Department of Neurology, Northern Health, 185 Cooper Street, Epping, Victoria, 3076, Australia.
Department of Radiology, Northern Health, Epping, Victoria, 3076, Australia.
BMC Neurol. 2021 Feb 18;21(1):80. doi: 10.1186/s12883-021-02110-1.
Chronic lymphocytic infiltration with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a neuro-inflammatory syndrome first described in 2010. It has a relationship with lymphoproliferative disorders that has not been fully elucidated. This case represents an unusual progression of CLIPPERS to Epstein-Barr Virus (EBV)-related lymphomatoid granulomatosis (LYG). The exact connection between CLIPPERS and LYG remains poorly understood.
We present a case of a 75-year-old man who was diagnosed with CLIPPERS with initial response to immunosuppression but later progressed to EBV-related LYG. EBV polymerase chain reaction (PCR) was detected in his cerebrospinal fluid (CSF), and repeat imaging revealed findings that were uncharacteristic for CLIPPERS; thereby prompting a brain biopsy which led to a diagnosis of EBV-related LYG. This case highlights the following learning points: 1) CLIPPERS cases are often part of a spectrum of lymphomatous disease, 2) CLIPPERS can be associated with EBV-related lymphoproliferative disorders such as LYG, and 3) EBV detection in CSF should prompt earlier consideration for brain biopsy in patients.
Our case highlights the difficulty in distinguishing CLIPPERS from other steroid-responsive conditions such as neoplastic and granulomatous diseases. Given the association of CLIPPERS with EBV-related LYG as demonstrated in this case, we recommend testing for EBV in CSF for all patients with suspected CLIPPERS. An early referral for brain biopsy and treatment with rituximab should be considered for patients with suspected CLIPPERS who test positive for EBV in their CSF.
伴有桥脑血管周围强化反应的慢性淋巴细胞浸润（CLIPPERS）是 2010 年首次描述的一种神经炎症综合征。它与淋系增生性疾病有关，但尚未完全阐明。本病例代表了 CLIPPERS 向 Epstein-Barr 病毒（EBV）相关的淋巴组织细胞样肉芽肿病（LYG）的不常见进展。CLIPPERS 和 LYG 之间的确切联系仍知之甚少。
我们介绍了一例 75 岁男性，最初诊断为 CLIPPERS，对免疫抑制有初始反应，但后来进展为 EBV 相关的 LYG。在他的脑脊液（CSF）中检测到 EBV 聚合酶链反应（PCR），重复成像显示的结果不符合 CLIPPERS 的特征；因此促使进行脑活检，导致 EBV 相关的 LYG 诊断。本病例强调了以下学习要点：1）CLIPPERS 病例通常是淋巴肿瘤性疾病谱的一部分，2）CLIPPERS 可与 EBV 相关的淋系增生性疾病相关，如 LYG，3）CSF 中 EBV 的检测应促使患者更早考虑进行脑活检。
我们的病例强调了区分 CLIPPERS 与其他类固醇反应性疾病（如肿瘤性和肉芽肿性疾病）的困难。鉴于本例中 CLIPPERS 与 EBV 相关的 LYG 相关，我们建议对所有疑似 CLIPPERS 的患者进行 CSF 中 EBV 检测。对于 CSF 中 EBV 检测阳性的疑似 CLIPPERS 患者，应考虑早期转介进行脑活检和利妥昔单抗治疗。
