What is chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids?

• DOI: 10.14253/acn.2020.22.2.129
• Publication Date: 2021-06-16T06:37:56Z
• Authors: Byung Joon Kim; Kang Min Park
• Keywords: 0301 basic medicine; 03 medical and health sciences

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a treatable inflammatory disorder of the central nervous system (CNS), especially involving cerebellum, spinal cord, or brainstem.The typical presenting features CLIPPERS in brain magnetic resonance imaging (MRI) are multiple punctate and curvilinear gadolinium-enhanced lesions peppering pons. 1 Here we report patient gait disturbance dysarthria who was diagnosed probable CLIPPERS.A 57-year-old female visited our hospital complaints disturbance, dysarthria, diplopia 2-week history.She had previously been healthy no history medications.She presented ataxic tilting on both sides, but she other symptoms signs such as motor sensory focal deficits limbs.Normal findings were obtained all applied laboratory tests, including for autoimmune paraneoplastic antibodies her serum, oligoclonal bands, IgG index, viral markers, cytology cerebrospinal fluid, infection, vasculitis, demyelinating CNS disease.The nerve conduction tests (including blink reflex) MR angiography also normal.She underwent MRI gadolinium enhancement, which revealed patchy increased signal intensities pons, strong contrast (Fig. 1).She treated high-dose steroid pulse therapy (methylprednisone at 500 mg 5 days).Her improved significantly after this treatment, discharged prescribed oral prednisone.Follow-up performed 4 months, complete resolution enhancing abnormalities.With CLIPPERS, name already implies, clinical radiological rapidly by treatment intravenous methylprednisone.However, diagnosing challenging, primarily due lack gold-standard diagnosis method, secondarily because its pathogenesis poorly understood. 2 These reasons