Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory syndrome predominantly affecting the brainstem, cerebellum, and spinal cord. Following its initial description, the salient features of CLIPPERS have been confirmed and expanded upon, but the lack of formalized diagnostic criteria has led to reports of patients with dissimilar features purported to have CLIPPERS. We evaluated clinical, radiological and pathological features of patients referred for suspected CLIPPERS and propose diagnostic criteria to discriminate CLIPPERS from non-CLIPPERS aetiologies. Thirty-five patients were evaluated for suspected CLIPPERS. Clinical and neuroimaging data were reviewed by three neurologists to confirm CLIPPERS by consensus agreement. Neuroimaging and neuropathology were reviewed by experienced neuroradiologists and neuropathologists, respectively, both of whom were blinded to the clinical data. CLIPPERS was diagnosed in 23 patients (18 male and five female) and 12 patients had a non-CLIPPERS diagnosis. CLIPPERS patients’ median age of onset was 58 years (interquartile range, 24–72) and were followed a median of 44 months (interquartile range 38–63). Non-CLIPPERS patients’ median age of onset was 52 years (interquartile range, 39–59) and were followed a median of 27 months (interquartile range, 14–47). Clinical symptoms of gait ataxia, diplopia, cognitive impairment, and facial paraesthesia did not discriminate CLIPPERS from non-CLIPPERS. Marked clinical and radiological corticosteroid responsiveness was observed in CLIPPERS (23/23), and clinical worsening occurred in all 12 CLIPPERS cases when corticosteroids were discontinued. Corticosteroid responsiveness was common but not universal in non-CLIPPERS [clinical improvement (8/12); radiological improvement (2/12); clinical worsening on discontinuation (3/8)]. CLIPPERS patients had brainstem predominant perivascular gadolinium enhancing lesions on magnetic resonance imaging that were discriminated from non-CLIPPERS by: homogenous gadolinium enhancing nodules <3 mm in diameter without ring-enhancement or mass effect, and homogenous T₂ signal abnormality not significantly exceeding the T₁ enhancement. Brain neuropathology on 14 CLIPPERS cases demonstrated marked CD3-positive T-lymphocyte, mild B-lymphocyte and moderate macrophage infiltrates, with perivascular predominance as well as diffuse parenchymal infiltration (14/14), present in meninges, white and grey matter, associated with variable tissue destruction, astrogliosis and secondary myelin loss. Clinical, radiological and pathological feature define CLIPPERS syndrome and are differentiated from non-CLIPPERS aetiologies by neuroradiological and neuropathological findings.

中文翻译：

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诊断为类固醇对桥脑血管周围血管增强的慢性淋巴细胞性炎症的诊断标准（CLIPPERS）

对类固醇有反应的桥脑血管周围增强的慢性淋巴细胞性炎症（CLIPPERS）是一种中枢神经系统炎症综合症，主要影响脑干，小脑和脊髓。按照其最初的描述，CLIPPERS的显着特征已经得到确认并得到扩展，但是缺乏正式的诊断标准已导致报告称具有CLIPPERS的特征相异的患者。我们评估了被怀疑患有CLIPPERS的患者的临床，放射学和病理学特征，并提出了诊断标准，以将CLIPPERS与非CLIPPERS病因区分开。对35名患者进行了怀疑的CLIPPERS评估。三位神经科医生对临床和神经影像学数据进行了审查，以通过共识协议确认CLIPPERS。神经影像学和神经病理学分别由经验丰富的神经放射科医生和神经病理学家进行审查，他们都不了解临床数据。CLIPPERS诊断为23例患者（男性18例，女性5例），其中12例非CLIPPERS诊断。CLIPPERS患者的中位发病年龄为58岁（四分位间距为24-72），而中位发病年龄为44个月（四分位间距为38-63）。非CLIPPERS患者的中位发病年龄为52岁（四分位间距为39-59），随后为中位期27个月（四分位间距为14-47）。步态共济失调，复视，认知障碍和面部感觉异常的临床症状并未将CLIPPERS与非CLIPPERS区分开。在CLIPPERS中观察到明显的临床和放射皮质类固醇反应性（23/23），停用皮质类固醇激素后，所有12例CLIPPERS病例均发生临床恶化。在非CLIPPERS患者中，皮质类固醇反应性很常见，但并不普遍[临床改善（8/12）；放射学改善（2/12）; 停药后临床恶化（3/8）]。CLIPPERS患者在磁共振成像中具有脑干为主的血管周围peri增强病变，通过以下方式与非CLIPPERS区别：g直径均一的结节增强结节，直径<3 mm，无环增强或质量效应，T均一_2个信号异常没有明显超过T ₁增强。14例CLIPPERS病例的脑神经病理学表现为CD3阳性T淋巴细胞，轻度B淋巴细胞和中度巨噬细胞浸润，血管周围占优势，弥漫性实质浸润（14/14），表现为脑膜，白和灰质，与各种组织破坏，星形胶质增生和继发性髓磷脂丢失。临床，放射学和病理学特征定义了CLIPPERS综合征，并通过神经放射学和神经病理学发现与非CLIPPERS病因区分开。