A Rare Case of Pseudo Foster Kennedy Syndrome in a HIV Positive Patient Brief Communication

ISSN 0972-0200 Brief Communication

presentation and a diagnosis of exclusion, with only a few case reports and case series in the literature. In our case A Rare Case of Pseudo also no cause for the asymmetric optic nerve involvement could be found besides the direct effect from the HIV virus. Foster Kennedy Our case had a rare presentation of pseudo-Foster Kennedy syndrome in a HIV positive patient. A Thirty nine years old male patient presented with complaints of sudden onset Syndrome In a HIV defective vision in the right eye since two weeks and in the left eye since four days associated with painful ocular Positive Patient movements. Patient was diagnosed with HIV in 2010 and Delhi J Ophthalmol 2014; 24 (4): 277-278 was on anti-retro viral treatment since then with no other DOI: http://dx.doi.org/10.7869/djo.60 systemic illness. On examination the best corrected visual acuity (BCVA) was 1/60 in both eyes. On ocular examination Maharajan Padmavathy *, Ahuja Ashish A, R.Ramakrishnan** in both eyes the pupils showed afferent pupillary defect. Fundus examination showed disc edema in the right eye A 39 year old male patient presented with complaints of (Figure 1) and in the left eye there was disc pallor (Figure 2). sudden onset diminished vision in the right eye since two MRI of brain (flair, DWI, constrast) showed left optic weeks and in the left eye since four days associated with painful ocular movements. On examination patient had optic disc edema in the right eye and the left eye showed optic disc pallor. Patient was also suffering from human immunodeficiency virus (HIV) infection. In this case study we report that the patient had pseudo Foster Kennedy syndrome directly attributed to HIV. Patient was started on intravenous steroids and responded with the improvement in vision in the right eye. Pseudo foster kennedy syndrome is a very rare disease and HIV as a predisposing factor is even more uncommon . It is important to rule out the opportunistic infections which occur in an HIV positive patient. Steroids have been found to be effective in improving the visual outcome and decreasing the morbidity in such patients. In 1911, Foster kennedy published six cases in which he demonstrated ipsilateral optic atrophy with contralateral Figure 1: Right eye fundus picture at presentation showing Optic disc edema papilledema due to expanding frontal lobe lesions. These signs were pathognomic for space occupying lesion in the region of basofrontal area on the side of the optic atrophy.1 In 1916, Foster kennedy published a paper in which he added ipsilateral anosmia to his previously described signs.2 The Foster Kennedy syndrome then became a triad consisting of ipsilateral optic atrophy, contralateral disc edema, and ipsilateral anosmia. This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary to a space occupying lesion (such as meningioma or plasmacytoma, usually an olfactory groove meningioma). Pseudo foster kennedy syndrome is defined as one-sided optic atrophy with papilledema in the other eye but with the absence of a space occupying lesion.3 HIV infection as a direct cause of optic neuropathy has been postulated. It is an uncommon Figure 2: Left eye fundus picture at presentation showing Optic disc pallor Consultant, Neuro-ophthalmology department & Glaucoma department, atrophy and there was no evidence of demyelination, space Aravind Eye Hospital ,Tirunelveli occupying lesion, infection in the brain parenchyma. Total *Address for correspondence leucocyte count was 4490, Absolute CD4 count - 558 , CMV IgM - negative, RBS - 163mg % , VDRL - negative. The patient Padmavathy Maharajan MS was then started on IV methyl prednisolone 1 gram OD for Aravind Eye Hospital 3 days. Following this patient showed improvement in the S.N. High road, Tirunelveli junction right eye with visual acuity of 6/36 and in the left was 1/60. Tirunelveli, Tamil Nadu 627001 The patient was discharged with oral steroids on tapering Email: [email protected] dose and was asked to follow up after two weeks when his

