For patient 6, one enhancing lesion localized in the cerebellum seems to be connected to a vein extending to the straight sinus.This supplementary material has been provided by the authors to give readers additional information about their work.Customize your JAMA Network experience by selecting one or more topics from the list below.Taieb G, Duflos C, Renard D, et al.

Neuroimaging Sempere AP, Mola S, Martin-Medina P, Bernabeu A, Khabbaz E, Lopez-Celada S. Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up [published online August 17, 2011]. For each patient, data were retrospectively analyzed since the onset of their disease. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). In 38 relapses treated with corticosteroids, progressive EDSS score worsening was seen until pulse corticosteroid therapy was initiated (At the end of the follow-up period, the mean EDSS score was 3.8 (range, 0-10), with only one asymptomatic patient (patient 11, with an EDSS score of 0). In some reported cases of CLIPPERS, effective immunosuppressive maintenance treatment to prevent relapses (after complete corticosteroid withdrawal) was described using methotrexate in 4 patients 1,4,6,8 and cyclophosphamide in 1 patient. The clinical and radiologic recovery more than 2 years prior to recognition and successful treatment of his malignancy is inconsistent with his presentation being due to paraneoplastic syndrome. Its cause is as yet unknown.

Autoimmune serological evaluations included antinuclear and anti–extractable nuclear antigen antibodies, rheumatoid factor, complement levels, cryoglobulinemia, antineutrophil cytoplasmic antibodies, lupus anticoagulant, anti–βBrain MR imaging (1.5 T) was performed, including T1-weighted, T2-weighted, fluid-attenuated inversion recovery, T2-weighted gradient-echo images, and diffusion-weighted images, as well as apparent diffusion coefficient, time-of-flight MR angiography, and gadolinium-enhanced T1-weighted imaging. Immunoglobulin not seems to be effective (Gabilondo et al., 2011). Results To avoid corticosteroid-related adverse effects, other immunosuppressive treatment may be proposed.© 2020 American Medical Association.
After repeated biopsy, the histopathologic diagnosis confirmed CLIPPERS.CLIPPERS syndrome may become manifest with a progressive tumor-like pontine lesion.

Neuropathological characteristics Monoclonal antibody and immunosuppressive medications Kastrup O, van de Nes J, Gasser T, Keyvani K. Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study. Symptoms of CLIPPERS include subacute onset of brainstem symptoms--most prominently, • CLIPPERS is a relapsing subacute brainstem syndrome presenting with symptoms such as ataxia, diplopia, • Neuropathological examination of affected regions shows a perivascular lymphocytic inflammation that may involve both white and grey matter, with a CD4+ T cell predominance.• No biomarker is available for CLIPPERS, and the differential diagnoses is broad, requiring a careful workup of alternative causes. Good places to start looking elsewhere on the web are Google Scholar and PubMed with search terms like "CLIPPERS syndrome" or "CLIPPERS brain".

This may have contributed … Summary: We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. This biopsy showed dense CD3+ T-cell infiltration of the white matter with strong perivascular predominance, consistent with CLIPPERS (see the next section). Twelve patients from 9 university multiple sclerosis centers were included to assess multiple sclerosis diagnosis. The diagnosis of CLIPPERS syndrome was supported by (1) postcontrast T1-weighted MRI with typical punctate, nodular, or curvilinear and occasionally more confluent gadolinium-enhancing lesions, (2) perivascular inflammation in the brainstem dominated by T cells, (3) clinical course with several steroid-responsive relapses, and (4) absence of other diseases.



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