title: Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients.
abstract: BACKGROUND
  Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory disease.
  
  
  OBJECTIVE
  To describe the disease course of CLIPPERS.
  
  
  METHODS
  A nationwide study was implemented to collect clinical, magnetic resonance imaging, cerebrospinal fluid, and brain biopsy specimen characteristics of patients with CLIPPERS.
  
  
  METHODS
  Academic research.
  
  
  METHODS
  Twelve patients with CLIPPERS.
  
  
  METHODS
  The therapeutic management of CLIPPERS was evaluated.
  
  
  RESULTS
  Among 12 patients, 42 relapses were analyzed. Relapses lasted a mean duration of 2.5 months, manifested frequent cerebellar ataxia and diplopia, and were associated with a mean Expanded Disability Status Scale (EDSS) score of 4. Besides typical findings of CLIPPERS, magnetic resonance imaging showed brainstem mass effect in 5 patients, extensive myelitis in 3 patients, and closed ring enhancement in 1 patient. Inconstant oligoclonal bands were found on cerebrospinal fluid investigation in 4 patients, with an increased T-cell ratio of CD4 to CD8. Among 7 available brain biopsy specimens, staining was positive for perivascular CD4 T lymphocytes in 5 samples. Thirty-eight of 42 relapses were treated with pulse corticosteroid therapy, which led to improvement, with a mean residual EDSS score of 1.9 (range, 0-7). In 1 patient with untreated relapses, scores on the EDSS progressively increased to a score of 10 at death. Among 5 patients without long-term corticosteroid therapy, the mean annualized relapse rate was 0.5 (range, 0.25-2.8). Among 7 patients taking oral corticosteroids, no relapses occurred in those whose daily dose was 20 mg or higher. No progressive course of CLIPPERS was observed. Four patients with a final EDSS score of 4 or higher had experienced previous severe relapses (EDSS score, ≥5) and brainstem and spinal cord atrophy.
  
  
  CONCLUSIONS
  CLIPPERS is a relapsing-remitting disorder without progressive forms. Long-term disability is correlated with the severity of previous relapses. Further studies are needed to confirm that prolonged corticosteroid therapy prevents further relapses.
authors: Department of Neurology, Centre Hospitalier Universitaire, Nimes, France.
authors: Guillaume
authors: G
authors: Taieb
authors: Claire
authors: C
authors: Duflos
authors: Dimitri
authors: D
authors: Renard
authors: Bertrand
authors: B
authors: Audoin
authors: Elsa
authors: E
authors: Kaphan
authors: Jean
authors: J
authors: Pelletier
authors: Nadège
authors: N
authors: Limousin
authors: Christine
authors: C
authors: Tranchant
authors: Stephane
authors: S
authors: Kremer
authors: Jérome
authors: J
authors: de Sèze
authors: Romain
authors: R
authors: Lefaucheur
authors: David
authors: D
authors: Maltête
authors: David
authors: D
authors: Brassat
authors: Michel
authors: M
authors: Clanet
authors: Patrice
authors: P
authors: Desbordes
authors: Eric
authors: E
authors: Thouvenot
authors: Laurent
authors: L
authors: Magy
authors: Thierry
authors: T
authors: Vincent
authors: Jean-Luc
authors: JL
authors: Faillie
authors: Nicolas
authors: N
authors: de Champfleur
authors: Giovanni
authors: G
authors: Castelnovo
authors: Sandrine
authors: S
authors: Eimer
authors: Dominique Figarella
authors: DF
authors: Branger
authors: Emmanuelle
authors: E
authors: Uro-Coste
authors: Pierre
authors: P
authors: Labauge
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D002552:Cerebral Ventricles / Q000473:pathology*; D004185:Disability Evaluation; D004660:Encephalitis / Q000134:cerebrospinal fluid / Q000188:drug therapy* / Q000473:pathology*; D005260:Female; D005682:Gadolinium; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011149:Pons / Q000473:pathology*; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D015703:Antigens, CD; D007166:Immunosuppressive Agents; D005682:Gadolinium