title: CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases.
abstract: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory CNS disorder characterized by 1) subacute onset of cerebellar and brainstem symptoms, 2) peripontine contrast-enhancing perivascular lesions with a "salt-and-pepper" appearance on MRI, and 3) angiocentric, predominantly T-lymphocytic infiltration as revealed by brain biopsy. Inflammatory diseases including neuroinfections, CNS lymphoma and neurosarcoidosis must be excluded. Since CLIPPERS was described in 2010, many patients might have been misdiagnosed in the past. We therefore searched medical records from a large tertiary neurological center, the Department of Neurology at Rigshospitalet, Copenhagen University Hospital, for patients discharged between 1999 and 2013 with a diagnosis of "sarcoidosis with other localization", "other acute disseminating demyelination", "other demyelinating disease in the CNS" or "encephalitis, myelitis or encephalomyelitis". Of 206 identified patients, 24 had been examined by brain biopsy and were included for further evaluation. Following clinical, neuroradiological and neuropathological review, 3 patients (12.5%) were reclassified as having CLIPPERS. Median long-term follow-up was 75 months. The present results suggest that clinical re-evaluation of patients previously diagnosed with unspecified inflammatory demyelinating CNS disease or atypical neurosarcoidosis may increase the detection rate of CLIPPERS. Further, potentially severe neurological deficits and progressive parenchymal atrophy on MRI may suggest neurodegenerative features, which emphasizes the need for early immunomodulatory treatment.
authors: Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Denmark; Department of Neurology, Herlev Hospital, Copenhagen University Hospital, Copenhagen, Denmark. Electronic address: bjorgmorell@gmail.com.
authors: B M
authors: BM
authors: Kerrn-Jespersen
authors: Department of Neurology, Herlev Hospital, Copenhagen University Hospital, Copenhagen, Denmark.
authors: M
authors: M
authors: Lindelof
authors: Department of Neurology, Odense University Hopital, Odense, Denmark.
authors: Zsolt
authors: Z
authors: Illes
authors: Department of Neurology, Odense University Hopital, Odense, Denmark.
authors: Morten
authors: M
authors: Blaabjerg
authors: Department of Pathology, Copenhagen University Hospital, Copenhagen, Denmark.
authors: E L
authors: EL
authors: Lund
authors: Department of Radiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
authors: C
authors: C
authors: Klausen
authors: Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Denmark.
authors: I
authors: I
authors: Christiansen
authors: Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Denmark.
authors: F
authors: F
authors: Sellebjerg
authors: Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Denmark.
authors: D
authors: D
authors: Kondziella
mesh_terms: D000328:Adult; D001921:Brain / Q000187:drug effects / Q000473:pathology; D017252:CD3 Complex / Q000378:metabolism; D002493:Central Nervous System Diseases / Q000150:complications* / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007249:Inflammation / Q000150:complications* / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013116:Spinal Cord / Q000187:drug effects; D013256:Steroids / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D017252:CD3 Complex; D013256:Steroids
keywords: CLIPPERS; Lymphocytic; Neuroinflammation; Perivascular; Pons; Steroids