Neuroretinitis

This 21YO male presented with one month of intermittent headaches and vision loss in his left eye. Vision was 20/20 in his normal OD and 20/60 OS.

Color photography of the macula shows patches of yellow-white inner retinal opacification with superior and temporal lipid. OCT shows outer nuclear layer fluid with hyperreflective dots of lipid in the outer plexiform layer. Mild vitreous cells are present.

A presumed clinical diagnosis of Bartonella neuroretinitis was made, although serology could not be performed due to cost concerns. He was started on oral doxycycline 100mg PO BID and oral corticosteroids. Two weeks later, vision was 20/40 with resolving retinal opacification and fluid (not shown). Following a full month of oral therapy, vision improved to 20/20 with decreasing lipid and no fluid.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia and later lipid exudation within Henle’s layer (neuroretinitis), including cat scratch (Bartonella) and syphilis. When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis.

This healthy 32YO female presented with a 10-day history of headaches, followed by vomiting and blurred vision in her right eye. She also reported a rash on her hands and legs that appeared before her visual symptoms. Vision was 20/300 OD and 20/20 in her normal OS.

Pseudocolor SLO imaging shows a swollen optic nerve, a serous detachment extending through the fovea, and some mostly temporal radiating foveal lipid. OCT scanning shows outer nuclear layer fluid with hyperreflective lipid deposits and subretinal fluid. Fundus fluorescein angiography shows marked disc leakage. Infectious disease consultation revealed a negative infectious workup, and she was started on intravenous followed by a tapering dose of oral steroids.

One week later, vision was 20/300 with a striking new lipid star from lipid in the outer plexiform layer. There is a marked reduction in the intra- and subretinal fluid. Her findings continued to improve, and 4 months following presentation, vision was 20/20 with faint residual lipid.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia (not seen in our case) and later lipid exudation within Henle’s layer (neuroretinitis), including cat scratch (Bartonella) and syphilis. When no underlying cause is found, as in our patient, the entity is called Leber’s idiopathic stellate neuroretinitis.

This healthy 35YO male presented with recent severe vision loss in his right eye. Vision was counting fingers OD and 20/20 in his normal OS.

Optos color RG imaging shows moderate optic nerve swelling, which is confirmed by OCT. There is moderate nerve leakage on fluorescein angiography. Bloodwork, including syphilis and Bartonella, was negative, so he was observed without treatment.

One month later, vision improved to 20/200. The disc edema was markedly improved, and new lipid radiated nasally from the macular center. Three months later, the disc edema was fully resolved with secondary atrophy, and the lipid was decreasing. Vision was 20/25.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia (not seen in our case) and, later, with lipid exudation within Henle’s layer (neuroretinitis), including cat-scratch (Bartonella) and syphilis. When no underlying cause is found, as in our patient, the entity is called Leber’s idiopathic stellate neuroretinitis.

This 18YO female presented with a several-day history of vision loss in her right eye. Vision was 20/70 OD and 20/40 in her normal OS.

Optos color RG imaging shows inferior disc edema with a serous macular detachment. Triton swept-source OCT confirms the serous detachment extending towards the nerve.

Bartonella serology was positive, and she was placed on a 6-week course of oral antibiotics. Two months following presentation, vision improved to 20/40. The disc edema and serous detachment resolved, with the development of a lipid star.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia (not seen in our case) and, later, with lipid exudation within Henle’s layer (neuroretinitis), including cat-scratch (Bartonella) and syphilis. When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis

This 52YO female presented with one week of bilateral vision loss. She has multiple cats at home. Vision was 20/80 OD and counting fingers OS.

Optos color RG imaging shows an area of inferonasal macular inner retinal opacification OD, and a superiorly swollen left optic nerve with a superonasal macular lipid star.

Fluorescein angiography shows early blockage with late staining of the retinal lesion OD with an adjacent area of vasculitis. Marked optic nerve leakage is seen OS.

Bartonella henselae antibody testing was positive for both IgG >1:1024 and IgM 1:126. She was subsequently lost to follow-up.

Learning Points:
Bartonella henselae is the bacterium associated with cat scratch disease. It is transmitted from cat fleas carrying the bacterium or from flea feces that can be present on the cat’s claws/teeth, and transferred to humans from a cat scratch, lick, or bite. Common systemic findings include fever, malaise, and progressive lymphadenopathy. These findings usually occur within 1-2 weeks of a cat scratch/bite. Ocular effects primarily include neuroretinitis with rare cases of Parinaud’s ocular glandular syndrome, or, as in our patient’s case, chorioretinitis with vasculitis.

