| OCR Text |
Show Itll/ mal of Clillical Neuro- ol''' thul", olog.~ 8( 1): 39-- 43. 1988. Optic Neuropathy Associated with Chronic Lymphomatous Meningitis James R. Coppeto, M. D., Mario L. R. Monteiro, M. D., and Dante B. Cannarozzi, M. D. .{; 1988 Raven Press, Ltd .. New York A patient with paranasal sinus lymphoma ( recognized retrospectively) developed unilateral, acute, self- limited optic neuritis during the course of chronic lymphocytic meningitis with elevated intracranial pressure and headache. Meningeal symptoms were adequately controlled with analgesics alone for 14 months and corticosteroids alone for a subsequent 11 months without evidence of development of other involvement of organs outside the central nervous system ( CNS). Eventually, the visual alteration from optic neuritis prompted a repeat evaluation, which disclosed lymphoma in bone marrow. The subject of para neoplastic optic neuritis is reviewed. Key Words: Chronic atrophic papilledema- Chronic meningitis- Lymphomatous meningitis- Optic neuritis, lymphoma. From St. Mary's Hospital ( J. R. C., D. B. C. J, Waterbury, Connecticut, U. S. A.; the Department of Ophthalmologv. Hospitdl das Clinicas, University of Sao Paulo Medicdl School ( M. L. R. M.), Sao Paulo, Brazil; and the Department of Laboratory Medicine, Yale University School of Medicine ( D. B. C.). New Haven, Connecticut, U. s. A. Address correspondence and reprint requests t( l Dr. lames Robert Coppeto at 1906 North Main Street, Waterbury, CT 06704, U. SA 39 Non- Hodgkin's lymphoma can rarely present as meningitis or as optic neuropathy ( 1,2). The disease can either arise primarily in the central nervous system ( CNS) or invade it secondarily and is usually rapidly fatal when not treated ( 3,4). We present a patient with a 2- year history of chronic " lymphocytic" meningitis manifesting bilateral papilledema, intermittent headache, hearing loss, and visual loss. Despite extensive investigations, the diagnosis was not made until a positive bone marrow biopsy was obtained 2 years later. It is remarkable that this patient was stable for 14 months without any treatment and continued to do well on corticosteroid treatment alone for a subsequent ll- month period. CASE REPORT In January 1983, a 51- year- old man developed bifrontal headaches. Bilateral papilledema was the only abnormality on physical examination. A lumbar puncture disclosed a pressure of 240 mm and clear cerebrospinal tluid ( CSF) with 1740 leukocytes/ mm3 ( 97 clr lymphocytes) that " appeared reactive, not neoplastic," 10 erythrocytes/ mm3, protein of 151 mg/ dl, glucose of 49 mg/ dl ( serum glucose of 109 mg/ dl). CSF showed negative staining for fungi and bacteria, including mycobacteria, negative cryptococcal antigen A and B, and negative India ink preparation. Serum hematocrit and leukocyte counts, chest x- ray, brain scan, serum glutamic- oxaloacetic transaminase ( SCOT), serum glutamic- pyruvic transaminase ( SCPT), lactate dehydrogenase ( LDH), VORL, FTA- ABS, and anti- nuclear antibody ( A A) were normal. Erthrocyte sedimentation rate ( ESR) was 88 mm/ h. In June, he developed retro- orbital pain in the left eye that was attributed to ethmoid and maxillary sinusitis visible on a skull radiograph. Bilat- OPTIC NEUROPATHY IN LYMPHOMATOUS MENINGITIS 4/ , "" i,,; I<- 1- · ~" .. ! i• .... \ . .. "... FIG. 1. Bone marrow infiltration by malignant lymphoid cells ( x 33) . regated from the systemic circulation. Our patient's disease, however, differed from primary leptomeningeal lymphomas because the disease was also present initially in a paranasal sinus. We could not find similar cases of chronic " lymphocytic" meningitis lasting more than a few months where the case was ultimately proved to harbor a preexisting associated lymphoma elsewhere in the body. Our patient had rapid visual loss with vision down to finger counting and associated with pain upon eye movement and absent visual evoked potentials. Treatment with prednisone produced rapid recovery, and vision remained stable afterward. As our patient was maintained on low- dose prednisone after that episode, it is possible that visual loss was the result of optic nerve infiltration from lymphomatous cells that were very sensitive to prednisone and were kept under control with this medication. However, the clinical evolution in our case, including pain upon eye movement, the lack of optic nerve enlargement or compression by a mass lesion on CT scan that is generally present in optic nerve lymphomatous infiltration ( 2,8), and the lack of recurrences of visual loss suggests optic nerve demyelination. We believe that our case expands the clinical spectrum of optic neuropathies reported to be associated with lymphomas into the arenas both of acute optic neuritis and chronic atrophic papilledema. Kline et al. ( 8) reviewed optic neuropathies associated with a variety of lymphomas. Almost invariably, the optic neuropathy is severe, destructive, and progressive. Unlike our case, corticosteroids do not bring about sustained improvement unless radiotherapy or chemotherapy are added. An exception may be the case of Kraus and 1Clill N" lIro- ol'lrthalmol, Vol. 8, N, J. 1, 1988 42 J. R. COPPETO ET AL. FIG. 2. Nasal sinus mucosa infiltration by similar malignant lymphoid cells ( x33). O'Rourke ( 9), in which the