Lyme disease ED Presentations / Management Pearls and Pitfalls

Authors: Oleg Uryasev, MD (EM Resident Physician, Virginia Tech Carilion Emergency Medicine Residency) and Jack Perkins, MD (Assistant Program Director, Virginia Tech Carilion Emergency Medicine Residency) // Edited by: Jennifer Robertson, MD, MSEd and Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW Medical Center / Parkland Memorial Hospital)

 Background

The first case of Lyme disease was documented more than 5000 years ago. (1) Today, it is the most commonly reported vector borne disease with ~30,000 cases per year in the United States. The vast majority of cases are found in the Northeast and in the upper Midwest, but there is distribution nationally. (2,3) Lyme is a manifestation of infection by the spirochete Borrelia burgdorferi. This spirochete is harbored by the deer tick Ixodes scapularis and Ixodes pacificus.

The chance of Lyme transmission depends on the duration of tick attachment. If an infected tick is attached for less than 24 hours, then there is an almost zero percent chance of transmission. However, the tick has been attached for more than 72 hours, there is an almost one hundred percent chance of disease transmission. (4) It is important to keep in mind that patients with tick bites are at risk for many other tick borne illnesses such as Babesiosis, Rocky Mountain Spotted Fever, Southern Tick-associated Rash Illness, Ehrlichiosis, Anaplasmosis and Tularemia.

Case Presentations and Discussion:

Case 1:

13 year-old male presents after a camping trip with reports of tick bite and subsequent rash. He is otherwise healthy and has no medical or surgical history. Vital signs are unremarkable. His examination is normal except for a target-like lesion with central clearing on his abdomen which is pruritic and mildly painful.

What are some manifestations of early Lyme Disease?

Presentation of Lyme depends on the degree of systemic dissemination. Early on, when locally contained, the manifestation tends to be dermatologic. Erythema migrans (EM), an isolated, circumscribed, target like lesion with central clearing, is present in 70-80% of patients and usually erupts on the lower extremities or the trunk within 3-30 days of tick bite.  Atypical presentations can have multiple EM lesions, associated vesicles or necrosis, no central clearing, or be located in an unusual location such as the scalp. Some individuals with Lyme disease will never manifest a rash. Borrelial lymphocytoma, a reddish-blue nodule located on the nipple or ear is an extremely rare, and predominantly European dermatologic presentation for Lyme. (5, 6, 7) Pediatric presentations for all stages tends to mimic adult presentations. (8)

As the disease progresses, systemic symptoms occur and may mimic a viral illness that lacks respiratory or gastrointestinal complaints. Fatigue, myalgias, arthralgias, fevers, anorexia, headache and neck stiffness are the hallmarks of this stage. Conjunctivitis can occur as well. (9, 10)

Which antibiotics should I choose when treating Lyme disease?

In early infections or indolent infections with arthritis as a preliminary manifestation, oral doxycycline is sufficient. In cases where doxycycline is contraindicated, such as in children under <8 years old and pregnant patients, amoxicillin and cefuroxime axetil are acceptable alternatives. For advanced cases requiring inpatient management, (e.g. neurological or cardiac involvement) intravenous (IV) ceftriaxone is recommended. Duration of therapy depends on degree of disease progression but it usually is for 14-28 days. (4)

Case 2:

A 40 year-old female presents for facial droop. The droop started in the right side of her face several days ago and is progressively worsening. She reports having a viral illness the month prior with myalgias, fevers and a headache. Her examination is remarkable for right facial nerve palsy, otherwise unremarkable.

When should I suspect Lyme as the causative agent for Bell’s Palsy?

Bell’s Palsy is the most common cranial neuropathy associated with Lyme. Lyme can cause neuropathy in any cranial nerve, but the facial nerve is the most commonly affected. Cranial neuropathies can happen weeks to months after initial infection. In order to have neurological symptoms, the spirochetes must be disseminated in the nervous system. Lyme should be suspected as the causative agent when a cranial neuropathy occurs in the setting of symptoms consistent with disseminated Lyme such as myalgias/arthralgias, fevers, headache or meningismus.  (11)

Should I be testing/treating all patients with Bell’s Palsy for Lyme?