This healthy 56YO female presented with 1-2 weeks of blurred vision in her left eye. Vision was 20/25 in her normal OD and 20/100 OS.

Color imaging shows a diffusely swollen left nerve. Optic nerve Triton swept-source OCT B-scan confirms the diffuse retinal nerve fiber layer edema. A few dots of subclinical hyperreflective lipid are noted in the outer plexiform layer. 30-2 visual field testing shows an inferior unilateral arcuate scotoma extending from the blind spot.

Extensive blood work and chest X-ray were negative except for a positive QuantiFERON-TB Gold. Infectious disease consultation led to the diagnosis of latent tuberculosis, and she was started on systemic anti-tuberculous therapy.

Over the following several weeks, as the optic nerve edema resolved, the papillomacular outer plexiform layer lipid became more prominent.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia (not seen in our case) and later lipid exudation within Henle’s layer (neuroretinitis), including cat scratch (Bartonella), Lyme disease, and syphilis. When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis.

Tuberculosis is a rare cause of neuroretinitis (Stechschulte et al, J Neuroophthalmology 1999;19:201-204). It is unclear whether our patient’s findings are idiopathic or related to her latent tuberculosis.

This 36YO female presented with unilateral vision loss in her left eye. She denied any systemic symptoms except for headaches for 3 months. Vision was 20/70 in her right eye and 20/25 in her left eye.

Optos color imaging shows severe optic nerve head edema, a foveal lipid star, dilated and tortuous vessels, and a variable frosted branch appearance to the retinal veins. Fluorescein angiography shows diffuse venous staining with optic nerve leakage.

Extensive blood work was positive for both Bartonella henselae (IgG 1:64) and Bartonella quintana (IgG 1:128), and negative for Bartonella IgM antibodies. Although she has cats at home, she denied cat scratches or exposure to cat fleas. She was referred to an infectious disease specialist who started her on doxycycline 100mg PO BID.

When she returned 6 weeks later, her headaches had resolved, and her vision was 20/80. The optic nerve swelling was dramatically improved with secondary peripapillary pigmentary changes.

Learning Points:
Bartonella henselae is the bacterium associated with cat scratch disease. It is transmitted from cat fleas carrying the bacterium or from flea feces that can be present on the cat’s claws/teeth, and transferred to humans from a cat scratch, lick, or bite.

Common systemic findings include fever, malaise, and progressive lymphadenopathy. These findings usually occur within 1-2 weeks of a cat scratch/bite.

Ocular effects primarily include neuroretinitis, with rare cases of Parinaud’s ocular glandular syndrome and, as in our patient’s case, chorioretinitis with vasculitis.

This healthy 27YO male presented with a few days of a paracentral scotoma in his right eye. Vision was 20/25 OD and 20/20 OS.

Color imaging shows a small inferonasal foveal area of inner retinal opacification. Swept-source OCT through this region shows inner retinal hyperreflectivity. An additional, larger subretinal lesion is noted in the inferonasal midperiphery. OCT scanning through this lesion shows marked choroidal thickening, overlying retinal disorganization and hyperreflectivity, and some surrounding subretinal fluid. Fluorescein angiography shows moderate leakage from this choroidal lesion.

On further questioning, the patient told us that he recently adopted 2 stray cats. He didn’t recall any cat scratches, rashes, or adenopathy, but Bartonella titers were markedly elevated (IgG 1:1280, IgM 1:400).

Following infectious disease consultation, he started a 6-week course of oral doxycycline and rifampin. Four weeks later, vision was 20/20, and both lesions had significantly improved.

Learning Points:
Bartonella Henselae is the bacterium associated with cat scratch disease. It is transmitted from cat fleas (carrying the bacterium) or from flea feces that can be present on the cat’s claws/teeth, and transferred to humans from a cat scratch or bite.

Common systemic findings include fever, malaise, and progressive lymphadenopathy. These findings usually occur within 1-2 weeks of a cat scratch/bite.

Ocular effects primarily include neuroretinitis with rare cases of Parinaud’s ocular glandular syndrome, or, as in our patient’s case, chorioretinitis.

In our practice, posterior segment Bartonella involvement most commonly presents with neuroretinitis. Our patient’s inflammation spared the nerve, with multifocal involvement including the inner retina and choroid.

A 44YO female with type 2 diabetes presented on 4/25/22 with a 2-week history of severe vision loss in her left eye. Vision was counting fingers OS.