patient appeared to suffer a demyelinating self- limited optic neuritis that responded quickly to a limited course of corticosteroids and did not recur despite a year of follow- up. That the optic neuritis in our case, was not coincidentaL but rather para neoplastic, is partly supported by the presence of oligoclonal immunoglobulin in the CSF that presumably was produced by malignant or reactive lymphocytes in the CSF. These observations suggest to us that in patients with lymphoma, the optic nerve may be involved by a demyelinating process and not just direct infiltration of the optic nerve. Peripheral demyelinative neuropathy has also been reported in patients with lymphoma sometimes as the presenting sign of the disease ( 10). Our patient had oligoclonal im- I' I ' 11 ., . ; lin, ll fluid, but not in the - ,!, Ie th, lt he had optic nerve demyelination from disordered CSF immunocompetence. Paraneoplastic optic neuritis is one of the rarest forms of optic neuritis. Pillay et al. ( 11) reported subacute visual loss from pathologically verified optic nerve demyelination and perineuritis in a case of bronchiogenic carcinoma with widespread encephalitis. Interestingly, that case, like ours, harbored a lymphocytic meningitis. Unlike our case, however, that case showed no visual recO\' ery. Boghen et al. ( 12) related the histopathology of a case remarkably similar to the latter case which, however, showed partial visual recO\' ery. Their case, like ours, demonstrated oligoclonal bands in the CSF. Aside from this episode of transient visual loss, our patient also had chronic papilledema from long- standing increased intracranial pressure. When we saw him 2 years after the beginning of OPTIC NEUROPATHY IN LYMPHOMATOUS MENINGITIS 43 his symptoms, he had a bilateral mild deutan dyschromatopsia, visual field constriction, and the appearance of chronic atrophic papilledema. Although such changes are not uncommon with other causes of chronic increased intracranial pressure, including pseudotumor cerebri, we are not aware of other cases of atrophic papilledema from lymphomatous CNS involvement. Part of the cause for the delayed diagnosis in our case was the mistaken belief that such a protracted course was incompatible with lymphoma. In actuality, such an evolution is not only compatible with primary leptomeningeal lymphoma, but, as shown by our case, may also be compatible with other low- grade lymphomas. We therefore recommend peripheral blood Band T cell studies and bone marrow evaluations on all patients with cryptogenic chronic meningitis. Moreover, detailed analyses, inclusing Band T cell studies, of the lymphocytes of the spinal fluid sediment are important. The question arises as to why this patient's CSF lymphocytes initially appeared benign on cytological examination. One possibility is that the lymphoma was so well differentiated that ordinary staining techniques were nondiscriminating without testing for monoclonality. Alternatively, a reactive lymphocyte cell component in the material may have overshadowed the malignant cells ( 13). It is unlikely that our patient's optic neuritis was related to an occult pulmonary adenocarcinoma, as he had no evidence of other paracarcinomatous neurological dysfunction, and malignant CSF cells were all lymphoid. However, we cannot exclude that this apparent paraneoplastic optic neuritis was wholly or partly related to his presumed coexisting carcinoma. ACKNOWLEDGMENTS: The authors thank their technical and secretarial staff for their expert assistance. REFERENCES 1. Griffin JW, Thompson RW, Mitchinson Mj, Kiewiet Jc, Weiland FH. Lymphomatous leptomeningitis. Am J Med 1971; 51: 200- 8. 2. Kay Me. Optic neuropathy secondary to lymphoma. J Clin Neuro- aphtlzal 1986; 61 : 31- 4. 3. Budka H, Pilz P, Guseo A. Primary leptomeningeal sacromatosis. I Neurol 1975; 211 : 77- 93. 4. Rax I, Siegal T, Polliack A. CNS involvement by nonHodgkin's lymphoma. Response to a standard therapeutic protocol. Arch Neurol 1984; 41: II67- 71. 5. MacKintosh FR, Colby TV, Podolsky WJ, et al. Central nervous system involvement in non- Hodgkin's lymphoma: an analysis of 105 cases. Cancer 1982; 49: 586- 95. 6. Onofrio BM, Kernohan JW, Uihlein A. Primary leptomeningeal sarcomatosis. A review of the literature and report of 12 cases. ell/ ar 1962; 15: 1197- 1208. 7. Kepes JJ, Maxwell JA, Hedeman L, Slaven J. Primary diffuse malignant lymphoma of the leptomeninges prese'nting as " pseudotumor cerebri." Neurochirurgia 1971; 14: 188- 96. 8. Kline LB, Garcia JH, Harsh GR. Lymphomatous optic neuropathy. Arch OphthalmaI1984; 102: 1655- 7. 9. Kraus AM, O'Rourke J. Lymphomatous optic neuritis. Arch Ophtha/ mol 1963; 70: I73- 5. 10 Sumi SM, Farrell OF, Knauss TA. Lymphoma and leukemia manifested by steroid- responsive polyneuropathy. Arch Neural 1983; 40: 577- 82. 1I. Pillay N, Gilbert JJ, Ebers GC, Brown JD. Internuclear ophthalmoplegia and " optic neuritis": para neoplastic effects of bronchial carcinoma. Neur% glf 1984; 34: 788- 91. 12. Boghen 0, Michaud J, Sebag M. Oculomotor paresis and optic neuritis. Presentation at the annual Frank B. Walsh Society meeting, Feb. 22- 23, 1985. 13. DeVita VT. Lymphocyte reactivity in Hodgkin's disease: lymphocyte civil war ( editorial). N Ellgil Med 1973; 289: 801. 1Clill Neuro- ophthalllwl, Vol. 8, No. 1, 1988 |
