Ophthalmologic manifestations of acquired immune deficiency syndrome-associated progressive multifocal leukoencephalopathy

• Published in: Ophthalmology (Rochester, Minn.) Vol. 103; no. 6; pp. 899 - 906
• Main Authors: ORMEROD, L. D, RHODES, R. H, GROSS, S. A, CRANE, L. R, HOUCHIN, K. W
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier 01.06.1996
• Subjects: Adult; AIDS Dementia Complex - diagnosis; AIDS-Related Opportunistic Infections - diagnosis; AIDS/HIV; Biological and medical sciences; Brain - pathology; Cranial Nerve Diseases - diagnosis; Female; HIV Seropositivity - diagnosis; HIV-1; Humans; Immunodeficiencies; Immunodeficiencies. Immunoglobulinopathies; Immunopathology; Leukoencephalopathy, Progressive Multifocal - diagnosis; Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Nystagmus, Pathologic - diagnosis; Optic Chiasm - pathology; Optic Nerve Diseases - diagnosis; Retrospective Studies; Vision Disorders - diagnosis; Visual Fields; Human; Immunopathology; Nervous system diseases; Vision disorder; Visual field; Deficiency; AIDS; Hemopathy; Immune deficiency; Cerebral disorder; Progressive multifocal leukoencephalopathy; Infection; Eye; Eye disease; Visual field disease; Viral disease; Blindness; Complication
• ISSN: 0161-6420; 1549-4713
• DOI: 10.1016/S0161-6420(96)30589-7

Progressive multifocal leukoencephalopathy (PML) is increasingly described as a late complication of the acquired immune deficiency syndrome (AIDS). The purpose of this study is to evaluate retrospectively the ophthalmologic, clinical, and investigational aspects of AIDS-associated PML. The authors evaluated ten patients in whom ophthalmologic manifestations developed in the course of AIDS-associated PML. Findings at clinical examination and their progression over time, neuroimaging correlates, the results of pathologic investigation, and visual outcomes were reviewed. Progressive multifocal leukoencephalopathy was the AIDS-defining illness in six of ten patients. Homonymous visual field defects were the presenting symptom in three patients and detected in six patients overall. Occipital blindness developed in one patient. Cerebellar signs and brain stem nuclear and supranuclear palsies also were common. Confluent white matter lesions with increased intensity on T2-weighted magnetic resonance imaging were supratentorial in seven patients and infratentorial in three patients. With incomplete data, the median survival time was 3 months from PML onset. Histopathologic confirmation of PML diagnosis was available for nine of the ten patients. The development of progressive retrochiasmal visual field defects, supranuclear and nuclear cranial nerve palsies, or nystagmus ataxia in the relatively young patient should alert the ophthalmologist to the possibility of PML, particularly in the presence of long-tract central nervous system signs or dementia. Progressive multifocal leukoencephalopathy will often be human immunodeficiency virus associated. Human immunodeficiency virus encephalopathy, cerebral toxoplasmosis, lymphoma, and infarction need to be discriminated. Effective therapy is required urgently for this devastating disease.