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Show Journal of Clinical Neuro- ophthalmology 10( 4): 264- 265, 1990, Editorial Comment Cogan's Syndrome and Lyme Serology © 1990 Raven Press, Ltd" New York We are excited to present the report on Cogan's syndrome by Drs. Wilder- Smith and Roelcke in this issue of the Journal because of our recent encounter ( 1) with a patient with classic Cogan's syndrome of nonluetic interstitial keratitis and vestibuloauditory dysfunction with positive serologic tests for Lyme borreliosis. We wrote Dr. WilderSmith who kindly obtained additional serum specimens on their patient and these were submitted to the Alfred Fournier Institute in Paris, the French national center of reference for the treponematoses, and also to the Microbiology Reference Laboratory ( MRL) in California. As noted in the text, all syphilis serologic tests ( VORL, TPHA, FTAABS, and TPI), done through the courtesy of Mme. Paris- Hamelin, were negative in Paris. MRL also found nonreactive RPR and FTA- ABS tests, as well as a negative Lyme ELISA ( index 0.57) and IFA IgG « 1: 16) and IgM « 1: 16). Thus, the patient with Cogan's syndrome reported here was totally seronegative for these spirochetoses as confirmed in three different laboratories. One might at first thus discount the possibility that Lyme borreliosis can be an etiologic factor in some cases of Cogan's syndrome. However, the problem with the clinical diagnosis of seronegative Lyme borreliosis is a very real one at this time. We have a sheet of instructions for patients and physicians showing the serologic workup we now advise when there are definite clinical suspicions of Lyme disease. This tells how to obtain a Lymel treponemal panel, Lyme Western blot, and, if negative, then to proceed to get a Lyme lymphocyte stimulation assay and a Lyme PCR ( polymerase chain reaction) test. One can obtain information in this regard either directly from MRL, 10703 Progress Way, Cypress, CA 90630- 4714 or by calling them at 1- 800- 445- 0185. If you would like a copy of our sheet, you can obtain one by writing Suzanne Van Meter, c/ o Dr. J. L. Smith, 00. SCOm Palmer Eye Institute, P. O. Box 0168~( J, Miami, FL 33101. We have recently 1 a ! XltIt'nt with a history 264 consistent with late Lyme borreliosis who had a negative IFA and a negative ELISA test for B. burgdorferi infection, but who showed a definitely positive Western blot test for this disease, with 5 bands including the 41 kd band. We called Dr. Porschen, director of MRL, and he also performed a Lyme Western blot test on the remaining serum from Dr. Wilder- Smith's patient and reported that as negative as well. A 39- year- old man presented to our clinic June 1 with the history of an acute onset malaise, fever, and abnormal liver function tests starting in early May, and within 3 days of being hospitalized developed abruptly a profound drop of vision in both eyes and an associated bilateral severe deafness. On examination through the courtesy of Dr. M. Reynolds, the patient's vision was down to hand motions in both eyes with a fulminating bilateral hemorrhagic granulomatous iritis and secondary glaucoma in both eyes. He was quite deaf and one had to yell at him despite his wearing a hearing aid in order to get any history. His spinal fluid was clear, and his peripheral blood RPR was nonreactive. One laboratory reported a nonreactive serum FTA- ABS test, but this was repeated in our laboratory and found to be positive. The patient admitted having gonorrhea on at least two occasions, and was treated for gonococcal urethritis 3 years ago with one injection of penicillin and a week of oral antibiotic tablets. Because of the history of venereal exposure, inadequate penicillin therapy for syphilis, and a reactive serum FTA- ABS test, the patient was treated with intravenous aqueous penicillin G 4 million units every 4 h for 2 weeks. Examination yesterday Gune 14, 1990) showed that with a pinhole his vision was 20150 in the right eye and 20/ 40 in the left eye, and he could now hear whispered voices without a hearing aid. Thus, this patient referred for suspected Cogan's syndrome actually had " Pseudo- Cogan's syndrome" rlue to syphilis, and responded dramatically to pen' - illin therapy alone. COGAN'S SYNDROME 265 The old question of " seronegative syphilis" is obviously related to " seronegative Lyme disease." One can ask: " What is the definition of seronegative syphilis"? Is a patient with a nonreactive serum RPR and VDRL test who has a nonreactive FTA- ABS but a positive TPI " seronegative"? The answer will be different when interpreted from the standpoint of the investigative serologist than when encountered by the clinician treating the patient. Is a patient with a history consistent with late Lyme borreliosis who has a negative Lyme IFA, ELISA, and Western blot, but a positive PCR and positive lymphocyte stimulation assay, " seronegative Lyme disease" or not? Before these questions are resolved, we need to always remind ourselves that diagnosis is a clinical function, and that the laboratory confirms the clinical impression; it does not displace or supersede it. Until these problems are resolved, if I encounter a patient with suspected Cogan's syndrome, I would continue not only to obtain careful serologic testing for both syphilis and Lyme borreliosis as noted above, but if I elected to treat the patient with systemic steroids, I would certainly advise covering that therapy with concomitant intravenous antibiotic therapy. Thank you, Dr. Wilder- Smith and Dr. Roelcke for your interesting report. We hope to hear from our readers' additional encounters with Cogan's syndrome. J. Lawton Smith, M. D. REFERENCE 1. Fox GM, Heilskov T, Smith JL. Cogan's syndrome and seroreactivity to Lyme borreliosis. J Clin Neuro Ophthalmol 1990; 10: 83- 7. JClin Neuro- ophthalmol, Vol. 10, No. 4, 1990 |
