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Symptoms

Ophthalmic manifestations include decreased vision, double vision, pain, photophobia, and facial weakness. Systemic complaints may include headache, malaise, fatigue, fever, chills, palpitations, or muscle/joint pains. A history of a tick bite within the previous few months may be elicited.

Signs

Ocular

Conjunctivitis (most common), episcleritis, exposure keratopathy (due to cranial nerve VII palsy), stromal keratitis, iritis, vitritis, choroiditis, optic neuritis or perineuritis, bilateral optic nerve edema (frequently in children with disseminated disease), cranial nerve palsies, and idiopathic orbital inflammatory syndrome. See specific sections.

Critical Systemic

One or more flat, erythematous, or “bull’s eye” skin lesions, which enlarge in all directions (erythema migrans); unilateral or bilateral facial nerve palsies; polyarticular migratory arthritis. May not be present at the time ocular signs develop.

Other Systemic

Meningitis, peripheral radiculoneuropathy, synovitis, joint effusions, and cardiac abnormalities.

Differential Diagnosis

  • Syphilis: High-positive FTA-ABS titer may produce a low false-positive antibody titer against Borrelia burgdorferi. See 12.12, SYPHILIS.
  • Others: Rickettsial infections, acute rheumatic fever, juvenile idiopathic arthritis, sarcoidosis, tuberculosis, herpes virus infections, etc.

Work Up

Workup
  1. History: Does patient live in endemic area? Prior tick bite, skin rash, facial nerve palsy, joint or muscle pains, flu-like illness? Meningeal symptoms? Prior positive Lyme antibody test?
  2. Complete systemic, neurologic, and ocular examinations.
  3. Two-step diagnosis with a screening assay and confirmatory Western blot for B. burgdorferi.
  4. Serum RPR or VDRL and FTA-ABS or treponemal-specific assay. Consider serum angiotensin-converting enzyme, chest x-ray, and purified protein derivative and/or interferon-gamma release assay (e.g., QuantiFERON-TB Gold).
  5. Consider lumbar puncture when meningitis is suspected or neurologic signs or symptoms are present.
NOTE:

A positive interpretation is generally considered if 5 out of 10 IgG bands are positive or 2 out of 3 IgM bands are positive. IgM is helpful for acute presentation (<4 weeks). IgG antibodies may take 4 to 6 weeks to develop.

Treatment

Early Lyme Disease (Including Lyme-Related Uveitis, Keratitis, or Facial Nerve Palsy)

  1. Doxycycline 100 mg p.o. b.i.d. for 10 to 21 days.
  2. In children, pregnant women, and others who cannot take doxycycline, substitute amoxicillin 500 mg p.o. t.i.d., cefuroxime axetil 500 mg p.o. b.i.d., clarithromycin 500 mg p.o. b.i.d., or azithromycin 500 mg p.o. daily.

Patients With Neuro-Ophthalmic Signs or Recurrent or Resistant Infection

  1. Ceftriaxone 2 g i.v. daily for 2 to 3 weeks.
  2. Alternatively, penicillin G, 20 million units i.v. daily for 2 to 3 weeks.

Follow Up

Every 1 to 3 days until improvement is demonstrated and then weekly until resolved.