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Contrast-enhancing progressive multifocal leukoencephalopathy: radiological and pathological correlations: case
H H Woo1, A R Rezai, E A Knopp
1Department of Neurosurgery, New York University Medical Center, New York, USA.
Objective And Importance:
Progressive multifocal leukoencephalopathy (PML), a demyelinating disease caused by the JC papovavirus, is an opportunistic infection afflicting patients with impaired cellular immunity. Although initially described in patients with hematological malignancies, PML has become associated with several other immunocompromised states, particularly human immunodeficiency virus (HIV) infection. There are numerous central nervous system manifestations in patients with acquired immunodeficiency syndrome. A major characteristic that distinguishes PML from other more common lesions, such as toxoplasmosis or non-Hodgkin's lymphoma, is the lack of contrast enhancement. We describe a case of PML that exhibits contrast enhancement, and we conclude that the diagnosis of PML must be considered in patients with HIV who have contrast-enhancing lesions.
Clinical Presentation:
A 40-year-old woman presented with progressive hemiparesis, blurred vision, and ataxia. Magnetic resonance imaging revealed a contrast-enhancing lesion involving the left middle cerebellar peduncle, causing mild compression of the fourth ventricle.
Intervention:
The patient underwent a stereotactic serial biopsy with the presumptive diagnosis of moderate- to high-grade glioma. Histological examination of the biopsy specimen revealed early PML. Subsequently, a test for HIV was obtained and the results were positive.
Conclusion:
We have reported another atypical radiographic characteristic of PML associated with HIV. We conclude that PML lesions can enhance after the administration of gadolinium. Therefore, the diagnosis of PML must be entertained in patients whose test results were positive for HIV with contrast-enhancing lesions and that a stereotactic serial biopsy may be necessary to provide a definitive diagnosis.
Insights
Progressive multifocal leukoencephalopathy (PML) can present with contrast enhancement in patients with human immunodeficiency virus (HIV). This atypical finding necessitates considering PML in HIV-positive individuals with enhancing brain lesions.
Area of Science:
- Neuroscience
- Infectious Diseases
- Radiology
Background:
- Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection caused by the JC papovavirus, typically affecting individuals with compromised cellular immunity.
- While often associated with hematological malignancies, PML is increasingly recognized in other immunocompromised states, notably human immunodeficiency virus (HIV) infection.
- Central nervous system (CNS) manifestations are common in acquired immunodeficiency syndrome (AIDS), with toxoplasmosis and lymphoma being frequent differentials.
Observation:
- A 40-year-old woman with HIV presented with hemiparesis, visual disturbances, and ataxia.
- Magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion in the left middle cerebellar peduncle, causing mild ventricular compression.
- A stereotactic serial biopsy, initially presumed to be glioma, histologically confirmed early PML.
Findings:
- PML lesions can exhibit contrast enhancement following gadolinium administration, an atypical radiographic characteristic.
- This contrasts with the typical presentation of PML, which usually lacks contrast enhancement.
- The presence of contrast enhancement in PML lesions associated with HIV has been documented.
Implications:
- The diagnosis of PML should be considered in HIV-positive patients presenting with contrast-enhancing CNS lesions.
- Atypical imaging findings in PML necessitate a broader differential diagnosis in immunocompromised patients.
- Stereotactic serial biopsy may be required for definitive diagnosis of PML when characteristic radiographic features are absent or atypical.

