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Progressive Multifocal Leukoencephalopathy - Case 1

A 55-year-old right-handed man with no past medical history presented with a two week history of headache and progressive aphasia. He was found to be HIV positive, with HIV-1 RNA PCR showing 293,000 viral copies/ml.

Show the White Matter Lesions

Progressive Multifocal Leukoencephalopathy (PML): Flair axial MRIs. Note the prominent lesions affecting the white matter in the left frontal and parietal areas. Similar abnormalities are also seen in the splenium of the corpus callosum and the contralateral white matter. Also note the absence of any mass effect.

PML is a demyelinating disease that results from an opportunistic infection due to the JC virus (named after the initials of the patient in whom it was first discovered), and occurs in AIDS and other immunocompromised patients. It is recognized on imaging studies as a progressive, subacute process affecting the white matter without enhancement or mass effect. There is no known cure. Treatment is supportive and symptomatic.

This patient subsequently had a lumbar puncture which showed a normal cell count but with an elevated protein at 83 mg/dL. JC virus DNA was detected by PCR, and the patient was treated with HAART (highly active antiretroviral therapy).


Revised 11/29/06
Copyrighted 2006. David C Preston