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Progressive Multifocal Leukoencephalopathy: Recent Advances and a Neuro-Ophthalmological Review
Padmaja Sudhakar1, David M Bachman, Alexander S Mark
1Departments of Neurology (PS), University of Kentucky College of Medicine, Lexington, Kentucky; Departments of Ophthalmology (DMB), and Radiology (ASM), Washington Hospital Center, Washington, District of Columbia; Department of Neurology (JRB), Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; and Department of Neurological Sciences and Ophthalmology (SK), University of Nebraska Medical Center, Omaha, Nebraska.
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.
Insights
Visual complaints are common in progressive multifocal leukoencephalopathy (PML), often presenting the first sign. Early recognition of these neuro-ophthalmic symptoms aids in timely diagnosis and management of PML.
Area of Science:
- Neuroscience
- Infectious Diseases
- Ophthalmology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a severe, often fatal, opportunistic central nervous system infection.
- Caused by JC virus reactivation, PML typically presents with white matter lesions but can affect gray matter.
- Visual complaints are a frequent manifestation of PML.
Observation:
- Neuro-ophthalmic signs occurred in 20%-50% of PML patients, presenting initially in half of these cases.
- Cerebral visual pathway damage leads to visual field defects and cortical blindness.
- Ocular motility disorders are less common due to reduced infratentorial involvement.
Findings:
- Visual symptoms are a significant and often initial presentation of PML.
- Damage to visual pathways is the primary cause of visual complaints in PML.
- The frequency of visual complaints underscores the importance of neuro-ophthalmic evaluation in PML.
Implications:
- Increased awareness of visual symptoms in PML can expedite diagnosis and treatment.
- Prompt diagnosis of PML is crucial for managing the underlying condition and improving outcomes.
- Established diagnostic criteria and risk mitigation strategies are available for high-risk populations.
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