White Matter Lesions in Panencephalopathic Type of Creutzfeldt-Jakob Disease: MR Imaging and Pathologic Correlations

BACKGROUND AND PURPOSE: A panencephalopathic type of Creutzfeldt-Jakob disease (pCJD) is characterized by the extensive involvement of the cerebral white matter as well as the cerebral gray matter. It has been a point of controversy, however, whether the white matter changes represent primary or secondary degeneration. The aim of this study was to elucidate, by using MR images and histologic examinations, whether the white matter lesions in pCJD are primary or secondary degeneration. METHODS: Serial changes of T2 hyperintensities and histologic findings of six autopsy-proved cases of pCJD were retrospectively analyzed. RESULTS: Serial MR images of brains affected by pCJD revealed that T2 hyperintensities appeared in the cerebral gray matter 2–5 months after onset and in the cerebral white matter around the lateral ventricles approximately 5 months after onset. They rapidly extended to deep and subcortical white matter during the next several months and then to the entire cerebral white matter 10 months after onset. Histologic examination of the white matter lesions revealed spongy changes or tissue rarefaction associated with gemistocytic astrocytosis, which indicates primary involvement of the white matter. At the terminal stages of cases with a longer clinical course, MR images showed T2 hyperintensities in the corticospinal tracts in the internal capsule and brain stem, which histologically disclosed loss of myelin and axons accompanied by fibrillary gliosis that indicates secondary degeneration. CONCLUSION: Cerebral white matter lesions in pCJD were considered to be primary changes of the disease, but the lesions of the corticospinal tracts were secondary to cortical or cerebral or both white matter lesions. Creutzfeldt-Jakob disease (CJD), the most common form of human prion disease, primarily affects the gray matter of the brain and occurs in four forms: sporadic, familial, iatrogenic, and a new variant. The sporadic form occurs most frequently. The histologic hallmarks of CJD are spongiform change, neuronal loss, and gemistocytic astrocytosis, as well as abnor-mal prion protein deposition in the gray matter. White matter lesions are rare in cases of CJD i