酷似脱髓鞘病的原发性桥臂淋巴瘤一例

A case of primary lymphoma in bilateral middle cerebellar peduncles resembling demyelinating disease

原发性中枢神经系统淋巴瘤是一种罕见的局限于中枢神经系统的非霍奇金淋巴瘤，好发于深部脑白质、侧脑室周围及胼胝体，典型临床表现为认知功能下降和头痛。我们报告1例原发性桥臂淋巴瘤患者，其临床表现仅为头晕、走路不稳；早期磁共振成像不典型，呈双侧桥臂对称性长T1、长T2信号影，FLAIR高信号影，周围可见水肿带，增强扫描仅见边缘强化。激素冲击治疗后病灶消失、病情好转，后症状反复，酷似多发性硬化时间、空间多发的特征。结合既往自身免疫疾病病史及辅助检查示寡克隆IgG鞘内合成等均高度提示脱髓鞘疾病可能性大。分析了原发性桥臂淋巴瘤与多发性硬化的临床表现、磁共振成像特点、诊断标准、治疗与预后的差异，以提高临床上对此病的认识。

Primary central nervous system lymphoma （PCNSL） is a rare, extranodal form of non- Hodgkin lymphoma that is confined to the central nervous system. It mainly involves the deep brain white matter, the lateral ventricle and the corpus callosum. A decline in cognitive function and headache are the typical clinical manifestations of the disease. We report a patient with PCNSL in bilateral middle cerebellar peduncles, whose clinical manifestations were only dizziness and unstable walking. Brain MRI manifestations were not typical in early time, with symmetrical hypointensity lesions on T1 -weighted imaging, hyperintensity lesions on T2-weighted imaging, and edema zone around on FLAIR imaging. Enhanced scan showed marginal contrast enhancement, but no significant occupying effect. These changes were similar to the multiple characteristics of multiple sclerosis. The history of autoimmune diseases and cerebrospinal fluid examination highly indicated demyelinating disease. The differences of clinical manifestations, MRI characteristics, diagnosis, treatment and prognosis between PCNSL in bilateral middle cerebellar peduncles and multiple sclerosis were analyzed to improve the understanding of the disease in clinical practice.