title: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
abstract: The classification and pathological mechanisms of many central nervous system inflammatory diseases remain uncertain. In this article we report eight patients with a clinically and radiologically distinct pontine-predominant encephalomyelitis we have named 'chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids' (CLIPPERS). The patients were assessed clinically, radiologically and pathologically at Mayo Clinic, USA and Ghent University Hospital, Belgium from 1999 to 2009. Median follow-up duration from clinical onset was 22 months (range 7-144 months). Patients underwent extensive laboratory (serum and cerebrospinal fluid), radiological and pathological testing (conjunctival, transbronchial and brain biopsies) to search for causes of an inflammatory central nervous system disorder. All eight patients (five female, three male) presented with episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids. All patients had symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord. Radiological improvement accompanied clinical response to glucocorticosteroids. Patients routinely worsened following glucocorticosteroid taper and required chronic glucocorticosteroid or other immunosuppressive therapy. Neuropathology of biopsy material from four patients demonstrated white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas, infection, lymphoma or vasculitis. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a definable, chronic inflammatory central nervous system disorder amenable to immunosuppressive treatment. The T cell predominant inflammatory pathology in affected central nervous system lesions and the clinical and radiological response to immunosuppressive therapies is consistent with an immune-mediated process.
authors.affiliation: Department of Neurology, Mayo Clinic College of Medicine, 200 First St. SW, Rochester, MN 55905, USA.
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Pittock
authors.forename: Jan
authors.initials: J
authors.lastname: Debruyne
authors.forename: Karl N
authors.initials: KN
authors.lastname: Krecke
authors.forename: Caterina
authors.initials: C
authors.lastname: Giannini
authors.forename: Jelle
authors.initials: J
authors.lastname: van den Ameele
authors.forename: Veerle
authors.initials: V
authors.lastname: De Herdt
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.forename: Robert D
authors.initials: RD
authors.lastname: Fealey
authors.forename: Brian G
authors.initials: BG
authors.lastname: Weinshenker
authors.forename: Allen J
authors.initials: AJ
authors.lastname: Aksamit
authors.forename: Bruce R
authors.initials: BR
authors.lastname: Krueger
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Shuster
authors.forename: B Mark
authors.initials: BM
authors.lastname: Keegan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002531:Cerebellum / Q000473:pathology; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004679:Encephalomyelitis / Q000134:cerebrospinal fluid / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008206:Lymphatic Diseases / Q000150:complications / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D008875:Middle Aged; D011149:Pons / Q000187:drug effects / Q000473:pathology*; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone