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Show Journal of Clinical Neuro-ophthalmology 7(1): 17-19, 1987. Editorial Comment Syphilis Underground In this issue of The Journal of Clinical Neuro-ophthalmology, there are three articles dealing with different aspects of the problems with syphilis being encountered by physicians in neuro-ophthalmologic practice today. These emphasize the syndrome of optic perineuritis in secondary syphilis (Toshniwal), the importance of recognizing ocular syphilis in patients with AIDS (Zambrano), and the difficulties found in determining the correct dose of penicillin to use in patients with late ocular and neurosyphilis (Poitevin). If one studies the diagnosis and treatment of syphilis, it is evident that the disease has, to a large degree, gone underground and needs exposure. That the incidence of the disease is rapidly rising is seen by the statistics of the United States Communicable Disease Center, * which reveal that in 1956, there were 6,250 cases of infectious syphilis reported, whereas in 1983, 32,698 cases of infectious syphilis were reported. This fivefold increase must be appraised by the fact that any practicing clinician is aware that the majority of cases of late ocular and neurosyphilis seen in private practice are not reported. Thus, the reported numbers represent only the tip of the iceberg. Of 43 million blood tests in the U.S.A. in 1977, 1.5 million tests were reactive (3.5%). These represented untreated syphilis, previously treated syphilis, or false-positive tests. It is important to note that over one-half of all reported early syphilis cases were detected as the direct results of either contact tracing or serologic testing. In the past, the problems encountered by the ophthalmologist and neurologist were not related to infectious (primary or secondary) syphilis, and they were dealing with an entirely different population. The ophthalmologist found patients with abnormal pupils, optic atrophy, pigmentary retinopathies, traumatic dislocated lenses, or old interstitial keratitis, and often found • Presently, the Centers for Disease Control. 17 riJ 1987 Raven Press, New York nonreactive serum reagin (VORL or RPR) tests, yet reactive serum FTA-ABS (fluorescein treponemal antibody absorption) tests in such patients. That this is a frequent problem is evident to any ophthalmologist who actually obtains serum FTAABS tests. At the Bascom Palmer Eye Institute in Miami, blood tests were evaluated on 1,985 patients between September 1963 and February 1957; 718 cases showed reactive serum FTA-ABS tests. However, only 441 patients had a reactive serum VORL tests. Thus, 39% of the cases of late syphilis would have been overlooked if only a serum reagin test had been obtained. The FTA-ABS test was found to be reactive in 41% of the 1,985 sera tested. A recent confirmation of the high incidence of serum FTA-ABS test reactivity was presented by Spoor et al. at the 1986 American Academy of Ophthalmology meeting. They drew serum FTAABS tests on 227 patients in Detroit, Michigan prior to ambulatory outpatient oculoplastic surgery. They found that 52% of these patients had reactive serum FTA-ABS tests. However, the situation has now become even more explosive because of the cases of early infectious syphilis presenting to large city eye clinics. Young male patients between 17 and 35 presenting with an acute red eye-iritis, keratouveitis, vitritis, optic neuritis, optic perineuritis, big blind spot syndrome, neuroretinitis, and retinitis -require prompt serologic testing with both reagin (VORL and/or RPR tests with titers) and serum FTA-ABS tests. This is because of the extremely high incidence of syphilis in the male homosexual population. The indications for cerebrospinal fluid testing continue to cause controversy in eye and neurology departments. In patients with asymptomatic late syphilis, Wiesel et al. (1985) reported that lumbar puncture offers little additional benefit. However, Spoor et al. performed cerebrospinal 18 J. LAWTON SMITH fluid examinations on 50 patients who presented eye signs of late syphilis (usually optic atrophy, old chorioretinitis, or chronic