Imaging study of lymphomatosis cerebri characterized by diffuse lesions of bilateral cerebral white matter / 中华神经科杂志

ABSTRACT

Objective:To investigate multimodality imaging characteristics and clinical features of lymphomatosis cerebri (LC) and reasons for misdiagnosis,with the goal of potentially facilitating an early and accurate diagnosis for this often-missed disease.Methods:Clinical data and cerebral multimodality imaging findings from 11 patients with LC proven basing on pathology in the Affiliated Hospital of Guizhou Medical University from November 30, 2011 to December 28, 2020 were retrospectively extracted, analyzed, and reviewed in combination with the literatures.Results:The common presenting symptoms with subacute onset included cognitive decline (8/11), gait disturbance (9/11), and behavioral disturbance (5/11). Test of cerebrospinal fluid showed that the number of cells and the level of protein increased (8/10), the sugar content (2/10) and chloride (4/10) decreased. The imaging manifestations of 11 patients with LC were diffuse lesions of bilateral cerebral white matter in the both deep and lobar lesion distribution, involving the cerebral cortex and subcortical white matter in eight cases (8/11), basal ganglia in seven cases (7/11), thalamus in five cases (5/11), cerebellum in six cases and brain stem in six cases (6/11). All 11 patients showed equal or slightly low-density shadows on CT plain scan and slightly longer T 1WI and T 2WI signals on magnetic resonance imaging. Six cases (6/11) had no obvious enhancement in the early stage, and five cases and six follow-up cases showed heterogenous spots, patches, nodules or clusters of distinct enhancement. Diffusion-weighted imaging showed non restricted diffusion in nine (9/11) cases initially diagnosed, and restricted diffusion in two cases (2/11) and nine follow-up cases, which were hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient maps. Five patients (5/5) presented a marked decrease in N-acetyl aspartic acid (NAA)/creatine (Cr) and increase in choline (Cho)/Cr on hydrogen proton magnetic resonance spectrum, including an increase in lipid/Cr in three cases. One case (1/3) showed no abnormal increase in lesion metabolism, and two cases (2/3) showed slightly increased uptake on positron emission tomography/CT. Conclusions:Diffuse bilateral cerebral lesions especially in deep and lobar region, without enhancement or with patchy enhancement, marked decrease in NAA/Cr and increase in Cho/Cr and Lip/Cr are suggestive of LC. Misdiagnosis may be mainly due to insufficient understanding and improper brain biopsy.