• Guillaume Taieb, Claire Duflos, Dimitri Renard, Bertrand Audoin, Elsa Kaphan, Jean Pelletier, Nadège Limousin, Christine Tranchant, Stephane Kremer, Jérome de Sèze, Romain Lefaucheur, David Maltête, David Brassat, Michel Clanet, Patrice Desbordes, Eric Thouvenot, Laurent Magy, Thierry Vincent, Jean-Luc Faillie, Nicolas de Champfleur, Giovanni Castelnovo, Sandrine Eimer, Dominique Figarella Branger, Emmanuelle Uro-Coste, and Pierre Labauge.
• Department of Neurology, Centre Hospitalier Universitaire, Nimes, France.
• Arch. Neurol. 2012 Jul 1; 69 (7): 847-55.

BackgroundChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory disease.ObjectiveTo describe the disease course of CLIPPERS.DesignA nationwide study was implemented to collect clinical, magnetic resonance imaging, cerebrospinal fluid, and brain biopsy specimen characteristics of patients with CLIPPERS.SettingAcademic research.PatientsTwelve patients with CLIPPERS.Main Outcome MeasuresThe therapeutic management of CLIPPERS was evaluated.ResultsAmong 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.ConclusionsCLIPPERS 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.

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