title: Need for prolonged immunosupressive therapy in CLIPPERS--a case report.
abstract: BACKGROUND
  Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) was first described in 2010 by Pittock and colleagues. All reported patients presented with diplopia and gait ataxia and had similar typical MRI findings with punctuate gadolinium enhancement of the pons. Alternative diagnoses were excluded by means of laboratory, radiological and histological tests. All patients were successfully treated with steroids. We present a case in which the steroid therapy was switched to long term immunosuppressive therapy, leading to several severe side-effects, but sustained clinical improvement.
  
  
  METHODS
  A 63-year-old male presented with sub-acute diplopia and progressive gait ataxia. During admission his neurological condition worsened and he developed multiple cranial nerve deficits, paraparesis and urine retention. MRI-findings were remarkable with punctuate enhancement with gadolinium of the pons. Cerebrospinal fluid only showed elevated protein levels and all other additional investigations were normal. The probable diagnosis of CLIPPERS was made and intravenous corticosteroids were administered. This led to rapid clinical recovery and decreased enhancement on the MRI-scan. Long-term oral immunosuppressive therapy was started. One-and-a-half year later our patient has no recurrence of neurological symptoms, however due to the side effects of the immunosuppressive therapy he was readmitted several times.
  
  
  CONCLUSIONS
  CLIPPERS presents with distinctive clinical and MRI-findings and may be diagnosed after excluding other differential diagnoses. Patients are treated with corticosteroids with good clinical results. Since short term glucocorticoid treatment results into relapse of the disease, longer term immunosuppressive therapy appears to be mandatory for sustained improvement, although accompanied by severe side effects.
authors.affiliation: Department of Neurology, Sint Lucas Andreas Hospital, Jan Tooropstraat 164, 1061 AE Amsterdam, Netherlands. j.wijntjes@slaz.nl
authors.forename: Juerd
authors.initials: J
authors.lastname: Wijntjes
authors.forename: Ernest J
authors.initials: EJ
authors.lastname: Wouda
authors.forename: Carl E H
authors.initials: CE
authors.lastname: Siegert
authors.forename: Giorgos B
authors.initials: GB
authors.lastname: Karas
authors.forename: Annemarie M M
authors.initials: AM
authors.lastname: Vlaar
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002097:C-Reactive Protein / Q000378:metabolism; D002908:Chronic Disease; D004172:Diplopia / Q000209:etiology / Q000628:therapy; D020234:Gait Ataxia / Q000209:etiology / Q000628:therapy; D006801:Humans; D007154:Immune System Diseases / Q000150:complications / Q000628:therapy*; D007167:Immunotherapy / Q000379:methods*; D007249:Inflammation / Q000150:complications / Q000276:immunology* / Q000628:therapy*; D008214:Lymphocytes / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011149:Pons / Q000187:drug effects / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D002097:C-Reactive Protein; D011239:Prednisolone