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graft failure.[2] However, the safety and efficacy of intraocular intervention in eyes that have undergone DSAEK is not well established. Post-DSAEK surgical interventions may be fraught with the risk of endothelial cell damage or donor graft detachment or subsequent endothelial rejection. Therefore, patients with visually significant cataract often undergo cataract surgery at the time of endothelial keratoplasty. [3] It may be deferred in eyes where the anterior chamber cannot be properly visualized. In eyes with crystalline lens or early nuclear sclerosis that have undergone only endothelial keratoplasty, cataract formation may be hastened due to surgical manipulation, air tamponade at the time of surgery and use of postoperative steroid medications.[4] It is likely that all the mentioned factors might have led to accelerated cataract progression in this case. The specific surgery-related issues to be considered in these eyes are: the site of internal incision in relation to the edge of the lamellar graft, turbulence during insertion of the phaco handpiece with irrigation, which can potentially lead to graft dislodgement, reduced working space in the anterior chamber, and endothelial cell loss during surgery. The main postoperative concern is to prevent graft rejection secondary to surgical intervention. The case described here had an uneventful intraoperative and postoperative course. Appropriate surgical measures were taken to protect the graft throughout surgery. Intraoperatively, no deformation or detachment of the donor graft was noticed. The graft remained compact and well apposed. There was no noticeable corneal edema in the postoperative period. An endothelial cell loss of 14.7% was noted at 3 months after DSAEK and an additional loss of 19.3% was noted from the time of cataract surgery to 3 months later. The overall cell loss at 6 months post-DSAEK was 31.2%, which is comparable to
Progressive multifocal leukoencephalopathy presenting as homonymous hemianopia in a patient with acquired immunodeficiency syndrome Amit Pandey, Karishma Bandivdekar, Suresh Ramchandani1, Sushama Ramchandani1 Access this article online Quick Response Code:
Website: www.ijo.in DOI: 10.4103/0301-4738.103804 PMID: ***
Residents in Ophthalmology, MGM Hospital, Kamothe, 1Private Practice at Nerul, Navi Mumbai, India Correspondence to: Dr. Suresh Ramchandani, Shivam Eye Foundation, Plot no 14, Sector 25, Nerul, Navi Mumbai – 400706, Maharashtra, India. E-mail:
[email protected] Manuscript received: 20.02.11; Revision accepted: 11.09.11
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the endothelial cell density changes reported after DSAEK in various series.[5,6] This case shows that subsequent intraocular surgery with appropriate precautions can be performed safely in postDSAEK eyes. However, the long-term effect on the endothelium and graft survival needs to be better understood.
References 1. Price MO, Price DA, Fairchild KM, Price FW Jr. Rate and risk factors for cataract formation and extraction after Descemet stripping endothelial keratoplasty. Br J Ophthalmol 2010;94:1468-71. 2. Nagra PK, Rapuano CJ, Laibson PL, Kunimoto DY, Kay M, Cohen EJ. Cataract extraction following penetrating keratoplasty. Cornea 2004; 23:377-9. 3. Covert DJ, Koenig SB. New triple procedure: Descemet’s stripping and automated endothelial keratoplasty combined with phacoemulsification and intraocular lens implantation. Ophthalmology 2007;114:1272-7. 4. Price MO, Price FW Jr. Cataract progression and treatment following posterior lamellar keratoplasty. J Cataract Refract Surg 2004;30:1310-5. 5. Terry MA, Chen ES, Shamie N, Friend DJ. Endothelial cell loss after Descemet’s stripping endothelial keratoplasty in a large prospective series. Ophthalmology 2008; 115:488-96. 6. Lee WB, Jacobs DS, Musch DC, Kaufman SC, Reinhart WJ, Shtein RM. Descemet’s stripping endothelial keratoplasty: Safety and outcomes: A report by the American Academy of Ophthalmology. Ophthalmology 2009;116:1818-30. Cite this article as: Chaurasia S, Ramappa M, Sangwan V. Cataract surgery after Descemet stripping endothelial keratoplasty. Indian J Ophthalmol 2012;60:572-4. Source of Support: Nil, Conflict of Interest: None