277 Del J Ophthalmol 2014;24(4) E-ISSN 0976-2892 A Rare Case Of Pseudo Foster Kennedy Syndrome In a HIV Positive Patient Brief Communication

vision improved in the right eye to 6/12 with BCVA of 6/9 factor responsible in HIV induced neuronal apoptosis.8 with resolution of disc edema (Figure 3) but in the left eye the Management of our case was done with IV steroids and it BCVA persisted to be 1/60 with no improvement (Figure 4) . gave a good result. Newman et al reported two cases with bilateral optic neuropathies in HIV positive patients who responded well to treatment, one with steroid and the other with azidothymidine.9 Laurent-Coriat C et al reported a case with bilateral optic neuropathy as a presenting feature in HIV positive patient that developed after meningopolyradiculitis that did not respond to the conventional treatment with oral steroids or anti-viral therapy.10 Incidence of involvement of the anterior segment of the optic nerve reduced from 4 - 8% in the pre HAART era to nearly 0 % in the HAART era.11 HIV may directly lead to unilateral or bilateral optic neuropathy. HIV induced optic neuropathy is a diagnosis of exclusion. Management in such cases can be challenging with multi system involvement. Early detection and treatment of HIV induced optic neuropathy with steroids or highly active anti-retroviral drugs can give promising results. Figure 3: Right eye fundus picture post treatment showing resolution of Optic disc edema Financial & competing interest disclosure The authors do not have any competing interests in any product/ procedure mentioned in this study. The authors do not have any financial interests in any product / procedure mentioned in this study.

References

1. Kennedy F. Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Am J Med Sci 1911; 142:355. 2. Kennedy F. A further note on the diagnostic value of retrobulbar neuritis in expanding lesions of the frontal lobes, with a report of this syndrome in a case of aneurysm of the right internal carotid artery. JAMA 1916; 67:1361. 3. Bansal S, Dabbs T. Long V. Pseudo-Foster Kennedy Syndrome due to unilateral optic nerve hypoplasia: a case report. J Med Case Rep 2008; 1752-1947-2-86. 4. Ahmed M, Gittinger JW Jr. Optic neuritis, Contemporary Figure 4: Left eye fundus picture post treatment showing no improvement Ophthalmology 2009; 17:1-8. 5. Mwanza J-C, Nyamabo LK, Tylleskär T. Plant GT. Neuro- HIV manifests in various ways in the eye. Several optic ophthalmological disorders in HIV infected subjects with nerve disorders have been described, most commonly neurological manifestations. Br J Ophthalmol 2004; 88:1455- resulting from opportunistic infections, neoplasms and 1459. inflammatory causes.4 Mwanza et al described a sub-group 6. Otiti-Sengeri J, Colebunders R, Kempen JH, Ronald A, Sande M, Katabira E.. The Prevalence and Causes of Visual Loss of neurologically symptomatic HIV-infected patients from Among HIV-Infected Individuals With Visual Loss in Uganda, the Democratic Republic of Congo: In their study, Optic Journel of acquired immune deficiency syndromes 2009 nov neuropathies accounted for 31% of cases, although only 7% 19. of cases were ascribed solely to HIV.5 In a study conducted in 7. Sadun AA, Pepose JS, Madigan MC, Laycock KA, Tenhula Uganda by Otiti-sengeri j et al, the incidence of optic nerve WN, Freeman WR. AIDS-related optic neuropathy: a histological, virological and ultrastructural study. Graefes Arch involvement was found to be 14.7 % and bilateral decrease Clin Exp Ophthalmol 1995; 233:387-98. 6 in visual acuity was found in 6 % HIV positive patients. 8. Mahadevan A, Satishchandra P, Prachet KK, Sidappa NB, Presentation of HIV as a pseudo Foster Kennedy syndrome Ranga U, Santosh V,et al. optic nerve axonal pathology is is rare with only handful of cases mentioned in the related to visual evoked responses in AIDS. Acta neuropathol literature. The likely mechanism of the HIV induced direct 2006;112:461-9. damage to the optic nerve was analysed in a study by Sadun 9. Newman NJ, Lessell S. Bilateral optic neuropathies with remission in two HIV-positive men. J Clin Neuroophthalmol et al. They reported axonal degeneration, vacuolation of 1992; 12:1-5. oligodendrocytes and infiltration by mononuclear cells 10. Laurent-Coriat C, Tilikete C, Bouhour D, Boulliat J, Fleury which was indicative of optic nerve degeneration occurred J,Bernard M, Vighetto A. HIV infection presenting with due to HIV infected macrophages.7 The current knowledge bilateral optic neuropathy, Rev Neurol (Paris). 2006; 162:95-7. is supportive of a immune mediated mechanism may be 11. Rodrigues ML, Rodrigues Mde L, Figueiredo JF, de Freitas JA. Ocular Problems in Brazilian Patients With AIDS Before and because of the cytokines released by the virus infected in Highly Active Antiretroviral Therapy (HAART) Era, The macrophages, one of which is the TNF-a, which is the main Brazilian J of infectious diseases 2007; 11:199-202.