When caused by Lyme, it is rare to have an isolated cranial nerve palsy without any other symptoms of disseminated disease. For this reason, routine testing and treatment with an isolated Bell’s palsy with no other symptoms consistent with Lyme is low yield and should not be done. (12) In cases where Lyme is the suspected culprit of a cranial nerve deficit, the patient should be treated empirically without waiting for titers to be completed.

 Case 3:

21 year-old female presents to the ED for altered mental status. Her family reports she has not been feeling well for the last several months and now is not acting like herself. She is febrile and tachycardic. On examination she is ill-appearing, answers questions inappropriately, has meningismus and her skin is hot and dry.

When should I suspect Lyme as the cause of meningitis? What should the CSF analysis show?

Clinically, Lyme progressing to meningitis should demonstrate the regular prodrome of early disseminated disease. Patients will have fevers, myalgias, arthralgias, and rash prior to central nervous system (CNS) involvement of Lyme. On occasion, a history may be difficult to obtain, or your patient may have had an atypical presentation of early Lyme. Thus, the making the diagnosis of CNS Lyme meningitis may be very difficult. A lumbar puncture should be performed to confirm presence of meningitis.  Once patients develop meningitis, the presence of a cranial nerve palsy in conjunction with meningitis makes the diagnosis of Lyme slightly more likely. (13) Cerebral spinal fluid (CSF) findings will be almost indistinguishable from viral meningitis. (14)

If I suspect Lyme meningitis, how should I tailor ED management and antibiotic choices?

Management for Lyme meningitis in the ED is identical to management for any other suspected bacterial meningitis. Antibiotic therapy should not be delayed in order to obtain a lumbar puncture since a delay in antibiotic therapy leads to worse patient outcomes. Antibiotics should be ordered immediately while the LP is being performed. If Lyme is the causative agent for meningitis, the patient will already be covered appropriately if IDSA guidelines are followed for antibiotic choices. Every patient greater than one month old should receive either ceftriaxone or cefepime, either of which will adequately treat Borrelia burgdorferi. (15)

Case 4:

32 -year-old male presents to the ED for shortness of breath and chest pain. He reports a recent illness after which he started to have chest pain and shortness of breath. He is bradycardic on examination and his EKG demonstrates a 2nd degree AV block.

How do I approach cardiovascular manifestations of Lyme?

Untreated, the disseminated late stage disease of Lyme can progress to cardiac manifestations, but this is rare. Cardiac pathology due to Lyme usually involves varying degrees of atrioventricular (AV) block. The AV block fluctuates and can rapidly progress from first degree to third degree. Approximately 1/3rd of patients with Lyme carditis will require temporary AV pacing. Almost no patients require permanent pacemakers since the heart block resolves with antibiotic treatment. Some patients develop ST segment depression or T wave inversions, most commonly in inferior leads. Lyme can present with a myocarditis or pericarditis pattern as well.

While Lyme carditis can occur in any demographic group, young males are at highest risk. (4, 16, 17) Lyme carditis is a difficult diagnosis to make in the ED due to its infrequent occurrence. Lyme carditis should be considered in patients with heart block who are not typically at risk for heart blocks in endemic regions of the country or with systemic symptoms consistent with disseminated Lyme.

Which patients should I admit for parenteral therapy?

The IDSA recommends admission and parenteral antibiotics for (4):

Symptomatic patients (e.g. syncope, dyspnea, or chest pain)

Second or third degree AV block

First degree AV block with PR interval greater than 300 milliseconds

Case 5:

17 year-old male presents with an engorged tick on his right side. He has no medical or surgical history, and currently has no symptoms. His vital signs are unremarkable. On examination, he has no rashes, but a small unidentifiable tick is seen on his right flank.