We present a case of a Human Immunodeficiency Virus (HIV) positive patient who was referred for retinal evaluation to rule out ophthalmic manifestations of Acquired Immunodeficiency Syndrome (AIDS). She complained of some disturbance in vision in both eyes. Fundus examination showed no abnormality. Perimetry, done to rule out optic nerve pathology, showed a left homonymous hemianopia. Magnetic Resonance Imaging (MRI) scan showed features of Progressive Multifocal Leukoencephalopathy (PML). She had no other neurological symptoms or signs. Key words: Acquired Immunodeficiency Syndrome, demy elination, homonymous hemianopia, perimetry, progressive multifocal leukoencephalopathy
Progressive multifocal leukoencephalopathy (PML), also known as progressive multifocal leukoencephalitis, is a rare and usually fatal viral disease[1] that is characterized by progressive damage or inflammation of the white matter of the brain at multiple locations. It occurs almost exclusively in people with severe immune deficiency, such as transplant patients on immunosuppressive medications, patients receiving certain kinds of chemotherapy, patients receiving natalizumab (Tysabri) [1] for multiple sclerosis, psoriasis patients on long-term efalizumab (Raptiva) or AIDS patients. It is caused by a virus, the James Canyon (JC) virus, which
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is normally present and kept under control by the immune system. Immunosuppressive drugs prevent the immune system from controlling the virus.
Case Report A 50-year-old lady was referred for retinal evaluation to rule out AIDS-related retinal or ocular disorders. She complained of some disturbance in vision in both eyes since 15 days. She was a known case of AIDS since 10 years on treatment with Tab.Viraday (combination of Tenofovir, Emtricitabine and Efavirine) 1 per day. Her CD4 count was 128 cells/mm. She had no other neurological symptoms. Her vision in both eyes was 20/40, N/6. Anterior segment examination revealed no abnormality. There were no signs of uveitis. There was no relative afferent papillary defect (RAPD). Her intraocular pressure was 12 mm Hg in both eyes. Fundus examination showed no abnormality. There was no cytomegalovirus (CMV) retinitis. Optic nerves appeared normal. A visual field examination using 30-2 program on Oculus – Center Field perimeter was performed to rule out lesions along the visual pathway, as clinical examination could not explain the visual loss or the symptoms. It showed good reliability in both eyes and a left homonymous hemianopia [Figs. 1 and 2]. Magnetic Resonance Imaging (MRI) of brain was done to determine the cause of homonymous hemianopia, such as infarct/bleed or space-occupying lesion. MRI scan showed irregular white matter hyperintensity in the right occipital lobe and a hyperintense lesion in the left frontoparietal region in the subcortical and periventricular white matter [Fig. 3]. It also showed irregular white matter hyperintensity and a large area of increased T2-weighted and decreased T1-weighted signal in the left frontal lobe posteriorly involving the subcortical white matter and extending into the centrum semiovale and corona radiata, without enhancement or mass effect. A radio diagnosis of PML was made. She was referred to a neurologist for evaluation .Her neurological evaluation showed no abnormality. A cerebrospinal fluid (CSF) tap was advised for Polymerase Chain Reaction (PCR) evaluation for JC virus. But the patient refused to undergo the same.
