A 35-year-old man develops hemiparesis, ataxia, homonymous hemianopia, and cognitive deterioration. An MRI of the brain demonstrates widespread areas of abnormal T2 signal in the white matter. An electroencephalogram is remarkable for diffuse slowing over both cerebral hemispheres. Brain biopsy reveals demyelination with abnormal giant oligodendrocytes, some of which contain eosinophilic inclusions. This patient's condition is most closely related to which of the following diseases?
The condition is progressive multifocal leukoencephalopathy, which is a rapidly progressive demyelinating disorder in which the JC virus (a papovavirus) infects oligodendroglial cells in the brain. The eosinophilic inclusions represent accumulations of JC virus. PML occurs in about 1% of AIDS patients, and is the AIDS-defining illness in half of the patients who develop the condition. There is no effective treatment for this disorder. Shingles and post-infectious encephalitis can follow chickenpox, but varicella is not associated specifically with demyelination. Measles can cause an encephalitis, and in some cases, subacute sclerosing panencephalitis (SSPE) may follow previous measles infection. These disorders would affect not only white matter, but gray matter as well. Also, SSPE usually occurs before the age of 18. Syphilis can cause meningitis, encephalitis, and spinal cord damage, but the disease process would not be limited to the white matter.
AIDS
Chickenpox
Measles
Syphilis