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Case Reports
. 2015 Jul-Sep;18(3):345-7.
doi: 10.4103/0972-2327.152085.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature

Soma Madhan Reddy  1 , Rahul Lath  1 , Meenakshi Swain  2 , Alok Ranjan  1
Affiliations
Case Reports

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature

Soma Madhan Reddy et al. Ann Indian Acad Neurol. 2015 Jul-Sep.

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.

Keywords: Brainstem mass; CLIPPERS; pontine lesion.

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Conflict of interest statement

Conflicts of Interest: None declared.

Figures

Figure 1
Figure 1
(a): Axial T1-weighted MRI showing a bulky pons with an isointense lesion (b) The lesion was hyperintense on T2-weighted MRI (c) Contrast MRI with gadolinium showed multiple punctuate enhancing lesions giving the classic “peppering of pons” appearance
Figure 2
Figure 2
Histopathology (a) showing a marked lymphocytic infiltrate in the white matter with perivascular predominance (hematoxylin and eosin 40×). Immunohistochemistry showing the lymphocytic infiltrate was composed predominantly of T lymphocytes that were CD3 reactive (b) and few CD20-positive B lymphocytes (c). A few CD68-positive histiocytes were also seen (d)
Figure 3
Figure 3
Follow-up axial contrast MRI at 2 years showing complete resolution of the lesion
See this image and copyright information in PMC

References

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    1. Kastrup O, van de Nes J, Gasser T, Keyvani K. Three cases of CLIPPERS: A serial clinical, laboratory and MRI follow-up study. J Neurol. 2011;258:2140–6. - PubMed
    1. Taieb G, Wacongne A, Renard D, Figarella-Branger D, Castelnovo G, Labauge P. A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with initial normal magnetic resonance imaging. Brain. 2011;134:e182. - PubMed
    1. Jerca O, Manouchehrinia A, Tanasescu R. A new defined entity among the spectrum of central nervous system inflammation: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) Rom J Neurol. 2011;10:77–81.
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