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Show Journal of Clinical Neuro-ophthalmology 7(]): 6-10. 1987. Optic Perineuritis with Secondary Syphilis Pradip Toshniwal, M.D. © 1987 Raven Press. New York Optic perineuritis is characterized by swollen optic discs in the absence of raised intracranial pressure and visual dysfunction. A patient with secondary syphilis who presented with these features is described. The need to recognize the spectrum of ocular and optic nerve involvement in secondary syphilis is emphasized. Key Words: Optic perineuritis-Papilledema-Secondary syphilis. From the Division of Neurology, Cook County Hospital, Chicago, Illinois. This work was presented at the IXth International Congress of Infectious Diseases and Parasitology, Munich, July 1986. Address correspondence and reprint requests to Pradip Toshniwal, M.D., Division of Neurology, Cook County HospitaL 1835 West Harrison St., Chicago, IL 60612, U.s.A. 6 The advent of antibiotic therapy and changing social values have led to a marked change in the epidemiology of syphilis (1). The incidence of late syphilis has declined steadily, while that of early syphilis has increased, particularly among homosexuals. Central nervous system and visual dysfunctions in late syphilis are well recognized. However, visual dysfunction in early syphilis is not as well emphasized. For instance, a recent major textbook of infectious disease mentions only anterior uveitis and visual failure among manifestations of secondary syphilis (2). The spectrum of visual system manifestations of secondary syphilis is wide and includes conjunctivitis (3), iritis (4,5), anterior and posterior uveitis (6-8), chorioretinitis (9), vasculitis (10-12), papillitis (4,9,12), neuroretinitis (12,13), optic neuritis (14-16), optic perineuritis (7,17-21), papilledema (18), and a syndrome mimicking idiopathic orbital inflammation (9). Optic perineuritis is a syndrome characterized by swollen optic disc or discs, normal vision and fields except for enlarged blind spots, and normal cerebrospinal fluid (CSF) pressure (18). This report describes a patient who meets these criteria. CASE REPORT I.G., a 39-year-old, previously healthy, Hispanic woman, presented with a I-month history of right-sided frontotemporal throbbing headaches. The headaches occurred two to three times every day, lasted 2-3 h each time, and were relieved by aspirin. For 3 weeks, she had a rash which started in the arms and legs and then involved the trunk. Palms and soles were involved also. Three days prior to presentation, she developed itching and watering from the left eye and started to see "stars in front of the eyes." Examination showed generalized maculopapular rash involving palms and soles, thought to be characteristic of secondary syphilis by the con- A OPTIC PERINEURITIS 7 B FIG. 1. Fundus photographs. A: Elevated, hyperemic disc with peripapillary hemorrhages and edema on the right. B: Hyperemic disc on the left. suIting dermatologist. Left conjuctiva was congested. Cornea, anterior chamber, lens, and vitreous were normal. Pupils were 4 mm, round, equal, and normally reactive. Visual acuity was 20/20 in the right eye and 20/25 + 2 in the left eye with correction. She could read eight of eight Ishihara plates with either eye. Fundoscopy showed elevated, hyperemic disc with peripapillary hemorrhages on the right and a hyperemic disc on the left (Fig. 1). Retina was normal. Visual fields showed enlarged blind spot on the right (Fig. 2). Serum VDRL was positive, with a titer of 1:64. Serum fluorescent treponemal antibody absorption (FTA-ABS) and T. pallidum hemagglutination assay (TPHA) were also positive. Computerized tomography scan of the brain with and without contrast infusion was normal. A cerebrospinal fluid (CSF) examination showed opening pressure of 220 mm, protein 19 mg/dl, glucose 51 mg/dl, and red and white blood cell counts of 34 and 5 per mm3, respectively. Latex agglutination for Haemophilus inf/uenzae, meningococcus, and pneumococcus, and bacterial cultures were negative. CSF VDRL was nonreactive. Fundus fluorescein FIG. 2. Visual fields. A: Normal field on the left; B: enlarged blind spot on the right. JGill Neuro-ophthalrnol. Vol. 7, No.1, 1987 8 P. TOSHNIWAL A FIG. 3. Fluorescein angiography shows late dye leakage from both discs, worse in the