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Show , I'IHh Rawn Pre", New York Optic Neuritis In Cat Scratch Disease Paul W, Brazis, M.D., Hunter R. Stokes, M.D" and F. Richard Ervin, M.D. A patient with optic neuritis is described whose a..,socia ted lYmphadenopathY, c,ltexpllsurl', and positiYe cat scratch antigen skin test suggested the diagnosis 01 cat scratch disease. Optic nL'uritis and othl'r neurologic abnormalities, especially encephalopathY, may be associated with, or the presenting manifestation of, cat scratch disease. To our knowledge, this is the fourth case of cat scratch disease-related optic neuritis to be reported. From the Departments 01 Neurology (P. W. B. L Ophthalmology (H.R.S.), and Inll'ctious Diseases (F.R.E.), McLeod Rl'gional Medical Centl'r, Florl'nce, South Carolina. Addrl'ss Cllrrl'spondence and reprint requests to Paul W. Brazis, M.D., SOh E. Cheves St., Suite 206. Florencl', SC 2lJSO!. U.s.A /72 Cat scratch disease (benign lymphoreticulosis) is usually a self-limited infection characterized by chronic regional lymphadenopathy often preceded by a primary skin lesion following cat contact or scratches (1). Neurologic complications are rare, but include meningitis, radiculopathy, polyneuropathy (1), encephalopathy (2), myelopathv (3), and cerebral angiitis H). In this paper we do~ument a case in which optic neuropathy was the presenting and sole manifestation of neurologic inyolvement in a patient with cat scratch disease. To our knowledge, this is the fourth case of cat scratch disease-related optic neuritis to be reported. CASE REPORT A 17-year-old, right-handed male was admitted after complaining of visual difficulty. The patient described the abrupt appearance of a "purple hole" in his right e~'e 2 da~'s previously. He had no history of neurologic or ophthalmologic difficulties and denied e~'e or head pain. Vague upper respiratory s~'mptoms and a low-grade fever were noted also during this time. He had acquired a cat 1 month previousl~' and subsequently developed right submandibular swelling 2 weeks later. On careful questioning, he admitted that the cat would often pla~' around, and occasionally scratch, his lower face and at times sleep against his neck. Neurologic ex.amination was normal except for abnormalities of the right eye. The visual acuity (corrected) was 20/25 in the right eye. The optic disc was markedly swollen and hyperemic with dilated surface capillaries. Numerous peripapillary retinal hemorrhages were present. A relative afferent pupillary defect was noted in the right eye. Visual field examination via tangent screen showed a right central scotoma. The left eye had normal acuity, color vision, pupillary reactivity, OPTIC NEURITIS IN CAT SCRATCH DISEASE /73 and visual fields. The temperature was 101° F. A large, tender right submandibular lymph node was palpated on gent'ral examination. The white blood cell count was H,HOO with 56'1" polymorphonuclear It'ukocytes, 23'1, lymphocytes, 2'7c monocytes, 2';; eosil1l)phils, and 17% band forms. An enhanced computed tomography scan with orbital views was normal. A lumbar puncture yielded clear. coll)r!ess cert'brospinal fluid with an opening pressure of IH cm H20. Spinal fluid protein was 30 mg/dl. and glucose was 63 mg/dl; the fluid also contailwd four white blood cells (alllymphocytes) and two red bll)od cells. The IgG index was normal. Epstein - Barr virus serology was consistent with previl)us immunity. The RPR, T4, T3, FANA, B12 level. and erythrocyte sedimentation rate were all normal. Cat scratch skin test antigen (South Carolina State Laboratory reagent) was positive. Because of the continued visual complaints from the patient. prednisone was administered in a dosage of 40 mg daily for 7 days, then 20 mg daily for an additional 7 days. Symptomatology gradually resolved over several weeks and, at follow-up 4 months later, the optic disc was normal and the right submandibular adenopathy was markedly reduced in tenderness and size. All symptoms and signs had resolved completely 6 months later. DISCUSSION The patient's exposure to a cat, plausible regional inoculation, significant regional lymphadenopathy, and a positive skin test to cat scratch antigen all strongly support the clinical diagnosis of cat scratch disease. Although false positive skin tests may occur in 50/( of normal persons (5). the absence of other etiologies for this patient's syndrome