Lyme disease is a multisystem disorder caused by infection with the Bo
rrelia burgdorferi spirochete. The diagnosis of Lyme disease usually i
s based on several clinical criteria, with supportive data from labora
tory testing. The presence of the bullseye skin lesion, erythema migra
ns, is the single pathognomonic criterion. In the 20 years since the i
nitial description of Lyme disease in the United States, B. burgdorfer
i has been implicated as an etiologic agent in numerous ophthalmic and
neuro-ophthalmic syndromes, involving most structures from the cornea
to the cranial nerves. Neuro-ophthalmic and ocular manifestations of
Lyme disease include meningitis with papilledema, cranial neuropathies
, follicular conjunctivitis, nummular keratitis, and intraocular infla
mmation. Although an association with Lyme disease has been purported
for numerous other syndromes, a definite causal relationship has not b
een proved in many cases. During a period of rapidly increasing awaren
ess of Lyme disease, a high index of suspicion and poorly defined crit
eria for its presence have resulted in overdiagnosis of Lyme disease.
In the authors' experience, the incorrect diagnosis of Lyme disease in
itially has been made in patients with allergic conjunctivitis, kerato
conus, morning glory syndrome, craniopharyngioma, meningioma, CNS lymp
homa, paraneoplastic syndrome, multiple sclerosis, sarcoid, syphilis,
and functional illness. Nevertheless, this treatable infection must be
an important consideration in the differential diagnosis of certain o
cular or neurologic diseases.