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Isolated cortical blindness without simultaneous neurological involvement in progressive multifocal leukoencephalopathy in a patient with human immune deficiency virus infection

DOI: 10.1186/1869-5760-3-3

Keywords: Cortical blindness, Progressive multifocal leukoencephalopathy, HIV infection

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Abstract:

Young HIV-positive patient presented to us with sudden profound visual loss. On examination and further investigation, we have diagnosed cortical blindness without any other focal neurological deficit due to PML.Our case highlights the fact that PML needs to be suspected in patients with HIV, presenting with cortical blindness even without any other focal neurological defect.Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder of the central nervous system (CNS) found in immune-deficient patients. It is caused by John Cunningham virus, a human polyomavirus (DNA virus) belonging to Papovaviridae group [1]. PML was originally described in patients with chronic diseases associated with compromised immune response or with immunosuppressive drugs [2,3]. However, the disease has been most frequently documented in patients with acquired immunodeficiency syndrome (AIDS) [4,5]. Studies estimate that prior to effective antiretroviral therapy, as many as 5% of people with AIDS eventually developed PML [6]. Usually, the outcome of patients of PML is poor; the disease is most often rapidly fatal with an expected progression to death within 6 months of symptoms [6,7]. Demyelination of the CNS is a consequence of virus-induced killing of oligodendrocytes, although the exact mechanism of cell death is unknown.Characteristic presentations of PML are focal neurological deficits, generally without fever or headache. The progression of deficits leads to life-threatening disability and death over weeks to months. Neuroimaging plays a crucial role in the early diagnosis and longitudinal monitoring of functional integrity of the nervous system.Since the central visual pathways and brainstem may be affected, a variety of neuro-ophthalmic signs and symptoms may manifest like visual symptoms, nystagmus, and cranial nerve and supranuclear gaze palsies [8,9]. Visual symptoms alone are infrequent in PML. We report a patient who presented with cortical blindness and witho

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