title: IFN beta 1a as Glucocorticoids-Sparing Therapy in a Patient with CLIPPERS.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Hospital de Cabueñes, Gijón, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Rico
authors.affiliation: Department of Neurology, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Villafani
authors.affiliation: Department of Neurology, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Tuñón
authors.affiliation: Department of Neurology, Centrol Médico de Asturias, Oviedo, Spain.
authors.forename: Valentín
authors.initials: V
authors.lastname: Mateos
authors.affiliation: Department of Neurology, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: Pedro
authors.initials: P
authors.lastname: Oliva-Nacarino
mesh_terms: D000276:Adjuvants, Immunologic / Q000627:therapeutic use*; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D004679:Encephalomyelitis / Q000188:drug therapy*; D006801:Humans; D000068556:Interferon beta-1a / Q000627:therapeutic use*; D008206:Lymphatic Diseases / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D011149:Pons / Q000473:pathology; D011282:Pregnenediones / Q000008:administration & dosage
chemical_list: D000276:Adjuvants, Immunologic; D000893:Anti-Inflammatory Agents; D011282:Pregnenediones; C021988:deflazacort; D000068556:Interferon beta-1a
references.citation: Int MS J. 2005 Nov;12(3):96-108
references.pmid: 16417818
references.citation: Clin Exp Immunol. 2014 Mar;175(3):385-96
references.pmid: 24028073
references.citation: J Neurol. 2011 Dec;258(12):2140-6
references.pmid: 21556878
references.citation: J Neurol Neurosurg Psychiatry. 2012 Jan;83(1):15-22
references.pmid: 22056964
references.citation: J Manag Care Spec Pharm. 2015 Aug;21(8):650-60
references.pmid: 26233537
references.citation: Cochrane Database Syst Rev. 2013;6:CD003280
references.pmid: 23771584
references.citation: Brain. 2010 Sep;133(9):2626-34
references.pmid: 20639547
references.citation: PLoS One. 2014;9(6):e98192
references.pmid: 24887452
references.citation: Med Sci Monit. 2015;21:1512-9
references.pmid: 26009248
references.citation: Arch Neurol. 2012 Jul;69(7):847-55
references.pmid: 22777259