Figure 1: Left eye perimetry
Figure 2: Right eye perimetry
Discussion PML is a progressive fatal demyelinating disorder associated with oligodendroglial infection by the human papovavirus JC. It is seen as a complication of many immunocompromised states. [2] Occasional cases are described in pregnancy,[3] and some cases may have no identifiable evidence of immunosuppression. PML, first described by Astrom et al. (1958), manifests as progressive decline in neurologic function.[4] The causative agent, the JC virus, invades oligodendroglial cells, causing multiple foci of demyelination. Primary JC virus infection occurs in childhood and is asymptomatic. JC virus antibodies are detectable in approximately 50–70% of the adult population. After the primary infection, JC virus remains latent in kidneys and lymphoid organs. Up to 64% of healthy adults have shedding of JC virus in urine in the absence of any clinical symptoms, suggesting that asymptomatic active JC virus infection is common in immunocompetent persons. The presenting symptoms in a case of PML are focal
Figure 3: MRI showing lesions in right parieto-occipital lobe and elsewhere
deficits like mental deterioration, speech disturbance, ataxia, paralysis, hemiparesis, facial weakness, memory failure, and
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also ophthalmic symptoms like nystagmus, homonymous hemianopia and diplopia with cranial nerve palsy in one eye and cortical blindness in the other eye.[5] Previously reported cases are different from the one we are presenting here, as our patient had come with nonspecific visual complaints and no other neurological symptoms. Visual symptoms are common in PML, but not in isolation. However, PML should also be suspected in the differential diagnosis of apparently healthy patients with focal deficits, like our patient who had no other symptoms except some visual disturbances in both eyes which she could not categorize. The purpose of presenting this case was to highlight the fact that we, as ophthalmologists, may be the first to see patients with PML and we should have a high degree of suspicion in an appropriate clinical setting. This would help us to carry out necessary tests such as visual field examination and, if necessary, an MRI scan to arrive at a diagnosis of PML which is a potentially life-threatening disease but may be helped by highly active anti-retroviral therapy (HAART).[6] PML should be kept in mind along with other ocular manifestations, such as CMV retinitis, which are commoner. Diagnosis of PML may be difficult. Because a large percentage of the population is seropositive for JC virus, the presence of antibodies to JC is not of diagnostic value. CSF identification of JC virus by PCR is useful and studies suggest a sensitivity of 95% and specificity of 90–99%.[7] The MRI findings are characteristic in PML, with a high T2 signal and a low T1 signal which does not enhance with gadolinium and has no mass effect. Brain biopsy is the only definitive way to make the diagnosis. PML is uniformly fatal, and the survival rate is only a few months following the diagnosis.[2] There are recent reports of improved survival following aggressive HAART in HIV,[6] and specific JC antiviral treatment with interferon α or cidofovir favor early diagnosis. Hence, we should consider PML in all patients who present with progressive neurological deficits (such as
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visual field defects) which are usually, but not always multifocal. If JC virus is absent in the CSF, brain biopsy should be considered.
References 1. Kleinschmidt-DeMasters BK, Tyler KL. Progressive Multifocal Leukoencephalopathy Complicating Treatment with Natalizumab and Interferon Beta-1a for Multiple Sclerosis. N Engl J Med 2005;353:369-74. 2. Astrom KE, Mancall EL, Richardson EP. Progressive multi‐focal leukoencephalopathy: a hitherto unrecognised complication of chronic lymphatic leukaemia and Hodgkin’s disease. Brain 1958;81:93-111. 3. Rosas MJ, Simoes‐Ribeiro F, An SF, Sousa N. Progressive multi‐ focal leukoencephalopathy: unusual MRI findings and prolonged survival in a pregnant woman. Neurology 1999;52:657-9. 4. Oguz B, Oguz KK, Akpinar E, Cila A, Guven GS. A case of progressive multifocal leucoencephalopathy (PML): diffusionweighted MR imaging findings. Neuroanatomy 2003;2:9-12. 5. Wein F, Francis GS, Gans MS, Connolly WE, Burnier MN Jr. Neuro-ophthalmic findings in progressive multifocal leucoencephalopathy. Can J Ophthalmol 1998;33:270-5. 6. Clifford DB, Yiannoutsos C, Glicksman M, Simpson DM, Singer EJ, Piliero PJ, et al. HAART improves prognosis in HIV‐associated progressive multi‐focal leucoencephalopathy. Neurology 1999;52:623-5. 7. Cinque P, Vago L, Dahl H, Brytting M, Terreni MR, Fornara C, et al. Polymerase chain reaction on cerebrospinal fluid for diagnosis of virus‐associated opportunistic diseases of the central nervous system in HIV‐infected patients. AIDS 1996;10:951-8. Cite this article as: Pandey A, Bandivdekar K, Ramchandani S, Ramchandani S. Progressive multifocal leukoence-phalopathy presenting as homonymous hemianopia in a patient with acquired immunodeficiency syndrome. Indian J Ophthalmol 2012;60:574-6. Source of Support: Nil, Conflict of Interest: None declared.