Ocular examinations were normal except for a markedly swollen left optic nerve. There were very faint, scattered, hard exudates.

OCT showed an average nerve fiber layer thickness of 370 microns (not shown). Fluorescein angiography shows diffuse optic nerve leakage.

Extensive bloodwork was negative, including Bartonella, syphilis, Lyme, Rocky Mountain spotted fever, CRP, ACE, and serum lysozyme. MRI scanning was also normal.

Over the next 3-4 weeks, the optic nerve edema resolved with the development of a nasal lipid star. Unfortunately, vision remained at counting fingers.

Learning Points:

The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with acute inner retinal ischemia (not seen in our case) and later lipid exudation within Henle’s layer (neuroretinitis), including cat scratch (Bartonella), Lyme disease, and syphilis.

When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis.

This healthy 46YO male presented on 12/27/21 with 1 day of decreasing vision in his left eye. Vision was 20/25 in his normal right eye and 20/60 in his left eye.

The left nerve was swollen with a faint macular lipid star. There were bilateral multifocal scars and an inferotemporal streak lesion in his left eye. Fluorescein angiography shows minimal left optic nerve staining.

An extensive workup, including labs for Bartonella, Lyme, tuberculosis, sarcoidosis, toxoplasmosis, syphilis, and a chest x-ray, was all negative. He denied any systemic symptoms, although he recently had a COVID-19 vaccine. Serology showed positive IgG and negative IgM antibodies for COVID-19.

He was treated with a tapering 1-month course of oral prednisone, and at his 1/26/22 visit, the nerve edema had resolved, with a more prominent macular lipid star.

Learning Points:

The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with inner retinal ischemia and lipid exudation (neuroretinitis), including cat scratch (Bartonella), Lyme disease, and syphilis. When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis. The condition is self-limited, so treatment is not usually recommended.

We gave our patient the presumed diagnosis of Leber’s idiopathic stellate neuroretinitis due to the unilateral swollen nerve followed by a lipid star, in the absence of a positive infectious workup. The bilateral multifocal scars appeared inactive and were likely present long before he presented with a unilateral swollen nerve.

Our best guess is that the scarring represents ocular histoplasmosis or multifocal choroiditis unrelated to the swollen nerve, although we can’t rule out that the two might be related.

This patient presented with 20/200 vision OD with a severely swollen nerve, a secondary central retinal vein occlusion (CRVO), and a large patch of nasal macular neuroretinitis. The left eye was normal.

Three weeks later, the findings had improved spontaneously and dramatically, and vision was 20/50. Some faint superior and nasal lipid was noted.

Bloodwork for syphilis, Bartonella (cat scratch), and Lyme disease was negative.

Learning Points:
The diagnosis for a unilateral swollen nerve is extensive. However, it becomes much smaller when associated with inner retinal ischemia and lipid exudation (neuroretinitis), including cat scratch (Bartonella), Lyme disease, and syphilis.

When no underlying cause is found, the entity is called Leber’s idiopathic stellate neuroretinitis. The condition is self-limited, so treatment is not usually recommended.

This 17YO presented with 20/200 vision OD and diffuse optic disc edema with temporal peripapillary retinitis, peripapillary inner retinal blood, a few areas of focal inner retinitis, and a prominent macular lipid star. The left eye was normal.

Our patient tested positive for Bartonella and had a cat (should’ve gotten a dog and opted for a Toxocara worm instead…).

Patients will usually improve without treatment, although antibiotic therapy may sometimes be recommended.

Learning Points:
Optic nerve inflammation, often from malignant hypertension or neuroretinitis, can cause fluid and blood to leak into the optic nerve and peripapillary tissues. When the fluid absorbs, the lipid can precipitate in Henle’s layer, creating the lipid star.

There are numerous causes for neuroretinitis, including cat scratch (Bartonella), Lyme disease, syphilis, and idiopathic (Leber’s stellate neuroretinitis).

At initial presentation, our patient showed optic nerve edema clinically with inner retinal hyperreflective retinitis and an overlying vitritis on OCT.

A few weeks later the optic nerve and retinal inflammation improved with the development of a classic lipid star.

The lipid, which leaks out of inflamed optic nerve vessels, accumulates in the outer plexiform layer. Since it originates from the nerve, the star is usually most prominent in the nasal macula.

Learning Points:
Neuroretinitis can be infectious or idiopathic. The most common infectious cause is secondary to Bartonella Henselae (Cat-Scratch Disease).