iritis) and found that 33 (66%) had abnormal spinal fluid. Twenty patients (40%) had lymphocytic pleocytosis ranging from 5 to 214 cells. Spinal fluid protein was elevated greater than 45 mg % in 18 patients (36%). Pleocytosis and elevated protein was present in 8 (16%). Only 12 of the 50 patients (24%) had reactive serum VORL tests with titers ranging from 1 to 16 dils. None of the 50 patients had reactive VORL tests in the cerebrospinal fluids. The point Spoor et al. emphasized was that the abnormal cells and protein findings in the spinal fluids of these patients were consistent with late syphilis despite nonreactive cerebrospinal fluid VORL tests. This is quite consistent with the classic Konigsberg figures on blood and spinal fluid Wassermann reactions in neurosyphilis as viewed in Stokes (1944). There are many problems now begging investigation. A series of 100 cases with AIDS should be studied with serum VORL, FTA-ABS, and TPHA tests. I have heard of three patients with clinical signs of late syphilis who had nonreactive serum FPHA tests but reactive FTA-ABS tests. The incidence of that needs to be carefully determined with a larger series of patients because of the frequency of laboratories shifting from the FTA-ABS to TPHA tests because of automation. Without belaboring these points, the following merit emphasis: 1. Syphilis will not be diagnosed without continued clinical suspicion on the part of the physician. 2. To obtain only a serum reagin (VORL or RPR) tests without a serum FTA-ABS test will allow you to miss 40% of the cases of late ocular and neurosyphilis going through your practice. 3. The practice of certain laboratories of requiring a reactive reagin (VORL or RPR) test before performing an FTA-ABS or TPHA (Treponema pallidum hemagglutination test) reveals only lack of familiarity of the problems seen in diagnosing late (ocular and neurological) syphilis and a preoccupation with the findings seen in primary and secondary (infectious) syphilis. One needs to deal with a higher authority with confronted with such obstacles. 4. It is inadequate to get an FTA-ABS test on spinal fluid and not on peripheral blood. There has never been a documented reported case of a patient with a reactive spinal fluid FTA-ABS but with a nonreactive serum FTA-ABS test, I Clill Nellro-ophlhall1lol, Vol. 7, No. I, 1987 whereas it is quite common to find reactive serum FTA-ABS tests in patients with nonreactive spinal fluid VORl and FTA-ABS tests. 5. Once a serum FTA-ABS test is reactive, it usually remains reactive throughout the life of the patient, any amount of treatment notwithstanding. Thus, a reactive FTA-ABS test is quite comparable to a positive tuberculin skin test; it simply means former antigenic contact with the organism and does not convey information about current clinical status or adequacy of therapy at all. Finally, to help the reader who is interested in this problem, some pertinent references are included. This is not meant to be all inclusive, but these papers are primarily those seen in the past few years dealing with problems addressed above. We invite letters to the editor documenting interesting similar experiences. If the reader will begin to obtain serum FTA-ABS tests more frequently in suspicious cases, the following statements will prove to be true in his or her experience. "And have no fellowship with the unfruitful works of darkness, but rather reprove them" and "But all things that are reproved are made manifest by the light-for whatsoever doth make manifest is light." It only requires one little lighted match in mammouth cave to destroy an awful lot of darkness! We are currently using large doses of intravenous aqueous penicillin (3-4 million units every 4 hours by indwelling venous intracatheter) in hospital for 7-14 days in patients with active ocular and neurosyphilis. It appears that even 100 to 200 million units may not eradicate the organism late in the disease. The old experience that retreatment of patients with syphilitic eye disease may provide additional clinical improvement to the patient appears to be developing a stronger laboratory base as is seen from the report of Poitevin