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Neuroretinitis – The Great Masquerader

Home  / Intraocular Inflammation and Uveitis / Noninfections (Autoimmune) Uveitis

Authors : Natalie K. Modersitzki, Alexandra S. Vitale, BS, Lydia Sauer, MD, Paul S. Bernstein, MD, PhD, Marissa B. Larochelle, MD, Department of Ophthalmology, John A. Moran Eye Center, University of Utah, Salt Lake City, UT 84112.

Date: 06/19/19

Keywords/Main Subjects: Uveitis, Neuroretinitis, Optic Nerve,

Diagnosis: Idiopathic Neuroretinitis

An 18-year-old female college student presented to the student health center with temporal vision loss in the right eye, headaches in the right posterior region of her head, and severe photophobia. The patient described her vision loss as an opaque smudge in her temporal periphery. She noted these symptoms as well as intermittent nausea, vomiting and diarrhea two to three weeks before her initial visit. She also experienced a sore throat and flu-like symptoms. The patient was subsequently referred to an ophthalmologist.

At presentation, the patient had a visual acuity of 20/50 OD and 20/20 OS with trace afferent pupillary defect in her right eye. Her slit lamp examination was normal in the left eye, but showed significant 360º optic nerve edema accompanied by a peripapillary hemorrhage in the right eye. An MRI of the brain and the orbits was performed and revealed no abnormalities. On the following day, the patient vomited and lost consciousness, and was evaluated in the Emergency Department. A lumbar puncture was performed, which showed an opening pressure of 22 cm of H20 accompanied by normal protein and white blood cell count. The patient was initially prescribed acetazolamide, but due to a lack of clinical improvement she was taken off this medication after several doses. In addition, she received one dose of IV steroids. The patient was then referred to the Moran Eye Center and seen by a neuro-ophthalmologist for possible papillitis. After neurological examination was unfruitful, she was seen by a uveitis specialist for possible neuroretinitis, a form of posterior uveitis.[1]Examination of the right eye revealed trace vitreous cells, 360º of optic nerve edema, mild peripapillary hemorrhages, and minor perivascular changes around the optic nerve.

Treatment of neuroretinitis depends on whether there are underlying infectious or inflammatory conditions that require therapy, therefore it is important to identify the etiology.[2] Infectious causes of neuroretinitis include Bartonella henselae, hepatitis B, herpes simplex, herpes zoster, histoplasmosis, Lyme disease, leptospirosis, mumps, salmonella, toxocariasis, toxoplasmosis, tuberculosis and typhus.[3] It is recognized that Bartonella henselae accounts for two-thirds of neuroretinitis cases and is associated with cat scratch fever. The patient reported frequent exposure to cats, as well as an extensive travel history in the months preceding her ocular disturbances. She reported hiking along the East Coast as well as in New Mexico and the Mississippi River Valley. Based on this history, there was a high concern for an infectious etiology. However, despite broad lab testing for both infectious and inflammatory causes, all results came back within normal limits. Therefore, the patient was diagnosed with idiopathic neuroretinitis.

The presenting visual acuity of neuroretinitis patients can range from 20/50 to 20/200 and is usually associated with an afferent pupillary defect as seen in this patient’s case.[3] The most common visual field defect recorded are cecocentral scotomas.[3] Neuroretinitis is a type of optic neuropathy with optic disk edema, serous retinal detachment, and  peripapillary or macular hard exudates arranged in a star figure around the fovea.[2, 4, 5] It is recognized that the optic disk swelling precedes the macular exudates by 9-12 days.[2, 6]Due to this lag period, stellate maculopathy may not always be evident at the time of initial presentation.[3] The lipid exudates that make up the macular star, seen in the outer-nuclear plexiform space, are derived from leaking optic disk capillaries.[7]

Neuroretinitis illustrates the deep-seated relationship between the central nervous system (CNS) and the retina. The retina and the CNS tissue share embryological origin and are reminiscent of each other. Therefore, retinal pathology in some diseases may be an early indication of a more sinister CNS pathology. However, in neuroretinitis the ontogeny of a macular star suggests against the development of a demyelinating disease.[7] Many patients experience viral prodromes prior to the onset of visual disturbances, such as lymphadenopathy, sore throat, and fatigue.[6, 7] Neuroretinitis is often associated with autoimmune diseases, infections, and is typically unilateral, characterized by a single episode. However, recurrent episodes as well as bilateral involvement have also been observed.[8] The mean age of onset is 28 years, ranging from age 8-55 with no gender predilection.[3, 6]