right eye (A). B angiography showed increased vascularity and persistent dye leakage from both discs, but worse on the right (Fig. 3). Visual evoked responses showed well-defined wave forms with normal latency and amplitudes of PlOO (Fig. 4). The patient was treated with intravenous crystalline penicillin, 2 million units every 2 h for 10 days. There was an early increase in itching, which was thought to be secondary to JarischHerxheimer reaction. The headache, rash, and papilledema then slowly resolved without any residual symptoms or signs. Three months later, VORL titer was 1:4 and FTA-ABS and TPHA were still positive. DISCUSSION In the patient described, swollen optic discs without symptomatic visual dysfunction and normal CSF pressure in the presence of skin features of secondary syphilis, positive serology, and satisfactory response to penicillin therapy are consistent with a diagnosis of syphilitic optic perineuritis (18). The absence of chemical and cellular abnormalities in CSF suggests relative restriction of the meningeal inflammation to the optic nerves. It is interesting to note that, in the present patient, color vision as tested by Ishihara plates was normal and visual evoked responses showed normal wave form, amplitude, and latency, attesting to the lack of functional abnormality of the visual system. Whether contrast sensitivity evaluation or ",' 7l!1IPf, Vol. 7, No, L 1987 more detailed color vision testing with the Farnsworth- Munsell test would have detected subtle subclinical visual dysfunction remains open to question. The absence of discernible visual dysfunction may result from the fact that most tests of acuity, color vision, and electrophysiology eval- 0.5. 100 FIG. 4. Visual evoked potentials show normal wave form, P,oo latencies, and amplitUdes with monocular stimulation bilaterally. OPTIC PERINEURITIS 9 SWOLLEN DISC I VISUAL FUNCTION NORMAL I CSF PRESSURE ABNORMAL I OCULAR INFLAMMATION NORMAL I OPTIC PERINEURITIS ABNORMAL ABSENT PRESENT I , I MENINGITIS OPTIC NEURITIS NEURORETINITIS HYDROCEPHALUS PAPILLITIS UVEITIS FIG. 5. Algorithm describes the differential diagnosis of swollen optic discs with secondary syphilis. uate central fibers responsible for macular vision, while in perineuritis, pathology is restricted to peripheral optic nerve fibers, as discussed below. Optic perineuritis with syphilis was described by Uhthoff and Wilbrand and Saenger (17). Clinical description of a case was provided by Walsh and Hoyt (18). The patient described by Kline and Jackson was unusual in that the clinical features included decreased visual acuity, bilateral arcuate scotomas, and evidence of vitreous inflammation (7,24). This case underscores the confusion associated with the term "perineuritis" in pathologic and clinical contexts, as discussed below. Rush and Ryan reported a patient who meets the clinical criteria of perineuritis and appropriately cautioned about the possibility of confusion with papilledema and pseudotumor cerebri (20). In this context, it may be useful to mention that other processes, which can present with swollen discs, enlarged blind spot, and no or minimal visual impairment include papillophlebitis (22), big blind-spot syndrome (23), disc drusen (24), incipient ischemic optic neuropathy (25), diabetic papillopathy (26), dysthyroid optic neuropathy (27), cyanotic congenital heart disease, cavernous hemangioma, and rickettsial infection (24). Two other patients with optic perineuritis have been reported by Luxon et al. (19) and Bayne et al (21). The latter patient is of special interest, because the optic perineuritis and meningitis developed after clinical and serological cure of the initial manifestations of syphilis with penicillin therapy. Early studies on the optic nerve pathology in syphilis demonstrated chronic inflammation of the surrounding meninges, particularly concentrated around the vessels (17,28,29). The process mayor may not extend into the peripheral parts of the nerve along the intraneural septa. Such a process may result gradually in the loss of peripherally located nerve fibers, which correlates with the concentric constriction of the visual field encountered in syphilitic optic atrophy. While in terms of pathology both situations with or without nerve fiber loss may be described by the term "perineuritis," it