CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a recently described, rare neuroinflammatory disorder diagnosed by clinical symptoms involving the brain stem with a distinct pattern on neuroimaging and a perivascular T-lymphocyte infiltrate on brain biopsy. It is a condition usually described in adults in the fourth to fifth decade. We report a case of 13-year-old Indian boy who presented with recurrent episodes of ataxia and diplopia with onset at 7 years of age. He was investigated extensively to rule out infective, neoplastic, autoimmune, and demyelinating conditions over a span of 6 years. The diagnosis of CLIPPERS was entertained on the basis of clinico-radio-pathological correlation. Treatment with steroids and steroid-sparing agents, particularly methotrexate, seems to provide a promising outcome. With very few cases in literature so far, reporting of a larger case series with pediatric onset may expand it to CLIPPERS spectrum disorder.
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| http://dx.doi.org/10.1177/2329048X19831096 | DOI Listing |
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Clinics Hospital, Faculty of Medicine, University of Sao Paulo, São Paulo 05403-010, Brazil.
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View Article and Find Full Text PDFSpectrum of Autoimmune Diseases-Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)-Clinical Case.
Medicina (Kaunas)
March 2023
Department of Neurology, Pomeranian Medical University, 70-110 Szczecin, Poland.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a rare inflammatory disease of an undetermined aetiology. The condition is characterised by a range of clinical manifestations generally associated with damage to brainstem structures, the cerebellum, with characteristic magnetic resonance imaging (MRI) findings. The main feature is a good clinical and radiological response to glucocorticosteroid (GCS)-based immunosuppressive treatment.
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eNeurologicalSci
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