OPHTHALMOLOGIC MANIFESTATIONS OF ACQUIRED IMMUNE-DEFICIENCY SYNDROME-ASSOCIATED PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY

Purpose: Progressive multifocal leukoencephalopathy (PML) is increasin gly described as a late complication of the acquired immune deficiency syndrome (AIDS). The purpose of this study is to evaluate retrospecti vely the ophthalmologic, clinical, and investigational aspects of AIDS -associated PML. Methods: The authors evaluated ten patients in whom o phthalmologic manifestations developed in the course of AIDS-associate d PML. Findings at clinical examination and their progression over tim e, neuroimaging correlates, the results of pathologic investigation, a nd visual outcomes were reviewed. Results: Progressive multifocal leuk oencephalopathy was the AIDS-defining illness in six of ten patients. Homonymous visual field defects were the presenting symptom in three p atients and detected in six patients overall. Occipital blindness deve loped in one patient. Cerebellar signs and brain stem nuclear and supr anuclear palsies also were common. Confluent white matter lesions with increased intensity on T2-weighted magnetic resonance imaging were su pratentorial in seven patients and infratentorial in three patients. W ith incomplete data, the median survival time was 3 months from PML on set. Histopathologic confirmation of PML diagnosis was available for n ine of the ten patients. Conclusions: The development of progressive r etrochiasmal visual field defects, supranuclear and nuclear cranial ne rve palsies, or nystagmus ataxia in the relatively young patient shoul d 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 immunod eficiency virus associated. Human immunodeficiency virus encephalopath y, cerebral toxoplasmosis, lymphoma, and infarction need to be discrim inated. Effective therapy is required urgently for this devastating di sease.