Bilateral ocular perineuritis as the presenting feature of acute syphilis infection

Abstract

We describe the case of a 48-year-old man who presented with a 2-month history of recurrent, transient visual obscurations that were not exacerbated by straining or coughing. During this period, he felt unwell with headaches, malaise, and flu-like symptoms. Visual symptoms improved but failed to resolve to two courses of oral amoxicillin prescribed for a presumed upper respiratory tract infection. On examination, he had a normal body mass index. Bilateral disc edema (Fig. 1a) and absent ocular spontaneous venous pulsations were identified. Visual acuity (corrected), color vision, and visual fields were all normal. Slit-lamp examination confirmed disc swelling but was otherwise normal. No other neurological deficits were detected and general medical examination was normal. MRI brain, MR venogram, and MR angiogram were all normal. A lumbar puncture examination revealed an opening pressure of 19 cm of CSF (normal range 12–22 cm of CSF). The CSF white cell was 1, protein 0.32 g/dl (normal range 0.15–0.45) with normal glucose. Positive unmatched oligoclonal bands were detected but there was a normal IgG index. Blood tests, including a full blood count, urea and electrolytes, thyroid function tests, serum protein electrophoresis and anti-nuclear, extractable nuclear antigens, and anti-neutophil cytoplasmic antibodies were normal or negative. An HIV test was negative, however, an initial screen for syphilis demonstrated a positive VDRL and RPR IgM, with a titer of 1:32. Further studies including Treponema pallidum IgM enzyme immunoassay and Treponema pallidum particle agglutination were positive with a titer of [1/80, indicating acute infection. CSF treponemal studies were negative. On further questioning, the patient recalled painless ulceration of his glans penis 12 weeks prior to presentation. He had not sought medical attention and it had resolved spontaneously. He had a long-term female partner but reported unprotected intercourse with another female 16 weeks prior to presentation. We diagnosed ocular perineuritis appearing as bilateral disc swelling as a presenting sign of active syphilis. The patient made a full clinical recovery with resolution of the visual symptoms and signs following appropriate antibiotic therapy. Syphilis has reemerged within Europe and North America since the late 1990s. In Ireland in 2008, 221 cases (4.9/100,000) were reported [1], the majority of which being diagnosed in men who have sex with men. HIV coinfection is associated with higher rates of transmission and increased severity of symptoms [2]. Increases are considered to be a consequence of unprotected sexual encounters rather than antibiotic resistance [3]. Ocular manifestations of syphilis as the presenting feature are limited to case reports and small case series [4–9]. Symptoms can be unilateral or bilateral. The most common ocular presentation is uveitis [8], but chorioretinitis, retinitis, keratitis, retinal vasculitis, and optic neuropathy are reported, occurring at any stage of the disease [10]. Previous reports of syphilis presenting as optic disc swelling as a result of papillitis or perioptic neuritis have been associated with other evidence of central nervous system involvement as demonstrated by a CSF pleocytosis, raised protein, or positive CSF syphilis studies [4–9]. K. O’Connell (&) M. Marnane C. McGuigan Department of Neurology, St. Vincent’s University Hospital, Elm Park, Dublin, Ireland e-mail: kazzoc@hotmail.com