• Users Online: 281
  • Print this page
  • Email this page


 
 
Table of Contents
CASE REPORT
Year : 2022  |  Volume : 4  |  Issue : 1  |  Page : 38

Neuroretinitis as the only manifestation of Lyme disease: A case report


1 Department of Ophthalmology, Penido Burnier Institute, Campinas, São Paulo, Brazil
2 MUHC-McGill University Ocular Pathology and Translational Research Laboratory, Montreal, Quebec, Canada
3 MUHC-McGill University Ocular Pathology and Translational Research Laboratory; Department of Ophthalmology and Pathology, McGill University, Montreal, Quebec, Canada

Date of Submission10-Jun-2022
Date of Decision21-Jun-2022
Date of Acceptance27-Jun-2022
Date of Web Publication30-Jul-2022

Correspondence Address:
Mateus Pimenta Arruda
Durvalina Tavares Barreto Street, 30, Apartment 54, Sao Paulo
Brazil
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/pajo.pajo_31_22

Rights and Permissions
  Abstract 


Lyme disease is a multisystemic infection caused by the spirochete bacterium Borrelia burgdorferi, transmitted by the tick of the genus Ixodes sp. and has three stages: primary, secondary, and tertiary. Ocular involvement is possible in any of these stages. Neuroretinitis is a rare manifestation. It is recommended that the diagnosis be performed by serology using an immunoenzymatic test, and confirmed by the Western Blot test. The recommended therapy is doxycycline 100 mg 12/12 h or 200 mg once a day for 14 days to 21 days or amoxicillin 500 mg three times a day for 14–21 days. We herein present a patient who had neuroretinitis related to a previous diagnosis of Lyme disease. The presence of neuroretinitis in a setting of Lyme disease represents a very uncommon manifestation of this particular infection.

Keywords: Borrelia burgdorferi, fundus oculi, Ixodes, Lyme disease, retinitis


How to cite this article:
Arruda MP, Nogueira Costa MA, Coblentz J, Burnier MN. Neuroretinitis as the only manifestation of Lyme disease: A case report. Pan Am J Ophthalmol 2022;4:38

How to cite this URL:
Arruda MP, Nogueira Costa MA, Coblentz J, Burnier MN. Neuroretinitis as the only manifestation of Lyme disease: A case report. Pan Am J Ophthalmol [serial online] 2022 [cited 2022 Aug 20];4:38. Available from: https://www.thepajo.org/text.asp?2022/4/1/38/353006




  Introduction Top


Neuroretinitis is defined as a focal inflammation of the optic nerve and the peripapillary retina or macula, whether the cause is infectious or idiopathic.[1],[2] It can be one of the manifestations of Lyme Disease, also known as Lyme borreliosis and Chronic Erythema Migrans, a multisystemic infection caused by the spirochete bacterium Borrelia burgdorferi, transmitted by the tick of the genus Ixodes sp.[1],[3],[4] It has three stages, and ocular involvement can occur in any of them; however, neuroretinitis has been described as a result of the secondary stage.[3],[5] To make the diagnosis of Lyme disease with extracutaneous manifestations, it is necessary to confirm the diagnosis a positive serological test, such as, a positive enzyme-linked immunosorbent test, which can be confirmed by the Western Blot test.[1]


  Case Report Top


A 23-year-old woman presented on May 13, 2020 complaining of low visual acuity in the left eye (LE) for 30 days. She had no history of trauma or medication use.

