Lewerenz Jan, Ding Xiao-Qi, Matschke J, Schnabel C, Emami P, von Borczyskowski D, Buchert R, Krieger T, de Wit M, Münchau A
Salk Institute for Biological Studies, 10100 North Torrey Pines Road, La Jolla, California, CA 92037, USA.
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.08.2008.0752. Epub 2009 Feb 2.
Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma (PCNSL). Clinically, the disease typically presents with a rapidly progressive dementia and unsteadiness of gait. Its presentation on cerebral MRI, which is characterised by diffuse leukoencephalopathy without contrast enhancement, often causes diagnostic confusion1 with suspected diagnoses ranging from Binswanger's disease to leukoencephalopathy or encephalomyelitis. Here we report a patient with subacute dementia and diffuse bilateral white matter changes in the cerebral hemispheres and additional involvement of the brainstem, basal ganglia and thalamus on MRI. Initially, she was considered to suffer from an autoimmune encephalitis, transiently responded to immunosuppression but then developed multiple solid appearing cerebral lymphomas.
脑淋巴瘤病(LC)是原发性中枢神经系统淋巴瘤(PCNSL)的一种罕见变异型。临床上,该疾病通常表现为快速进展的痴呆和步态不稳。其在脑部MRI上的表现以无强化的弥漫性白质脑病为特征,这常导致诊断混淆,疑似诊断范围从宾斯旺格病到白质脑病或脑脊髓炎。在此,我们报告一例患者,其患有亚急性痴呆,大脑半球出现双侧弥漫性白质改变,MRI显示脑干、基底神经节和丘脑也有额外受累。最初,她被认为患有自身免疫性脑炎,对免疫抑制治疗有短暂反应,但随后发展为多个实性脑淋巴瘤。