and the benign subsequent course lend further evidence for the diagnosis. To our knowledge, this is the fourth case of optic neuritis which has been documented in association with cat scratch disease. The first case was that reported by Sweeney and Drance (6) of a 39year- old male patient who developed typical acute optic neuritis approximately 10 days after the onset of adenopathy. Cerebrospinal fluid study revealed four lymphocytes and two polymorphonuclear cells per cubic millimeter. The patient received adrenocorticotropic hormone, and later oral corticosteroids, and had significant improvement in visual acuity when seen 10 months after initial presentation. Gass (7) mentioned the development of optic nerve involvement in a child recovering from cat scratch disease, but no further information was recorded. Perkins and Rose (8) mention a third patient who developed papillitis and ocular pain 6 weeks after receiving a cat scratch to the lower lid. Inflammatory cells were noted in the vitreous and anterior chamber, but cerebrospinal fluid studies were normal except for an elevated protein of 80 mg/dl. Full recovery was noted after 16 months. The ocular findings in our patient were diagnostic of optic neuritis and similar to those described by Sweeney and Drance (6). Corticosteroids were given to our patient also, but their usefulness in this rare complication of cat scratch disease has not been proven and remains largely anecdotal. It is noteworthy that the etiology for our patient's illness was unsuspected until the lymphadenopathy was found and a careful history was obtained. Findings of optic neuritis should prompt a careful examination of the entire head and neck for other abnormalities. The other, and much more common, ophthalmologic complication of cat scratch disease is the ocular glandular disease of Parinaud (7,8), in which a localized conjunctival granuloma occurs often associated with injection, chemosis, and follicular response. This granuloma is often polypoid in configuration, measuring 0.5-2 cm in diameter, and has an irregular border. As the granuloma ages, it may enlarge and develop areas of necrosis. Encephalopathy is the most frequently reported neurologic complication of this disease (2), with lethargy and confusion often progressing to coma and convulsions. Motor weakness, signs of meningeal irritation, radicular pain, myoclonus, and respiratory arrest all may occur. Cerebrospinal fluid is frequently normaL but may show a mild pleocytosis and elevated protein. Fortunately, most reported cases with neurologic involvement show complete recovery without residuaL but convalescence may be prolonged. The etiology of cat scratch disease is unknown, but recently a bacterial agent (a gram-negative pleomorphic bacillus) has been suggested to be the causative agent (11). REFERENCES 1. Margilt'lh. AM. Cat scratch disease: a bacterial infection. In: Mandell GL. Douglas RG Jr. Bennett JE. eds. Principles alld practice of i"lec/iolls diseases. 2nd ed. New York: J. Wiley & Sons. 1985:1381-3. 2. Torres JR, Sanders CV, Strub RL, Black FW. Cat-scratch disease causing reversible encephalopathy. JAMA 1978;240:1628-9. 3. Pickerill RG. Milder JE. Transverse myelitis associated with cat-scratch disease in an adult. lAMA 1981;246:2840-1. 4. Selby G, Walker GL. Cerebral arteritis in cat-scratch disease. Nellrology 1979;29:1413-8. I C1il1 Neuro-ophthalll1ol. Vol. 6, No, 3. 1986 IN P. W. BRAZIS ET AL. :i. B"ker AB. Vir,ll L'I1l'l'ph"hti, 111: B"kL'r AB. B"ker LH, ed,. Clllli""IIIl·III'1J/"S.I/. I'hil"LtL-lphi": ILlrper & Row, I'IH5; Ilh-l). h. SWL'L'I1L'V VI', Dr,lIlce SM. Optic lWlIriti, "nd cOlllprL'"ivL' I1L'lIrL1p.,tll\' .1'SllL'i,lted with C,lt ,cr"kh disL''''L'. COli Med :\SStl<' I 1l)70; 1m: J]HO~ I. 7. C,ISS IDM. DisL',lSL'S 01 till' optic l1erve th.ll m,ly ,imlilatL' 1ll.1ClIl,lr disL',lSL'. rl'lillS /\/11 lI<'IId Ul'htllllllll,,1 ()/"lan/lIx,,1 1l)77;HJ:7hJ-70. / ellll NClfnH1/'htlllllmol, Vol. 6, No.3, 198b H. Perkins GO, Rose FC Optic neuritis and its differential diag"" sis. Oxford: Oxford Medical Publications, 1979. 'I. Carithers HA. OculogJandular disease of Parinaud: a manifestation of cat-scratch disease. Am I Dis Child 1'!7H;132:11'!5-1200. lO. Maichuk IF. Viral eye diseases. Rev Int Trach Pathol Owl rr,,1' Subtwl' 1977;54:7-108. II. Wear OJ. Margileth AM, Hadfield TL. et al. Cat scratch disease: a bacterial infection. Science 1983;221:1403-4. |