When is prophylaxis indicated?

The answer is based on the individual, history of the tick bite, and practice location. The IDSA criteria by which a provider should determine whether prophylaxis is indicated:

1) Tick identified as I. Scapularis and is present ≥ 36 hours (engorgement or by patient history)

2) Local rate of infection ≥ 20%

3) Treatment begun < 72 hours from tick removal

4) No contraindication to Doxycycline

For an updated distribution of endemic areas with Lyme visit the CDC website: http://www.cdc.gov/lyme/stats/maps.html

Prophylaxis for Lyme is a single dose of 200mg of doxycycline after a tick with high risk of infection. Amoxicillin has never been verified to be effective in prophylaxis and should not be used. (4)

When should we pursue diagnostic testing in the ED?

Sensitivity of diagnostic testing depends on degree of disease dissemination. ELISA and Western blot testing are available for IgM and IgG antibodies to Borrelia burgdorferi. Testing in the early stages of Lyme (e.g. tick exposure with or without EM rash) will likely be falsely negative due to lack of systemic reaction. (18) In the ED, testing for Lyme will not change our management since tests require several days for results. Patients should be treated empirically and dispositioned appropriately based on severity of symptoms. Testing is generally discouraged due to the delay in obtaining results and the potential for false results.

Case 6:

A 66 yo female with a history of hypertension, fibromyalgia, and anxiety presents with five years of fatigue, arthralgias and generalized weakness. She has had intermittent chills as well. She states she was tested a year ago for Lyme’s disease and this was positive. She has subsequently had three separate courses of antibiotics for her Lyme’s disease and is requesting admission for IV antibiotics for Chronic Lyme Disease resistant to oral antibiotics. Her vitals signs and a complete examination are unremarkable. EKG, laboratory tests and a recent magnetic resonance imaging (MRI) of the brain are reviewed and also unremarkable except for a positive PCR for Lyme 12 months prior.

How do we approach the patient with “Chronic Lyme Disease”?

A lot of confusion surrounds the diagnosis of “Chronic Lyme Disease.” When we say Chronic Lyme Disease (CLD) we do not mean untreated Lyme with progression to late disseminated disease, or undiagnosed Lyme with chronic arthritis or neurological manifestations. Rather, CLD is one of two broad categories:

  • Physical manifestations of the disease after successful treatment. More correctly this diagnosis should be Post-Lyme syndrome (PLS). PLS defined by fatigue, weakness, arthralgias and other symptoms of Lyme for a prolonged period after successful treatment with antibiotics. In fact, many patients require 12-24 months for all symptoms to clear. This period may even longer for those with disseminated disease.
  • Vague complaints in a patient with no true diagnosis of Lyme. The patient may have positive PCR testing but is likely false positive or positive due to a previous Lyme infection. Alternatively, he or she may have had negative PCR results but he or she may still believe that Lyme is responsible. Both of these patient groups have an alternative diagnosis causing their symptoms.

Are antibiotics indicated for Chronic Lyme Disease?

Chronic Lyme Disease is not a prolonged infection despite treatment. Antibiotics are not indicated in the Emergency Department. (19)

Conclusion

Lyme disease is the most common among a handful of uncommon tick borne illnesses and should be included in the differential of diagnoses, especially in patients who live in endemic areas. It is helpful for emergency providers to be familiar with all manifestations of Lyme disease especially since antibiotics may be curative.

 Take home points:

1) Add Lyme disease to your differential when you see a patient with a history viral illness with headache, myalgias/arthalgias and fevers, especially in endemic regions.

2) If Bell’s palsy is due to Lyme, systemic symptoms of Lyme should also be present. Testing for Lyme in isolated Bell’s palsy is not indicated.

3) Initial management for Lyme meningitis is identical to any other patient presenting to the ED for bacterial meningitis.

4) Prophylaxis is only appropriate in patients who reside in an endemic area who have had a tick attached for greater than 36 hours. Doxycycline is the only approved prophylactic antibiotic.