Ophthalmologic examination showed a Snellen best-corrected visual acuity (BCVA) of 20/20 in the right eye (RE) and 20/400 in the LE. A slip-lamp examination revealed clear conjunctiva, transparent cornea, phakic, and the absence of anterior chamber reaction in both eyes (OU); intraocular pressure 14 mmHg (OU). Fundus examination revealed physiologic cupping, and preserved macular brightness (RE). 4+/4+ papilledema, increased vascular tortuosity, presence of exudates and edema in the macular region, applied retina (LE) [Figure 1].
Figure 1: Fundus picture of left eye showing significant papilledema, increased vascular tortuosity, exudates, and macular edema

Click here to view


Optical coherence tomography (OCT), angiography, and retinography were performed. Requested erythrocyte sedimentation rate, complete blood count, serology for toxoplasmosis (immunoglobulin G [IgG], immunoglobulin M [IgM]), tuberculin skin test, venereal disease research laboratory, fluorescent treponemal antibody absorption, herpes simplex virus test (IgG and IgM), rheumatoid factor, antinuclear antibody, serology for Bartonella, chest radiography and angiotensin-converting enzyme. Since serologies results were pending, empirical treatment with doxycycline 100 mg 12/12 h was started, considering a probable neuroretinitis due to cat scratch disease.

The serology for Bartonella was negative; however, the IgM serology was 1:116 positive for Borrelia and was confirmed by the Western blot test. The diagnostic hypothesis was neuroretinitis caused by Lyme disease. It was decided to supplement with prednisolone 40 mg/day, as an anti-inflammatory, because of the disc edema, and the patient was followed up being observed clinically and on imaging tests. OCT and retinography were requested as follow-up.

LE OCT (before treatment): vitreoretinal interface unchanged, with loss of foveal depression. There was evidence of “wrinkling” of the inner limiting membrane, with an increase in the diffuse retinal thickness. There is the presence of intraretinal and subretinal fluid, generating a sensory retinal detachment [Figure 2].
Figure 2: Optical coherence tomography of left eye showing loss of foveal depression, “wrinkling” of the inner limiting membrane, with an increase in the diffuse retinal thickness. There is the presence of intraretinal and subretinal fluid, leading to sensory retinal detachment

Click here to view


LE angiography (late venous phase): Hyperfluorescence with blurring due to extravasation around the optic disc. Progressive hyperfluorescence is also evidenced, by staining in the arcades. The macular region is hypofluorescent, probably due to blockage. In the temporal periphery, the presence of hypofluorescence due to nonperfusion is noted [Figure 3].
Figure 3: Angiography (late venous phase) of the left eye showing hyperfluorescence with blurring of the optic disc. Progressive hyperfluorescence is also evidenced by staining in the arcades. The macular region, as well the temporal periphery are hypofluorescent

Click here to view


After 60 days, there was an improvement in the disc edema, exudation, and macular edema, with an important improvement in visual acuity [Figure 4].
Figure 4: Fundus picture of left eye after treatment showing improvement in the papilledema, exudation, and macular edema

Click here to view


LE OCT (after treatment): vitreoretinal interface without changes, with vitreous adhesion in the macular region. Thinning of the inner layers of the retina. The absence of subretinal fluid. Hyperreflective spots between inner and outer plexiform nuclear layers, temporally to the macula, and choroidal thickness preserved [Figure 5].
Figure 5: Optical coherence tomography of left eye after treatment showing thinning of the inner layers of the retina, absence of subretinal fluid, and hyperreflective spots between inner and outer plexiform nuclear layers, temporally to the macula

Click here to view



  Discussion Top


The infectious agent of Lyme disease is B. burgdorferi, a spirochete bacterium detected in blood, synovial fluid, cerebrospinal fluid, retina, and vitreous humor.[3],[4]

The incubation period can vary between 4 and 18 days and the immunity from the disease is not maintained; therefore, reinfection is possible.[1] The most common and initial manifestation is the skin lesion called erythema migrans, which is present in 70%–80% of patients and may be related to constitutional symptoms such as fever, myalgia, and headache.[1],[4] It usually occurs within 1–2 weeks after the tick bite, whereas the secondary stage (widespread infection) occurs after weeks to months of the primary stage, with predominantly neurological, cardiovascular and rheumatological involvement, and less frequently, ocular.[1],[4],[6] The absence of skin lesions, and also neuroretinitis as the only presentation of this infection, is indeed very uncommon; although, it has been reported in some cases.[1],[3],[7],[8] The similarity of these cases to our, was that the patient's outcome was sudden and painless blurring vision, without cutaneous manifestation and three of them had a serology confirmation by the enzyme-linked immunosorbent assay (ELISA) and Western blot.

