Lymphomatosis cerebri: Multimodality imaging features and misdiagnosis analysis

Abstract

Lymphomatosis cerebri (LC) is a significant challenge in terms of its clinical diagnosis due to it being a rare disease. The purpose of the present study was to investigate the multimodality imaging characteristics, clinical features and reasons for misdiagnosis of LC with the goal of potentially facilitating early and accurate diagnosis of this frequently misdiagnosed disease. In the present study, clinical data and cerebral multimodality imaging findings from 11 patients with LC proven based on pathology were retrospectively analyzed and reviewed with consultation of the literature. The results indicated that the common symptoms included cognitive decline (8/11), gait disturbance (9/11) and behavioral abnormalities (5/11). Cerebrospinal fluid analysis indicated that the number of cells and level of protein increased (8/10). All patients had both deep and lobar lesion distribution of bilateral cerebral white matter with equal or slightly low-density shadows on CT plain scan and slightly longer signals on T1- and T2-weighted MRI. Most of the lesions (9/11) exhibited isointensity or slight hyperintensity on diffusion-weighted imaging and hyperintensity on apparent diffusion coefficient maps. In addition, five patients presented with a marked decrease in N-acetylaspartate/creatine (Cr) and increase in choline/Cr on 1H-magnetic resonance spectroscopy, including an increase in lipid/Cr in 3 cases. Of these, one case exhibited no increase in lesion metabolism and 2 cases had slightly increased uptake on positron emission tomography/CT. The present study indicated that the multimodality imaging findings of LC have certain distinct characteristics and prompt recognition of these features may significantly improve early diagnosis and patient prognosis. Misdiagnosis may be mainly due to insufficient understanding knowledge of the condition and improper brain biopsy.