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Show Journal of Clinical Neuro-ophthalmology 7(4): 196-197, 1987. Editorial Comment Syphilis/Lyme/AIDS In this issue of the Journal, there are two major articles updating our knowledge of spirochetal infections in neuro-ophthalmology-one by Spoor dealing with Treponema pallidum and another by MacDonald dealing with Borrelia burgdorferi. Since these papers dealing with ocular and neurologic findings in syphilis and Lyme disease were prepared, however, a series of important papers has appeared about these disease entities and raising a major question about the influence of human immunodeficiency virus (HIV) and the acquired immune deficiency syndrome (AIDS). Johns et al. (1) documented four cases of neurosyphilis seen in the past 18 months at the Massachusetts General Hospital, all in young homosexual men who were seropositive for HIV virus. Two of them had neurosyphilis despite "adequate" previous therapy for early syphilis with benzathine penicillin. Meningovascular syphilis developed in one patient within 4 months after a primary infection, which suggested an accelerated course of syphilitic infection to these authors. Several important questions were raised by these authors, and indeed their paper prompted an editorial response in the same issue (2). There are so many new developments in this field that it is difficult to develop a proper title for this editorial! "Spirochetes Strike Again!", "AIDS aids syphilis!", "Syphilis/Lyme/AIDS", what should it be? It would appear that due to the limitations of space only a few highlights can be cited here, but a list of additional pertinent references will be cited that should help the interested reader begin to appreciate the extremely important and rapid developments now going on in this field of medicine. Before pointing out the extreme importance of Lyme disease (Borrelia burgdorferi) to the ophthalmologist and neurologist, it would be of interest to note the very first case cited in Johns et al. (1). Four months before admission, a 38-year-old homosexual male was seen elsewhere with penile chancres, and conjunctival injection and blurred vision 'in the right eye. Serum rapid plasma reagin (RPR) Vv';'\S reactive with titer 0' 1:512. He received © 1987 Raven Press, Ltd., New York one intramuscular injection of 2.4 million U of benzathine penicillin. However, his vision continued to drop, he lost 20 lbs, and hearing decreased in the left ear. Occipital headache, slightly stiff neck, mental dullness began, and on the day of admission, an abrupt decrease in right eye vision prompted hospitalization. Examination revealed posterior uveitis with a nearly complete retinal detachment in the right eye. The left eye was normal. A sensorineural hearing loss was present in both ears. He showed reddish-purple macules on both ankles. A scleral buckling procedure was performed on the night of admission. Spinal fluid examination revealed 111 lymphocytes, protein 130, VDRL reactive 1:8, FTA-ABS reactive 2+, HIV culture was positive, serum RPR was reactive 1:256, and serum FTA-ABS was reactive 4 +. He developed a mild left pure-motor hemiparesis, with the lesion not being seen on CT scan, but a small pontine infarct was visible on magnetic resonance (MR) imaging. The patient was treated with intravenous penicillin (20 million U/day), and showed a mild Herxheimer reaction after the first dose. The spinal fluid rapidly cleared after this treatment. The patient was doing relatively well 8 months after completion of therapy, although the vision in his right eye remained poor. This case reminds me of a case reported by DeLouise et al. (3). The significance of obtaining serum FTA-ABS tests for syphilis cannot be more eloquently stressed than by Spoor's finding that of 247 consecutive patients in a Detroit ambulatory oculoplastic surgery clinic, the test was reactive in 120 patients-over half of those tested! You may be asking, but what about Lyme disease? Two reports may suffice here. Steere et al. (4) reported on a 45-year-old woman who developed erythema chronicum migrans (the classic skin lesion after a tick bite) and 4 weeks later presented with unilateral iritis in the left eye followed by a violent panophthalmitis. Spirochetes morphologically consistent with B. burgdorferi were found in the specimen