss from day 8-9 without fever.

Features supportive of the SLE diagnosis include: leukopenia, positive ANA, a high DS-DNA titer, arthritis (R/O erosions).

During the hospital stay, a more thoughtful insurance medical director rescinded an earlier prohibition for intravenous antibiotics. Had the first “reviewer” acceded to my initial plan of care, the paralysis and the hospitalization it prompted could have been avoided. Treatment is advancing. Repeat DS-DNA, ESR, ANA, CPK, C3, CH50, and X-Ray and MRI evaluation of joints is anticipated. The anemia is being investigated.

The tabulation of rheumatologic syndromes, either caused by LD or affiliated with it, is growing. Drs. Weber and Schwartzberg have reported Polymyalgia Rheumatica apparently caused by LD. As in the case above, Sjogren’s Syndrome with LD has been published. Antibodies to Bb in the context of Psoriatic arthritis, systemic sclerosis and Reiter’s Syndrome have been documented.

An expanding cohort of patients in my practice appear to have long-standing LD that dates to their “growth spurt” years and their past medical history contains the previous development of Osgood-Schlatter’s Syndrome (water on the knee) in their teens. A relationship to LD can’t be excluded.

Arthralgias and bone pain in LD can be excruciating. It is the imprudent clinician or parent who lackadaisically attributes these symptoms to “growing pains” or aging.

Another patient of mine recalled recurrent and migratory polyarthritis since he was 18 (1966). Monthly Medrol Dose Packs (steroids) failed to alter the clinical picture. In 1987, he was hospitalized with an acute exudative synovitis of the knee, whereupon ear lobe biopsy demonstrated the classic histologic feature of Relapsing Polychondritis, a very rare disorder with a peak onset in the 5th decade of life. In spite of continual high dose steroid treatment, (and later methotrexate with non-steroidal anti-inflammatory agents), he eventually developed a deforming arthropathy in his hands which progressed slowly between 6/90 to 12/92. Earlier physicians had discounted the significance of a positive Lyme Elisa = 1.09 in 4/91 as “low titer”. An unsuspected encephalopathy betrayed its existence as memory and concentration impairment beginning 1/91. The patient presented for LD evaluation 1/93. Laboratory values include: an IgG by Elisa = 14.7, IgM by IFA = 80 and the Western Blot had bands at 31 and 41 KDA. the polysynovitis and encephalopathy proved rapidly responsive to antibiotics for LD. The patient was quickly emancipated from the other medications although steroids had to be slowly tapered and are now down to an inconsequential dose without recurrence of rheumatologic complications. The patient, although symptom free while on antibiotics, has permanent crippling deformities of the hands.

Cardiac complications arise in 8-12% of LD cases. Conduction defects and heart block, of which first-degree is the most common, can be discovered on EKG. A long rhythm strip should be employed to detect intermittent blocks. Higher degrees of heart block can result in fainting or death and may require cardiac pacemaker.

Sudden death can also result from arrythmias. Fast and slow heart rates occur, usually at the time of symptom flares, and sometimes in the manner of Sick Sinus Syndrome (tachy/brady). Ventricular tachycardia has been documented to attend LD infection in the heart, confirmed on cardiac biopsy. The cardiomyopathy may be complicated by congestive heart failure (CHF) as the following case might illustrate.