The disease is typically self-limiting, but immune modulatory therapy is indicated in some cases. Patients with idiopathic neuroretinitis usually do not need any treatment due to the propensity for the disease to spontaneously resolve. Nevertheless, antibiotics, steroids, or both have previously been used in treatment of neuroretinitis.[2] The patient in this case was treated with doxycycline initially and then later was administered high dose oral prednisone followed by a taper. Her ocular findings have since improved, and she has had resolution of systemic symptoms. Despite 20/20 vision, she does have a slightly enlarged blind spot as well as persistent mild red desaturation in the eye that was affected. Patients usually have excellent visual recovery of 20/40 or better with or without treatment, but in some cases visual improvement does not occur because of permanent optic disc atrophy.[6, 7] In an eye that has been affected by neuroretinitis, it has been noted that the thickness of the nerve fiber layer and the thickness of the macula are significantly diminished, in comparison to the thickness of the layers in the patients fellow eye.[9] This degradation indicates permanent injury that can occur as a result of the disease.

Funduscopically, neuroretinitis can be confused with optic neuropathies, such as papillitis, optic neuritis, hypertensive retinopathy and diabetic papillopathy.[5, 6] Therefore, it is important to comprehensively assess patients with enigmatic eye conditions. This involves taking a full assessment such as blood tests, retinal and neurologic imaging, ocular and spinal fluid analysis, travel history, and subjective patient complaints to gather a complete history and physical for accurate diagnosis and treatment.

Figure 1: Multispectral and autofluorescent imaging of the right eye

Image (A) from January 2018 is a multispectral image that shows optic nerve edema in the right eye and (B) resolution of this edema in May 2019.  Image (C) from January 2019 is a fundus autofluorescence of the right eye which shows hypoautoflourescence surrounding the optic nerve with subsequent improvement in Figure D from May 2019.

 

Figure 2 Color photography and optic nerve and macular OCT images of the right eye

The colored fundus photo of the right eye (A) shows swelling of the optic nerve and its resulting resolution (B). Similarly, the optic nerve OCT depicts swelling (C) and resolution of edema which is seen in (D); also seen is an epi-papillary membrane. Subretinal fluid can be seen in the macular OCT (E) beneath the foveal center, with normalization of the retina in figure (F).

 

Summary of the Case: An 18-year-old female presents with unilateral vision loss accompanied by headache and viral prodrome. An extensive work up and patient history were performed in order to identify the underlying diagnosis of idiopathic neuroretinitis.

References:

  1. Sudharshan, S., S.K. Ganesh, and J. Biswas, Current approach in the diagnosis and management of posterior uveitis. Indian J Ophthalmol, 2010. 58(1): p. 29-43.
  2. Esaki, Y., et al., Multimodal Imaging in a Case of Idiopathic Neuroretinitis. Case Rep Ophthalmol, 2018. 9(3): p. 487-492.
  3. Ray, S. and E. Gragoudas, Neuroretinitis. Int Ophthalmol Clin, 2001. 41(1): p. 83-102.
  4. Attawan, A., I.M. Pepper, and S.J. Hickman, The natural history of idiopathic neuroretinitis. Pract Neurol, 2015. 15(5): p. 387-8.
  5. Abdelhakim, A. and N. Rasool, Neuroretinitis: a review. Curr Opin Ophthalmol, 2018. 29(6): p. 514-519.
  6. Purvin, V., S. Sundaram, and A. Kawasaki, Neuroretinitis: review of the literature and new observations. J Neuroophthalmol, 2011. 31(1): p. 58-68.
  7. Dreyer, R.F., et al., Leber’s idiopathic stellate neuroretinitis. Arch Ophthalmol, 1984. 102(8): p. 1140-5.
  8. Narayan, S.K., S. Kaliaperumal, and R. Srinivasan, Neuroretinitis, a great mimicker. Ann Indian Acad Neurol, 2008. 11(2): p. 109-13.
  9. Berbel, R.F., et al., Evaluation of patients with diffuse unilateral subacute neuroretinitis by spectral domain optical coherence tomography with enhanced depth imaging. Clin Ophthalmol, 2014. 8: p. 1081-7.

Faculty Approval by: Marissa B. Larochelle, MD

Copyright statement: Natalie K. Modersitzki, ©2019. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/