removed at vitrectomy, de- 196 EDITORIAL COMMENT 197 spite large doses of antibiotics given to this patient. Indirect immunofluorescence titers for B. burgdorferi were strongly positive initially and dropped sequentially thereafter. One swallow may not make a spring time, you say! Wu et al. (5) reported on a 7-year-old boy who was found to have bilateral papilledema, but spinal fluid opening pressure was 170 and the fluid contained 14 leukocytes. CT scan was normal. This was, in all reasonable probability, optic perineuritis or optic neuritis with good vision because in hospital his vision dropped to 20/40 in both eyes, and enlarged blind spots and centrocecal scotomas were found in both eyes. Indirect immunofluorescence titer was 1:256, and he responded to 1.5 million U of penicillin G q4h i.v. for 10 days. One year later, vision was on the 20/20 line in both eyes, and he was free of visual complaints. It is now known that Lyme disease does essentially everything that syphilis does. Nine of 40 sera from patients with known Lyme disease were reactive at 1:5 dilution on serum FTA-ABS test (6). Thus, about 22% of patients with Lyme disease will show a reactive serum FTA-ABS test if one orders this blood test by itself! Lyme disease has now been found in over 32 states. A very important paper by Rawlings (7) points out that the Borrelia burgdorferi organism has been cultured in 14 cases since commencement of this procedure in the spring of 1985. Of these 14 positive cultures, seven cases (50%) showed no detectable serum antibody to the Lyme disease spirochete. In other words, seronegative Lyme disease exists beyond question. With the realization of the propensity of Lyme disease to cause Bell's palsy (unilateral or bilateral), other cranial nerve palsies, painful radiculoneuropathy syndromes, chronic lymphocytic meningitis, as well as inflammatory eye syndromes, such as iritis, vitritis, optic neuritis, optic perineuritis and the like, the reader is urged to begin looking for this newly recognized spirochetal infection. One can order the blood test for $30 from Microbiology Reference Laboratory, 10703 Progress Way, Cypress, CA 90630-4714 (phone 1-800-445-0185). Ask for "Lyme disease antibody." You will get IFA-IgG and IFA-IgM titers. It requires 1 ml serum. Dr. MacDonald has raised the question of amyotrophic lateral sclerosis syndromes due to Lyme disease (called Bannwarth's syndrome in Europe), as well as a possible relationship to Alzheimer's disease. He has pointed out that Rocephin (ceftriaxone, Roche) 2 g i.v. q12h for 14 days has been considered as 100 times as effective against B. burgdorferi as intravenous penicillin therapy. The possibility of a Herxheimer reaction at the beginning of treatment might make a pretreatment dose of 50 mg oral prednisone as a single dose 1-2 h before onset of treatment worth considering. There appears to be an evil triune relationship between T. pallidll1ll, B. burgdorferi, and human immunodeficiency virus in not only causing untold human suffering but in proving exceedingly cunning in hiding from the clinician's diagnostic abilities. However, light is coming forth and always dispels darkness! We invite letters to the editor of this Journal if you, dear readers, are encountering interesting instances similar to those noted here, and particularly with neuro-ophthalmologic manifestations of Borrelia burgdorferi infection. J. Lawton Smith, M.D. REFERENCES 1. Johns DR, Tierney M, Felsenstein D. Alterations in the natural course of neurosyphilis by concurrent infection with human immunodeficiency virus. N Eng/ I Med 1987; 316(25): 1569-72. 2. Tramont EC. Syphilis in the AIDS era. N Eng/ I Med 1987;316:1600-1. 3. DeLouise vr, et al. Syphilitic retinal detachment and uveal effusion. Am I Ol'htlza/mol 1982;94:757-61. 4. Steere AC, et al. Unilateral blindness caused bv infection with the Lyme disease spirochete, Borrelia bllrg,iorferi. Arm lnt Med 1985;103(3):382-4. 5. Wu G, et al. Optic disc edema and Lyme disease. Arm Ol'htha/ moI1986;18(8):252-5. 6. Hunter EF, et al. Evaluation of sera from patients with Lyme disease in the FTA-ABS test for syphilis. Sex Trans Dis 1986;13(4):232-6. 7. Rawlins JA, et al. Isolation of Borrelia bllrgdorferi spirochetes trom patients in Texas. I Clin Microbio/1987;25(7):1l48-50. , Clill Neuro-ophthallllo/' Vol. 7, No.4. 1987 |