Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review.

IF 0.9 Q4 CLINICAL NEUROLOGY
Nripesh Man Shrestha, Niranjan Acharya, Rabindra Desar
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引用次数: 0

Abstract

CLIPPERS is a rare, chronic inflammatory neurological syndrome affecting multiple regions of the brain including the brainstem, cerebellum, and spinal cord. More than 100 cases have been documented globally since its initial description in 2010. This article reports the first case of the CLIPPERS syndrome in Nepal. Clinical and radiological evidences of the patient lead to the diagnosis of this disease. Brain MRI reveals punctate and curvilinear gadolinium enhancement in the pons and cerebellum, which is diagnostic for the disease. Steroid therapy has been reported to be effective in treating CLIPPERS syndrome. Although its pathophysiology indicates an immune-mediated process, the etiology is yet unknown. The treatment and prognosis of this illness depend on an early and accurate diagnosis.

Abstract Image

Abstract Image

慢性淋巴细胞炎症伴桥桥血管周围增强对类固醇反应(CLIPPERS综合征):1例报告和文献复习。
CLIPPERS是一种罕见的慢性炎症性神经系统综合征,影响大脑的多个区域,包括脑干、小脑和脊髓。自2010年首次描述以来,全球已记录了100多例病例。本文报道尼泊尔首例CLIPPERS综合征病例。病人的临床和放射学证据导致本病的诊断。脑MRI显示脑桥和小脑呈点状和曲线状钆增强,可作为本病的诊断。据报道,类固醇治疗对CLIPPERS综合征有效。虽然其病理生理提示免疫介导的过程,但病因尚不清楚。这种疾病的治疗和预后取决于早期和准确的诊断。
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