Department of Neurology, Sint Lucas Andreas Hospital, Jan Tooropstraat 164, 1061 AE Amsterdam, Netherlands.
BMC Neurol. 2013 May 24;13:49. doi: 10.1186/1471-2377-13-49.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) was first described in 2010 by Pittock and colleagues. All reported patients presented with diplopia and gait ataxia and had similar typical MRI findings with punctuate gadolinium enhancement of the pons. Alternative diagnoses were excluded by means of laboratory, radiological and histological tests. All patients were successfully treated with steroids. We present a case in which the steroid therapy was switched to long term immunosuppressive therapy, leading to several severe side-effects, but sustained clinical improvement.
A 63-year-old male presented with sub-acute diplopia and progressive gait ataxia. During admission his neurological condition worsened and he developed multiple cranial nerve deficits, paraparesis and urine retention. MRI-findings were remarkable with punctuate enhancement with gadolinium of the pons. Cerebrospinal fluid only showed elevated protein levels and all other additional investigations were normal. The probable diagnosis of CLIPPERS was made and intravenous corticosteroids were administered. This led to rapid clinical recovery and decreased enhancement on the MRI-scan. Long-term oral immunosuppressive therapy was started. One-and-a-half year later our patient has no recurrence of neurological symptoms, however due to the side effects of the immunosuppressive therapy he was readmitted several times.
CLIPPERS presents with distinctive clinical and MRI-findings and may be diagnosed after excluding other differential diagnoses. Patients are treated with corticosteroids with good clinical results. Since short term glucocorticoid treatment results into relapse of the disease, longer term immunosuppressive therapy appears to be mandatory for sustained improvement, although accompanied by severe side effects.
慢性淋巴细胞性炎症伴脑桥血管周围强化对类固醇有反应（CLIPPERS）于 2010 年由 Pittock 及其同事首次描述。所有报告的患者均表现为复视和步态共济失调，并具有类似的典型 MRI 发现，表现为脑桥点状钆增强。通过实验室、影像学和组织学检查排除了其他诊断。所有患者均成功接受类固醇治疗。我们报告了一例患者，其类固醇治疗转换为长期免疫抑制治疗，导致多种严重副作用，但临床症状持续改善。
一名 63 岁男性表现为亚急性复视和进行性步态共济失调。住院期间，他的神经状况恶化，并出现多个颅神经缺陷、截瘫和尿潴留。MRI 发现显著，表现为脑桥点状强化。脑脊液仅显示蛋白水平升高，其他额外检查均正常。可能诊断为 CLIPPERS，并给予静脉内皮质类固醇治疗。这导致了快速的临床康复和 MRI 扫描上的强化减少。开始长期口服免疫抑制治疗。一年半后，患者无神经症状复发，但由于免疫抑制治疗的副作用，他多次入院。
CLIPPERS 具有独特的临床和 MRI 表现，可在排除其他鉴别诊断后进行诊断。患者接受皮质类固醇治疗，临床效果良好。由于短期糖皮质激素治疗会导致疾病复发，因此需要长期免疫抑制治疗以持续改善，尽管会伴有严重的副作用。