nonetheless remains clinically useful to restrict the use of this term to cases without nerve-fiber dysfunction and visual impairment and to describe other cases by the term "optic neuritis." More generalized optic-nerve inflammation and vasculitis involving the arteries and veins are described also (29). Optic neuritis occurs most frequently in the secondary stage (18). Numerous recent reports and a review attest to the importance of recognizing this fact in view of the present epidemiology of syphilis. Optic-disc swelling with syphilis is said to occur with meningitis in 90% of the cases, most often in the secondary stage (18). More recent data about relative frequency of different pathogenic mechanisms are not available. It is important to recognize, however, that disc swelling in secondary syphilis can occur due to a variety of different processes, which can be distinguished on the basis of the presence or absence of visual dysfunction and meningeal and/or ocular inflammation (Fig. 5). REFERENCES 1. Holmes KK. Syphilis. In: Petersdorf RG, Adams RD, Braunwald E, Isselbacher KJ, Martin IB, Wilson JD, eds. Harrison's principles of intenza/ medicine. New York: McGraw-Hill, 1983;1035-6. 2. Tramont EC. Treponema pallidllln (syphilis). In: Mandell Gl, Douglas RG, Bennett JE, eds. Principles and practice of infectious diseases. New York; John Wiley, 1985;1323-33. 3. Wuepper KD. "Red eye" as the presenting sign of syphilis d'emblee. Calif Med 1967;107:518-20. 4. Parker JDJ. Uncommon complications of early syphilis: hepatitis, periostitis, iritis with papillitis and meningitis. Br J Vener Dis 1972;48:32-6. 5. Schwartz lK, O'Connor GR. Secondary syphilis with iris papules. Am I Ophthalmol 1980;90:380-4. 6. Schlaegel TF. Granulomatous uveitis. An etiologic survey of 100 cases. Trans Am Acad OphthalnzoI1958;62:813-25. 7. Kline lB, Jackson WB. Syphilitic optic perineuritis and uveitis. In: Smith Jl, ed. Neuro-ophthalmolog\f focus 1980. New York; Mason, 1979;77-84. . JClill Neuro·oph/halmol, Vol. 7, No.1, 1987 10 P. TOSHNIWAL 8. Ross WH, Sutton HFS. Acquired syphilitic uveitis. Arch OphthalmoI1980;98:496-8. 9. Spoor Te, Wynn P, Hargel WC, Bryan CS. Ocular syphilis. Acute and chronic. JClin Neuro-ophthalmoI1983;3:197-203. 10. Smith JL. Acute blindness in early syphilis. Arch Ophthalmol 1973;90:256-8 11. Couch ER, Goldberg MF. Retinal periarteritis secondary to syphilis. Arch OphthalmoI1975;193:384-7. 12. Folk Je, Weingeist TA, Corbett JJ, Lobes LA, Watzke RC. Syphilitic neuroretinitis. Am JOphthalmoI1983;95:480-6. 13. Arruga J, Valentines J, Mauri F, Roca G, Salo MR, Rufi G. Neuroretinitis in acquired syphilis. Ophthalmology 1985;92: 262-70. 14. Graveson GS. Syphilitic optic neuritis. J Neurol Neurosurg Psychiatry 1950;13:216-24. 15. Lorentzen SE. Syphilitic optic neuritis: a case report. Acta OphthalmoI1967;45:769-72. 16. Weinstein JM, Lexow SS, Ho P, Spickards A. Acute syphilitic optic neuritis. Arch OphthalmoI1981;99:1392-5. 17. Wilbrand H, Saenger A. Die Neurologie des Auges, vol. 3. Wiesbaden: JF Germann, 1904;45-53. 18. Walsh FB, Hoyt WF. Clinical neuro-ophthalmology, 3rd ed. Baltimore: Williams and Wilkins, 1969;609-10, 1581-91. 19. Luxon L, Lees AJ, Greenwood RJ. Neurosyphilis today. Lancet 1979;1:90-3. I crill NCllro-nphllzalmol , Vol. 7, No.1, 1987 20. Rush JA, Ryan EJ. Syphilitic optic perineuritis. Am J Oph-' thalmoI1981;91:404-6. 21. Bayne LL, Schmidley JW, Goodin OS. Acute syphilitic meningitis. Arch NeuroI1986;43:137-8. 22. Hayreh 55. Optic disc vasculitis. Br J Ophthalmol 1972;56: 652-70. 23. Miller NR. The big blind spot syndrome: unilateral optic disc edema without visual loss or increased intracranial pressure. In: Smith JL, ed. Neuro-ophthalmology update. New York: Masson, 1977;163-9. 24. Miller NR. Walsh and Hoyt's clinical neuro-ophthalmology. 4th ed. Baltimore: Williams and Wilkins, 1982. 25. Hayreh SS. Anterior ischemic optic neuropathy. V. Optic disc edema as early sign. Arch Ophthalmol1981; 99:1030-40. 26. Lubow M, Makley TA Jr. Pseudopapilledema of juvenile diabetes mellitus. Arch OphthalmoI1971;85:417-22. 27. Trobe JO, Glaser JS, LaFlamme P. Dysthyroid optic neuropathy: clinical profile and rationale for management. Arch OphthalmoI1978;96:1199-209. 28. Harriman OGF. Bacterial infections of the nervous system. 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