44-year-old male with a history of optic neuropathy OD in 2011
Presents to LAC+USC Medical Center in May 2016 with pain OS associated with superotemporal injection
Pain is intermittent, 10/10, shoots from the periorbital area across the left temple to the left side of the head
No tenderness to palpation of the left temple; no jaw claudication
No photophobia
In May 2016 patient was diagnosed with idiopathic scleritis; started on topical and oral steroids with taper
September 2016 patient presented with decreased vision OS with persistent (though improved) pain and injection
Exam Findings
VAsc
OD: 20/100
OS: 20/50 PH 20/40
Pupils: Round and Reactive OU, 2+ RAPD OD
Ishihara color plates: OD 1/8 , OS 6/8 (decreased OS from prior exam of 8/8)
IOP OD: 12, OS: 12
External exam
Violaceous injection of the scleral vessels (non-blanching with phenylephrine), more predominate in the superotemporal quadrant
No scleral thinning
Slit Lamp Exam (pertinent positives and negatives)
K: clear OS
AC: deep and quiet OS
Iris: flat, round, no nodules
Vitreous: syneresis, no cell OS
Posterior segment
Media: clear
ON: OD Pale; OS CDR 0.1, hyperemic with increased vascularity temporally and temporal swelling, no obscuration of the vessels coursing over the nerve, no hemorrhages
Macula: flat OU, no exudates OS
Vessels: normal, no vascular sheathing
Periphery: normal, no holes, tears or detachments
Figure 1: Color disc photograph of the right eye demonstrates optic disc atrophy (CDR 0.1).
Figure 2: Color disc photograph of the left eye, cup-to-disc ratio of 0.1, the disc appears hyperemic with increased vascularity temporally with associated temporal swelling. There is no obscuration of the vessels coursing over the nerve. No hemorrhages.
Differential Diagnosis
Elevated ICP
Inflammation
Infection
Demyelination
Sarcoidosis
Vasculitis
Compression (Foster-Kennedy syndrome)
Infiltration
Additional Investigations
Fluorescein angiogram
Demonstrated early disc leakage with late staining OS
Figure 3: Fluorescein angiogram of the left eye demonstrates in A.arterial phase early temporal disc leakage and in B.venous phase late temporal disc staining.
Visual fields (Humphrey Visual Field 24-2)
OD: reliable, inferior altitudinal defect, enlargement of the blind spot
OS: reliable, normal
Figure 4: Humphrey Visual Field 24-2 of the right eye: reliable, notable for an inferior altitudinal defect and enlargement of the blind spot, classic findings in optic neuropathy.
Figure 5: Humphrey Visual Field 24-2 of the left eye: reliable exam, normal visual field exam.
In 2011 the patient had tested positive for a “venereal disease” and was treated with 2-3 intramuscular injections
Diagnosis
Ocular syphilis (Neurosyphilis)
Pathophysiology
Syphilis is an infection of the spirochete T. Pallidum. It is spread through direct contact with a chancre; transmission occurs during sexual contact. Pregnant women can also transmit the disease through the placenta within the first 16 weeks of pregnancy.
Syphilis is characterized by the phases of infectious process:
Ocular syphilis is considered Neurosyphilis from a management standpoint and its manifestations vary widely through the anterior and posterior segments
Microhemagglutination test for antibodies to T. pallidum (MHA-TP)
T. pallidum particle agglutination assay (TPPA)
T. pallidum enzyme immunoassay (TP- EIA)
Chemiluminescence immunoassay (CLIA)
Positive for life
Negative Non-Treponemal Test
For most patients this precludes a diagnosis of active syphilis
If symptomatic consider:
Early empiric treatment (seroreversion)
In early syphilis: Testing could be prior to Antibody formation, or prozone reaction (2 percent)
Late syphilis: advanced immunosuppression (B cell failure), natural history
Interpretation of CSF
VDRL in CSF = highly specific; poor sensitivity
If positive establishes the diagnosis
FTA-ABS can be ordered = sensitive; not specific
CSF-VDRL false+: traumatic tap when serum nontreponemal titer high
Pleocytosis >5 and Protein >45 is consistent with the diagnosis, but is also non specific
Non-specific pleocytosis also occurs in setting of HIV infection and makes interpretation difficult
Treatment
Treat ocular syphilis as neurosyphilis
Aqueous crystalline penicillin G (18 to 24 million units per day)
3 to 4 mil units IV q 4 H 10 to 14 days
Desensitization in patients with severe allergy
May consider doxycycline and ceftriaxone as alternatives in patients with severe allergy (excluding pregnancy)
References
Gajula V, Kamepalli R, Kalavakunta JK. A star in the eye: cat scratch neuroretinitis. Clinical Case Reports. 2(1):17.
Hicks, CB and M Clement. Syphilis: Screening and diagnostic testing, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on January 08, 2017)
Marra CM. Neurosyphilis. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on January 15, 2017)
Lee SY, Cheng V, Rodger D, Rao N. Clinical and laboratory characteristics of ocular syphilis: a new face in the era of HIV co-infection. J Ophthal Inflamm Infect. 2015 Dec; 5(1):56
Liu L, Lin L, Tong M, Zhang H, Huang S, Chen Y, et al. Incidence and Risk Factors for the Prozone Phenomenon in Serologic Testing for Syphilis in a Large Cohort. Clinical Infectious Diseases (2014);59(3):384-389.
Read RW, Acharya N, Levinson RD, Rao PK, Sen HN, Walker JD, et al. Section 09, Intraocular Inflammation and Uveitis. In: Cantor LB, Rapuano CJ, Cioffi GA, 2016-17 AAO, Basic and Clinical Science Course. Section 9: Intraocular Inflammation and Uveitis, 2016:359.
Weisenthal, RW, Afshari, NA, et al. Cantor LB, Rapuano CJ, Cioffi GA. 2016-2017 AAO, Basic and Clinical Science Course. Section 8: External Disease and Cornea, 2016:359.
