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Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
  1. Neil G Simon1,
  2. John D Parratt1,
  3. Michael H Barnett2,
  4. Michael E Buckland3,
  5. Ruta Gupta3,
  6. Michael W Hayes4,
  7. Lynette T Masters2,
  8. Stephen W Reddel4
  1. 1Northern Clinical School, University of Sydney, St Leonards, New South Wales, Australia
  2. 2Brain and Mind Research Institute, University of Sydney, Camperdown, New South Wales, Australia
  3. 3Department of Neuropathology, University of Sydney, Sydney, New South Wales, Australia
  4. 4Concord Clinical School, University of Sydney, Concord, New South Wales, Australia
  1. Correspondence to Professor S W Reddel, Concord Clinical School, C22-Concord Hospital, The University of Sydney, Concord, NSW, 2139, Australia. stephen.reddel{at}sydney.edu.au

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings.

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Footnotes

  • See Editorial commentary, p 2

  • Linked article 301626.

  • Competing interests None.

  • Patient consent Detail has been removed from these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.

  • Ethics approval This study was approved by University of Sydney Human Research Ethics Committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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