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CASE REPORT
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in limited cutaneous sclerosis: a rare disease combination
  1. Sucharita Anand,
  2. Animesh Das and
  3. Surjyaprakash Shivnarayan Choudhury
  1. Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
  1. Correspondence to Dr Animesh Das, animeshdas05{at}gmail.com

Abstract

A 26-year-old patient of limited cutaneous sclerosis presented to us with insidious-onset posterior fossa symptoms of headache, vomiting, vertigo and gait imbalance, progressing over a period of 3 weeks. A diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids was made by combining the clinical features with radiological evidence showing punctate infiltration of the pons, brainstem and cerebellum. Relevant differentials in the form of neurosarcoid, infections, central nervous system (CNS) lymphoma and Neuro-Behcet’s disease were ruled out by history and investigations. The patient responded dramatically to steroid therapy, and had no neurological deficits after 18 months of follow-up. This case highlights the rare association of a not-so-common immunological disease with a rare neurological disease.

  • neurology (drugs and medicines)
  • brain stem/cerebellum
  • neuroimaging
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Footnotes

  • Contributors SA collected the data. AD designed the report. SSC supervised the followup.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

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

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