et al. J. Lawton Smith REFERENCES Arruga J. Valentines J, Mauri F., Roca G., Salo MR Rufj, G. Neuroretinitis in acquired syphilis. Ophthalmology 1985;92:262-70. Bayne LL, Yee MHC, Swanson RA, et al. Acute neuroyphilis: six cases in three years, four with prior penicillin treatment. Neurology 1984;34(Suppl):137. Bayne, LL, Schmidley, JW, Goodin, DS: Acute syphilitic meningitis- its occurrence after clinical and serologic cure of secondary syphilis with penicillin G. Arch Neural 1986; 43(2):137-8. EDITORIAL: SYPHILIS UNDERGROUND 19 Cohen, MS, Gibson G, Olarte MR. Lissauer form of paretic neurosyphilis; forgotten but not gone. Ann. Neural. 1982; 11(2):219. Collart, P, Poitevin, M. Is penicillin therapy always infallible in syphilis? J Clin Neuro-ophthalmoI1982:2(2):77-83. DeLuise, VP, Clark SW, Smith, JL, Collart, P: Syphilitic retinal detachment and uveal effusion. Am J Ophthalmol 1982; 94:757-61. Fletcher WA, Sharpe, JA: Tonic pupils in neurosyphilis. Neurology 1986;36:188-92. Folk JC, Weingeist TA, Corbett JJ, Lobes LA, and Watzke, RC: Syphilisi neuroretinitis. Am I OphthalmoI1983;95:480-6. Harner, RE, Smith JL, Israel, CW: The FTA-ABS in late syphilis. A serological study in 1,985 cases. lAMA 1968; 203(8):545-8. Hart, G. Syphilis tests in diagnostic and therapeutic decision making. Ann. Int. Med. 1986;104:368-76. Holmes, KK. Syphilis Harrison's principles of internal medicine, New York: McGraw-Hill, 1983;1034-45. Kline, LB, Jackson, WB: Syphilis optic perineuritis and uveitis. In: Neuro-ophthalmology Focus 1980. Smith JL, ed. New York: Masson, 1979;77-84. Kranias, G, Schneider, 0, Raymond LA: A case of syphilitic uveitis. Am I OphthalmoI1981;91:261-3. Markovitz OM, Beutner KR, Maggio RP, et al. Failure of recommended treatment for secondary syphilis. JAMA 1986; 255:1767-8. Moskovitz BL, Klimek JJ, Goldman RL et al. Meningovascuiar syphilis after "appropriate" treatment of primary syphilis. Arch Intern Med 1982;142(1):139-40. Poitevin, M, Collart, P, Boigert, M. Syphilis in 1986 and its problems. J Clin Neuro-ophthalmoI1987;7(1): Ross WH, Sutton HFS. Acquired syphilitic uveitis. Arch OphthalmoI1980; 98:496-8. Rush JA, Ryan EJ: Syphilitic optic perineuritis. Am I Ophthalmol 1981;91:404-6. Sacks, JG, Osher, RH, Elconin, H. Progressive visual loss in syphilis optic atrophy. I Clin Neuro-ophthalmoI1983;3(1):5-8. Schwartz, LK and O'Connor, GR. Secondary syphilis with iris papules. Am. J. Ophthalmol. 1980;90(3):380-4. Smith, JL. Editorial: Whither late ocular and neurosyphilis? J Clin Neuro-ophthalmoI1982;2(2):75-6. Smith, JL. Editorial: Syphilitic optic atrophy. I Clill Neuro-ophthaimol 1983;3(1):3-4. Spoor, TC, Wynn, P, Hartel, We, Bryan, CS: Ocularsyphilisacute and chronic. I Clill Neuro-ophthalmol 1983;3(3):197203. Spoor, TC, Wynn, PJ, Dasco, C. Ocular syphilis In: Neuro-ophthalmology Now Chicago; Year Book Publishers, 1986;299310. Spoor Te, Ramocki, JM, Nesi, FA, Sorscher, M: Ocular syphilis 1986 (Fta-ABS reactivity and cerebrospinal fluid findings) presented at 1986 American Academy of Ophthalmology (in press). Stokes, JH. Modern clinical syphilology. Philadelphia: WB Saunders. 1944:992-4. Tramont, EC: The case against benzathine penicillin as the treatment for neurosyphilis. In: Smith US, ed. Neura-ophthalmology update New York: Masson, 1977;325-8. Toshniwal, P: Optic perineuritis with secondary syphilis. I Clin Neura-ophthalmoI1987;7(1): Weinstein JM, Lexow SS, Ho P., and Spickards A: Acute syphilitic optic neuritis. Arch OphthalmoI1981;99:1392-5. Wiesel, J, Rose ON, Silver AL, et al. Lumbar puncture in asymptomatic late syphilis. Arch Intern Med 1985;145:465-8. Zaidman GW, Weinberg RS, Humphrey WT. acquired syphilitic uveitis in homosexuals. Ophthalmology 1983;90(Aug Suppl):I06. Zaidman, GW: Neurosyphilis and retrobulbar neuritis in a patient with AIDS. Alln OphthalmoI1986;18(9):260-1. Zambrano, W, Perez GM, Smith, JL: Acute syphilitic blindness in acquired immunodeficiency syndrome. I Clin NeuroopthalmoI1987; 7(1): Ziaya, PR, Hankins, GDV, Gilstrap, LC, Ha!sy, AB: Intravenous penicillin desensitization and treatment during pregnancy. lAMA 1986;256(18):2561-2. J elin Neuro-ophthalmol, Vol. 7, No.1, 1987 |
