Progressive Multifocal Leukoencephalopathy: A Rare Cause of Cerebellar Edema and Atypical Mass Effect

Abstract

Progressive multifocal leukoencephalopathy (PML) is an opportunistic demyelinating disease of the CNS caused by the JC papovavirus (JCV). Demyelination due to oligodendrocyte death leads to multifocal, asymmetric lesions. MRI is a valuable tool for detecting and differentiating PML from other neuropathies. Radiographically, PML classically presents as bilateral, subcortical white matter lesions with a lack of brain atrophy. As the disease progresses, lesions become larger and coalesce to become confluent. Minor edema and mass effect are infrequently described and the presence of significant mass effect suggests an alternative diagnosis. In our case, a patient demonstrated atypical marked infratentorial mass effect. Bilaterally, cerebellar lesions with associated mass effect were observed, as well as effacement of cerebellar folia and partial effacement of the fourth ventricle. The diagnosis of PML was confirmed with a biopsy of the right cerebellar lesion showing classic PML histology, with JCV DNA detection by polymerase chain reaction in the biopsy material.