Lyme disease is reportable, and approximately 30,000 cases/year are notified to the local and state health departments. However, other estimates based on insurance records suggest there are close to 476,000 cases/year that are diagnosed and treated. In addition to a large burden of illness, areas where Lyme disease is common are expanding. Therefore, clinicians should be aware of uncommon presentations of this condition. We describe the case of a 5-year-old girl who presented with papilledema as an isolated manifestation of Lyme disease. Of note, her ocular symptoms were intermittent and worse when tired. In endemic areas, Lyme disease must be considered in the differential diagnosis for patients presenting with isolated ophthalmic findings even outside the usual Lyme season.
Lyme disease is the most common vector-borne illness in the United States (US) and is endemic in Northeast Ohio [
A previously healthy 5-year-old Caucasian female presented with photophobia, diplopia, eye pain, and internal deviation of the right eye. Symptoms started 10 days prior to her initial evaluation and were reported to be intermittent and worse when tired. She had no associated headaches or facial palsy. She was initially evaluated by an optometrist who referred her to ophthalmology due to the concern for possible optic nerve swelling. On fundoscopic exam, she was found to have bilateral optic disc swelling with elevation, blurring, and large vessels crossing elevated margins consistent with papilledema (Figure
Disc edema at presentation. (a) Left eye fundus exam. (b) Right eye fundus exam.
Reportedly, the patient had a rash on the right side of her face a month prior that was described as nonitchy and nonpainful. In the ED, the patient had normal vitals and nonfocal neurologic exam. Computerized tomographic (CT) scan of the head without contrast was normal. Magnetic resonance imaging (MRI) showed bilateral papilledema, normal venous phase study, and normal appearance of the brain tissue. Cerebrospinal fluid (CSF) opening pressure was 17 cm of H2O (normal range: 10–28). CSF had 29 white blood cells/high-power field (normal range: 0–7) with lymphocytic predominance (77%). Protein and glucose were within normal limits. CSF cultures were sterile. Multiplex PCR panel (Biofire FilmArray®) on the cerebrospinal fluid did not identify any of the amplified targets. Blood tests including metabolic panel, complete blood count, thyroid-stimulating hormone, lactate dehydrogenase, uric acid, ferritin, and C-reactive protein were within normal limits. Erythrocyte sedimentation rate was 26 mm/hr (normal: 0–20). Antinuclear antibody, antineutrophilic cytoplasmic antibody, and antimyelin oligodendrocyte glycoprotein were all negative. Arboviral antibody panel from serum (including West Nile, Eastern, and Western equine encephalitis and LaCrosse all of which are reported in Ohio) was negative.
Isolated papilledema is a rare manifestation of Lyme disease that typically presents as diplopia [
Our patient was a previously healthy 5-year-old female who presented with diplopia, photophobia, internal deviation of the right eye, and eye pain. She was found to have bilateral optic disc swelling. Extensive workup showed positive Lyme serology with immunoblot confirmation. She did not recall any tick exposure. She did have a nonpruritic, nontender rash on the right side of her face one month prior, but as is often the case, this may have been confused for other nonspecific rashes.
PubMed review (1998–present) of ocular manifestations in Lyme disease in children revealed the following (please see Table
Summary of the literature review of ocular manifestations of Lyme disease.
Citation | Patient details | Initial symptoms | Time from initial symptoms to ocular symptoms (weeks) | Ocular symptoms | Ocular signs | Antibody to | Antibody to | Opening pressure (cm H2O) | CSF white cells | Brain MRI findings | Treatment | Outcome |
---|---|---|---|---|---|---|---|---|---|---|---|---|
Bands on WB | CSF/serum ratio | |||||||||||
Rothermel et al. [ | An 8-year-old male | Headache | 8 | Decreased vision | Swollen discs with elevation of the retina | 10/10 IgG bands | Not done | Normal | 0 | Normal | Ceftriaxone | Resolution of symptoms |
A 16-year-old male | Knee arthritis | 28 | Blurred vision of the left eye | Swollen optic disc, central scotoma | 8/10 IgG bands | Not done | Normal | <10 | Normal | Ceftriaxone | Resolution of symptoms | |
A 13-year-old girl | Fever, headache, and neck pain | 3 | Horizontal diplopia and eye pain | Bilateral papilledema, sixth nerve palsy, and decreased visual acuity | 7/10 IgG bands | IgG 1.42 | 60 | 82 | Normal | Ceftriaxone | Resolution of symptoms | |
An 11-year-old male | Rash, headaches, vomiting, and fever | 2.5 | Double vision | Photophobia and bilateral sixth and seventh nerve palsies | 8/10 IgG bands | <1 for IgG, IgM, and IgA | 57 | 3 | — | Ceftriaxone and methylprednisolone | Improvement in symptoms with residual sixth nerve palsy | |
Ezequiel et al. [ | A 9-year-old male | Headache, pallor, photophobia, and phonophobia | — | — | Papilledema | Positive | Positive IgG and IgM | 50 | 30 | Normal | Ceftriaxone | Resolution of symptoms |
Kan et al. [ | An 8-year-old female | Headaches, vomiting, and diplopia | — | Diplopia | Papilledema and left sixth nerve palsy | Positive | Positive CSF antibodies | 32 | 115 | Dural enhancement | Ceftriaxone and acetazolamide | Papilledema resolved, and mild sixth nerve palsy remained |
Rothermel et al. [
Ezequiel et al. [
Kan et al. [
Similar to previous reports, our patient also had the symptoms of diplopia, photophobia, and eye deviation, but the unusual feature of this case is isolated ocular symptoms. All of the patients described above had additional symptoms including headache or low-grade fever that were helpful in guiding workup and diagnosis. Given our patient’s young age, there is the possibility that she had difficulty reporting headaches. However, one would expect that since she was able to articulate her visual symptoms, she would be able report headaches as well.
Our patient was treated with a 21-day course of doxycycline therapy. This choice was based on the recommendation that “A growing body of evidence suggests that oral doxycycline is effective for the treatment of Lyme meningitis and may be used as an alternative to hospitalization and parenteral ceftriaxone therapy in children who are well enough to be treated as outpatients.” [
Improved disc edema on follow-up. (a) Left eye fundus exam. (b) Right eye fundus exam.
Lyme disease must be considered in the differential diagnosis of papilledema for patients living in endemic areas (not only during the Lyme season). The significance is even greater when one recognizes that all the reported patients improved with antimicrobial treatment.
No additional data were available to support this manuscript.
The authors declare that they have no conflicts of interest.