Dept. of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
Dept. of Infectious Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
BMC Neurol. 2019 Nov 4;19(1):266. doi: 10.1186/s12883-019-1507-z.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder in the central nervous system (CNS) with distinct clinical, radiological, and pathological characteristics. The pathophysiology of CLIPPERS still remains unclear. Because a few cases about lymphoma mimicking the manifestations of CLIPPERS were reported and the prognosis of lymphoma is much worse, early identification of lymphoma is very important.
A 31-year-old woman was admitted with 3 months' history of diplopia, dizziness, gait ataxia, and right facial numbness. The diagnosis of CLIPPERS was established based on the finding of punctate enhancing lesions in the cerebellum, thalamus, pons, medulla, and midbrain region in magnetic resonance imaging (MRI), together with the favorable clinical and radiological responses to corticosteroids. However, she was diagnosed as peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) by the pulmonary nodular and the skin biopsy almost 10 years later, and she got complete remission within 1 year after chemotherapy.
We report the first case of CLIPPERS developing PTCL-NOS. This case proposes that when brain biopsy was difficult to achieve, biopsies in extra-cerebral lesions under the assisting examination of positron emission tomography-computed tomography (PET-CT) can be helpful in further identification.
伴有桥脑旁血管周围强化对类固醇有反应的慢性淋巴细胞性炎症（CLIPPERS）是一种中枢神经系统（CNS）的炎症性疾病，具有独特的临床、放射学和病理学特征。CLIPPERS 的病理生理学仍然不清楚。由于有少数关于淋巴瘤模仿 CLIPPERS 表现的病例报道，而且淋巴瘤的预后要差得多，因此早期识别淋巴瘤非常重要。
一名 31 岁女性因复视、头晕、步态共济失调和右侧面部麻木 3 个月而入院。基于磁共振成像（MRI）发现小脑、丘脑、桥脑、延髓和中脑区域有斑点状增强病变，以及皮质类固醇治疗的良好临床和放射学反应，诊断为 CLIPPERS。然而，近 10 年后，通过肺部结节和皮肤活检诊断为外周 T 细胞淋巴瘤，非特指型（PTCL-NOS），并且在化疗后 1 年内获得完全缓解。
我们报告了首例 CLIPPERS 发展为 PTCL-NOS 的病例。该病例提示，当难以进行脑活检时，在正电子发射断层扫描-计算机断层扫描（PET-CT）辅助检查下对脑外病变进行活检有助于进一步明确诊断。
