IFN beta 1a as Glucocorticoids-Sparing Therapy in a Patient with CLIPPERS
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymph...
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Published in | The American journal of case reports Vol. 17; pp. 47 - 50 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
United States
International Scientific Literature, Inc
27.01.2016
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Subjects | |
Online Access | Get full text |
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Summary: | Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymphocytic perivascular infiltrate on pathological exam, and a dramatic response to and dependence on steroids therapy. Since its initial description in 2010, different glucocorticoid-sparing agents, mostly immunosuppressant drugs, have been used to minimize the dosage, but these therapies also carry the risk of important secondary effects. We present the first reported case of CLIPPERS treated with interferon beta 1a as add-on therapy.
A previously healthy 31-year-old man presented with gait ataxia and dysarthria. MRI showed pons-centered hyperintense patchy lesions on T2-weighted images. Additional tests ruled out other possible diagnoses and symptoms reversed with intravenous methylprednisolone. Over the years the patient presented with several episodes of deterioration each year, which were partly reversed with glucocorticoid therapy, but leaving him with growing sequelae. Four years after the initial event, treatment with interferon-beta-1a was initiated, achieving reduced frequency of the relapses to 1 every 4 years, which were no longer associated to increasing disability. This allowed reducing glucocorticoids to 30 mg of Deflazacort every other day.
Interferon beta-1a could be an alternative to corticosteroid-combined therapy in CLIPPERS and its more benign profile of secondary effects compared to immunosuppressants could make it an attractive choice. |
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Bibliography: | ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 Funds Collection Authors’ Contribution Data Interpretation Literature Search Data Collection Study Design Manuscript Preparation Conflict of interest: P Oliva-Nacarino has received speaker honoraria from Almirall, Biogen Idec, Genzyme, Novartis, Merck-Serono and Sanofi. He has served as a consultant or on the advisory board of Bayer Shering Healthcare, Biogen Idec, Merck Serono, and Genzyme. He has received research support from Almirall, Biogen Idec, Genzyme, Merck Serono, and Sanofi. J Villafani has received speaker honoraria from Almirall, Biogen Idec, Genzyme, Novartis, Merck-Serono, and Sanofi. He has served as a consultant or the advisory boards of UCB, Bayer Shering Healthcare, Biogen Idec, Merck Serono, and Genzyme. He has received research support from Almirall, Biogen Idec, Genzyme, Merck Serono, and Sanofi. A Tuñón has received speaker honoraria from Almirall, Biogen Idec, Genzyme, Novartis, Merck-Serono, and Sanofi Statistical Analysis |
ISSN: | 1941-5923 1941-5923 |
DOI: | 10.12659/AJCR.896102 |