Lyme borreliosis is caused by infection with Borrelia burgdorferi, a spirochete transmitted by deer ticks. The disease, which typically occurs in 3 stages, can produce ocular and neuro-ophthalmic manifestations. Lyme disease is discussed further in BCSC Section 1, Update on General Medicine, and Section 9, Uveitis and Ocular Inflammation.
Along with various uveitic entities, neuro-ophthalmic findings occur primarily in stage 2 and include papilledema (IIH-like syndrome), optic neuritis, papillitis, neuroretinitis, and orbital myositis. Two-thirds of patients have ocular findings at this stage. Cranial neuropathies can occur, most commonly CN VII palsy, as well as radiculopathies and meningitis with headache and neck stiffness.
In stage 3, neurologic conditions (neuroborreliosis) predominate, including chronic encephalomyelitis, spastic paraparesis, ataxic gait, subtle mental disorders, and chronic radiculopathy. The neurologic picture may resemble that of MS, clinically and radiographically. In rare instances, Lyme can cause repeat cerebrovascular events in multiple locations within short time intervals.
The diagnosis is made clinically when the patient has been exposed to an endemic area (the patient might not recall a tick bite) and shows the typical rash of erythema chronicum migrans (Fig 14-22). The presence of an elevated Lyme antibody titer in the serum or CSF is helpful. The enzyme-linked immunosorbent assay (ELISA) is typically used for screening, but a positive result using the Western blot technique confirms the diagnosis.
Figure 14-22 Erythema chronicum migrans, the characteristic skin rash of stage 1 Lyme disease.
(Courtesy of Robert L. Lesser, MD.)
Treatment should be orchestrated by a specialist in infectious diseases.
Träisk F, Lindquist L. Optic nerve involvement in Lyme disease. Curr Opin Ophthalmol. 2012;23(6):485–490.
Excerpted from BCSC 2020-2021 series: Section 5 - Neuro-Ophthalmology. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.