An 80-year-old man with a history of stroke presented with eyelid ptosis of three years’ duration. Magnetic resonance imaging (MRI) of the brain revealed findings (Figure 1) consistent with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). As limitations precluded anatomopathological examination and the imaging pattern was characteristic enough to support treatment without biopsy, the decision was made to prescribe long-term corticosteroids. At the two-year follow-up, MRI findings had regressed (Figure 2) and the patient had improved clinically, meeting criteria for treatment response11. 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(pt 8):182-183. https://doi.org/10.1093/brain/awq390
https://doi.org/10.1093/brain/awq390...
. Biopsy is indicated when symptoms and MRI findings are unresponsive to treatment22. Taieb G, Allou T, Labauge P Therapeutic approaches in CLIPPERS. Curr Treat Options Neurol. 2017 May;19(5):17. https://doi.org/10.1007/s11940-017-0455-4
https://doi.org/10.1007/s11940-017-0455-...
. The differential diagnosis should include CNS lymphoma, neurosyphilis, and vasculitides33. Taieb G, Duflos C, Renard D, Audoin B, Kaphan E, Pelletier J, et al. Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol. 2012 Jul;69(7):847-55. https://doi.org/10.1001/archneurol.2012.122
https://doi.org/10.1001/archneurol.2012....
,44. Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, et al. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol. 2014 Mar;175(3):385-96. https://doi.org/10.1111/cei.12204
https://doi.org/10.1111/cei.12204...
.
References
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1Taieb 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(pt 8):182-183. https://doi.org/10.1093/brain/awq390
» https://doi.org/10.1093/brain/awq390 -
2Taieb G, Allou T, Labauge P Therapeutic approaches in CLIPPERS. Curr Treat Options Neurol. 2017 May;19(5):17. https://doi.org/10.1007/s11940-017-0455-4
» https://doi.org/10.1007/s11940-017-0455-4 -
3Taieb G, Duflos C, Renard D, Audoin B, Kaphan E, Pelletier J, et al. Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol. 2012 Jul;69(7):847-55. https://doi.org/10.1001/archneurol.2012.122
» https://doi.org/10.1001/archneurol.2012.122 -
4Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, et al. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol. 2014 Mar;175(3):385-96. https://doi.org/10.1111/cei.12204
» https://doi.org/10.1111/cei.12204
Publication Dates
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Publication in this collection
10 Jan 2020 -
Date of issue
Dec 2019
History
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Received
23 Jan 2019 -
Reviewed
19 July 2019 -
Accepted
27 July 2019