TY - JOUR
T1 - Progressive multifocal leukoencephalopathy
T2 - Recent advances and a neuro-ophthalmological review
AU - Sudhakar, Padmaja
AU - Bachman, David M.
AU - Mark, Alexander S.
AU - Berger, Joseph R.
AU - Kedar, Sachin
N1 - Publisher Copyright:
© 2015 by North American Neuro-Ophthalmology Society.
PY - 2015/9/29
Y1 - 2015/9/29
N2 - Background: Progressive multifocal leukoencephalopathy (PML) is a severe often fatal opportunistic infection of the central nervous system caused by reactivation of a ubiquitous polyoma virus, JC virus. Although typically characterized by multifocal asymmetric subcortical white matter lesions, it may be monofocal and affect the cortical gray matter. Among the broad spectrum of clinical manifestations that occurs with PML, visual complaints are common. Evidence Acquisition: Combination of representative personally observed cases of PML and comprehensive review of case series of PML from 1958 through 2014. Results: Neuro-ophthalmic signs and symptoms were reported in approximately 20%-50% of patients with PML and can be the presenting manifestation in half of these. A majority of these presentations occur from damage to cerebral visual pathways resulting in visual field defects, cortical blindness, and other disorders of visual association. Given the decreased frequency of infratentorial and cerebellar involvement, ocular motility disorders are less common. Conclusions: Visual complaints occur in patients with PML and are often the presenting sign. Awareness of this condition is helpful in avoiding unnecessary delays in the diagnosis of PML and management of the underlying condition. Recent guidelines have established criteria for diagnosis of PML in the high-risk patient population and strategies to mitigate the risk in these populations.
AB - Background: Progressive multifocal leukoencephalopathy (PML) is a severe often fatal opportunistic infection of the central nervous system caused by reactivation of a ubiquitous polyoma virus, JC virus. Although typically characterized by multifocal asymmetric subcortical white matter lesions, it may be monofocal and affect the cortical gray matter. Among the broad spectrum of clinical manifestations that occurs with PML, visual complaints are common. Evidence Acquisition: Combination of representative personally observed cases of PML and comprehensive review of case series of PML from 1958 through 2014. Results: Neuro-ophthalmic signs and symptoms were reported in approximately 20%-50% of patients with PML and can be the presenting manifestation in half of these. A majority of these presentations occur from damage to cerebral visual pathways resulting in visual field defects, cortical blindness, and other disorders of visual association. Given the decreased frequency of infratentorial and cerebellar involvement, ocular motility disorders are less common. Conclusions: Visual complaints occur in patients with PML and are often the presenting sign. Awareness of this condition is helpful in avoiding unnecessary delays in the diagnosis of PML and management of the underlying condition. Recent guidelines have established criteria for diagnosis of PML in the high-risk patient population and strategies to mitigate the risk in these populations.
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U2 - 10.1097/WNO.0000000000000271
DO - 10.1097/WNO.0000000000000271
M3 - Review article
C2 - 26132966
AN - SCOPUS:84940478263
SN - 1070-8022
VL - 35
SP - 296
EP - 305
JO - Journal of Clinical Neuro-Ophthalmology
JF - Journal of Clinical Neuro-Ophthalmology
IS - 3
ER -