Neuroretinitis is an inflammatory disorder characterized by optic disc edema and subsequent macular star imaging.[1],[2] It is one of its possible manifestations, commonly in the secondary stage,[1],[2] but neuroretinitis can also occur in cat-scratch disease, syphilis, mumps, toxoplasmosis, salmonellosis, tuberculosis, and histoplasmosis.

It is recommended that the diagnosis be performed by serology using an immunoenzymatic test, and confirmed by the Western Blot test.[4] It can be helpful in identifying false-positive reactions which may occur in an ELISA.[9],[10] However, some disadvantages including comigration of multiple proteins to the same area and inconsistent test results between laboratories due to differences in the reagents must be considered.[9] Once the diagnosis is confirmed, the indicated therapy is doxycycline 100 mg 12/12 h or 200 mg once a day for 14–21 days, or amoxicillin 500 mg three times a day for 14–21 days.[4] Allergic patients may use erythromycin or tetracycline. For late manifestations, ceftriaxone 2 g can be used once a day for 14–28 days or crystalline penicillin.[1]

In cases in which there was association with neuroretinitis, the patient commonly presents with decreased visual acuity and often without ocular pain, as presented by Guliani BP et al. In our patient, oral doxycycline and corticosteroids were prescribed, with significant improvement in vision after 60 days (BCVA: 20/30) and also in OCT (which is possible to monitor the evolution of macular edema and optic nerve).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Vanya M, Fejes I, Jako M, Tula A, Terhes G, Janaky M, et al. Lyme disease associated neuroretinitis – Case report. Acta Microbiol Immunol Hung 2015;62:403-8.  Back to cited text no. 1
    
2.
Purvin V, Sundaram S, Kawasaki A. Neuroretinitis: Review of the literature and new observations. J Neuroophthalmol 2011;31:58-68.  Back to cited text no. 2
    
3.
Guliani BP, Kumar S, Chawla N, Mehta A. Neuroretinitis as presenting and the only presentation of Lyme disease: Diagnosis and management. Indian J Ophthalmol 2017;65:250-2.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Sanchez E, Vannier E, Wormser GP, Hu LT. Diagnosis, treatment, and prevention of Lyme disease, human granulocytic anaplasmosis, and babesiosis: A review. JAMA 2016;315:1767-77.  Back to cited text no. 4
    
5.
Chawla R, Pundlik GA, Chaudhry R, Thakur C. Rickettsial retinitis: Direct bacterial infection or an immune-mediated response? Indian J Ophthalmol 2017;65:1038-41.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
John M, Raman M, Ryan K. A tiny tick can cause a big health problem. Indian J Ophthalmol 2017;65:1228-32.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Lochhead J, Thompson GM. Bilateral papilloedema with concomitant neuroretinitis in a 7-year-old girl with Lyme disease. Eye (Lond) 2001;15:799-801.  Back to cited text no. 7
    
8.
Babu K, Murthy PR. Neuroretinitis as a manifestation of Lyme disease in South India: A case report. Ocul Immunol Inflamm 2010;18:97-8.  Back to cited text no. 8
    
9.
Magnarelli LA. Current status of laboratory diagnosis for Lyme disease. Am J Med 1995;98:10S-2S.  Back to cited text no. 9
    
10.
Talagrand-Reboul E, Raffetin A, Zachary P, Jaulhac B, Eldin C. Immunoserological diagnosis of human borrelioses: Current knowledge and perspectives. Front Cell Infect Microbiol 2020;10:241.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed335    
    Printed10    
    Emailed0    
    PDF Downloaded38    
    Comments [Add]    

Recommend this journal