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Page 2 of 7 Montabone et al. Neuroimmunol Neuroinflammation 2019;6:7 I http://dx.doi.org/10.20517/2347-8659.2019.09

peripapillary retina, taking a characteristic macular star shape. Usual presentation is monocular central
[1]
visual loss with discromatopsia and papillitis on funduscopic evaluation .

The differential diagnosis includes inflammatory optic neuropathies such as sarcoidosis, systemic lupus
[2]
erythematosus, Behçet syndrome, inflammatory bowel disease, and paraneoplastic syndromes . Other
causes are infections: herpetic viruses, HIV, West Nile virus and rarely flu or mumps, toxoplasmosis and
[3]
toxocariasis, syphilis, Lyme disease, tuberculosis, rickettsiosis, and cat-scratch disease (CSD) . CSD is
[4]
related to Bartonella species and transmitted through skin lesions after contact with infected animals . A
[5]
contact with cats is reported in 90% of CSD, and scratches or bites in 60% of the patients .
Typical CSD presentation is a small papule at the inoculation site, followed by regional and systemic
lymphadenopathy and fever. Ocular involvement occurs in 5%-10% of the patients, mostly presenting
Parinaud syndrome (fever, regional lymphadenopathy, and follicular conjunctivitis). Neuroretinitis occurs in
[6]
1%-2% of the patients , and other neurological manifestations are meningoencephalitis, myelitis, and acute
[7,8]
polyneuropathies .

Neuroretinitis is characterized by subretinal fluid, retinal thickening, and hyper-reflective exudates in the
[9]
outer plexiform layer at optical coherence tomography. Fluorescein angiography shows fluid leakage, due
to increased vessel permeability.

Currently, antibiotic therapy for Bartonella complicated infection with doxycycline and rifampin is
[10]
recommended, either alone or in combination with corticosteroids. Habot-Wilner et al. showed a
significant visual acuity improvement in those patients treated with antibiotics and corticosteroids if
compared to those receiving only antibiotics. In the majority of cases no further progression of ocular
[11]
inflammation was observed after therapy, and visual acuity recovered after 1-4 weeks .

CASE REPORT
A 44-year-old woman presented to the Emergency Department with a progressive visual loss and altered
color perception in the right eye, started one week before with no ocular pain, headache, dizziness, nausea
or vomiting.

Her past medical history included Hashimoto’s disease, treated with hormonal replacement therapy in the
last 10 years. Particularly, the patient denied previous neurological or visual deficit. Her familial history was
negative for neurological, vascular or autoimmune disease, and both her parents suffered from colorectal
cancer. About two weeks before, she had fever and was treated with amoxicillin and clavulanic acid (875/125 mg b.i.d)
for otitis. She reported a mild indolent cervical lymphadenopathy occurred 5-7 days after fever, and denied
any cutaneous manifestation. Routine blood examinations were normal, including reactive C protein (0.05
mg/dL, N.V. < 1.00 mg/dL). She had neither fever, night sweats, weight loss or other constitutional symptoms
nor lymphnod involvement at the examination.

Ophthalmological findings
Ophthalmological examination revealed severely compromised visual acuity (1/10 in right eye vs. 8/10 in
left eye), and computerized visual field testing evidenced a temporal loss in right eye. Ocular tonometry was
normal. At the slit lamp the anterior segment was regular with normal pupillary reflexes, but there was a
right relative afferent pupillary defect. On that side, fundoscopy showed marked papillary oedema involving
macular region, hard retinal exudates with appearance of macular star, increased retinal vessel tortuosity and
arteriovenous nicking. Left eye fundoscopy showed milder signs of inflammation with moderate edema. A
fluorescein angiography confirmed the presence of retinal edema with leakage and optic coherence tomography
(OCT) showed retinal thickening with initial exudates within the outer plexiform layer [Figure 1].