5) Chronic Lyme disease is not a continued spirochete infection and is often misdiagnosed when in fact a completely separate disease process (e.g. multiple sclerosis) is the underlying etiology.  Chronic Lyme disease refers to prolonged symptoms after the infectious component has been treated.

 References / Further Reading

1) http://www.livescience.com/18704-oldest-case-lyme-disease-spotted-iceman-mummy.html Last accessed 7/23/2016

2) http://www.cdc.gov/lyme/stats/ Last accessed 7/23/2016

3) Kugeler KJ, Farley GM, Forrester JD, Mead PS. Geographic distribution and expansion of human Lyme disease, United States. Emerg Infect Dis. 2015 Aug;21(8):1455-7

4) Gary P.  Wormser, Raymond J.  Dattwyler, Eugene D.  Shapiro, John J.  Halperin, Allen C.  Steere, Mark S.  Klempner, Peter J.  Krause, Johan S.  Bakken, Franc Strle, Gerold Stanek, Linda Bockenstedt, Durland Fish, J.  Stephen Dumler, and Robert B.  Nadelman. The Clinical Assessment, Treatment, and Prevention of Lyme Disease, Human Granulocytic Anaplasmosis, and Babesiosis: Clinical Practice Guidelines by the Infectious Diseases Society of America. IDSA Guidelines 2006

5) Müllegger RR, Glatz M. Skin manifestations of lyme borreliosis: diagnosis and management. Am J Clin Dermatol 2008; 9: 355-68.

6) Biesiada G, Czepiel J, Leśniak MR, Garlicki A, Mach T. Lyme disease: review. Archives of Medical Science : AMS. 2012;8(6):978-982.

7) Steere AC, Sikand VK. The presenting manifestations of Lyme disease and the outcomes of treatment. N Engl J Med 2003; 348:2472.

8) Gerber MA, Shapiro ED, Burke GS, et al. Lyme disease in children in southeastern Connecticut. Pediatric Lyme Disease Study Group. N Engl J Med 1996; 335:1270.

9) Bitar I, Lally EV. Musculoskeletal manifestations of Lyme disease. Med Health R I 2008; 91: 213-5.

10) Nadelman RB, Nowakowski J, Forseter G, et al. The clinical spectrum of early Lyme borreliosis in patients with culture-confirmed erythema migrans. Am J Med 1996; 100:502.

11) Kuiper H, Devriese PP, de Jongh BM, Vos K, Dankert J. Absence of Lyme borreliosis among patients with presumed Bell’s palsy. Arch Neurol. 1992;49(9):940–943.

12) Albers JR, Tamang S. Common questions about Bell palsy. Am Fam Physician. 2014 Feb 1;89(3):209-12.

13) Garro AC, Rutman M, Simonsen K, et al. Prospective validation of a clinical prediction model for Lyme meningitis in children. Pediatrics 2009; 123:e829.

14) Lakos A. CSF findings in Lyme meningitis. J Infect 1992; 25:155.

15) Tunkel AR, Hartman BJ, Kaplan SL, Kaufman BA, Roos KL, Scheld WM, Whitley RJ, Practice Guidelines for the Management of  Bacterial Meningitis by the Infectious Diseases Society of America. IDSA Guidelines 2004

16) Forrester JD, Mead P. Third-degree heart block associated with lyme carditis: review of published cases. Clin Infect Dis. 2014 Oct;59(7):996-1000.

17) Scheffold N, Herkommer B, Kandolf R, May AE. Lyme carditis–diagnosis, treatment and prognosis. Dtsch Arztebl Int. 2015 Mar 20;112(12):202-8.

18) Steere AC, McHugh G, Damle N, Sikand VK. Prospective study of serologic tests for lyme disease. Clin Infect Dis 2008; 47:188.

19) Marques A. Chronic Lyme Disease: An appraisal. Infectious disease clinics of North America. 2008;22(2):341-360.

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