title: CLIPPERS syndrome: Case report in a Brazilian patient with a long term disease evolution.
abstract: BACKGROUND
  Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome) was first described in 2010. Since then, about 50 cases have been reported around the world, but none in Portuguese-speaking countries. We report a case of patient of the Neurological Rehabilitation ward of Sarah Hospital/Brasilia, with clinical and radiologic features compatible with CLIPPERS Syndrome. The diagnosis was made only after fourteen years of symptoms onset.
  
  
  METHODS
  49-year-old male, presenting with progressive pancerebellar syndrome followed by spastic paraplegia with neurogenic bladder and progressive worsening over 14 years. The radiological response to steroid was suggestive of CLIPPERS Syndrome, however it was not accompanied by significant clinical improvement.
  
  
  CONCLUSIONS
  This is the first described case in Brazil,and this pattern of progression suggests that CLIPPERS is a degenerative disease. Its relevance must be acknowledged for being an important differential diagnosis of multiple sclerosis and other demyelinating diseases. Early diagnosis may be critical to halt the progression and affect outcome of the disease.
authors.affiliation: Neurologist, Departament of Neurology, Sarah Network of Rehabilitation, Brasília, Brazil. Electronic address: patricia-marinho@uol.com.br.
authors.forename: Patricia Beatriz Christino
authors.initials: PB
authors.lastname: Marinho
authors.affiliation: Neurologist, Departament of Neurology, Sarah Network of Rehabilitation, Brasília, Brazil.
authors.forename: Vinícius Viana Abreu
authors.initials: VV
authors.lastname: Montanaro
authors.affiliation: Neurologist, Departament of Neurology, Sarah Network of Rehabilitation, Brasília, Brazil.
authors.forename: Maria Cristina Del Negro
authors.initials: MC
authors.lastname: Barroso Freitas
authors.affiliation: Neurologist, Neurology Postgraduate Program of Federal University of Rio de Janeiro State, Rio de Janeiro, Brazil.
authors.forename: Regina Maria Papais
authors.initials: RM
authors.lastname: Alvarenga
mesh_terms: D001921:Brain / Q000473:pathology; D001938:Brazil; D020278:Demyelinating Autoimmune Diseases, CNS / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013116:Spinal Cord / Q000473:pathology
keywords: CLIPPERS; Demyelinating; Inflammatory diseases.