title: CLIPPERS Syndrome: An Entity to be Faced in Neurosurgery.
abstract: BACKGROUND
  Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder of the central nervous system; it has only recently been defined and to date has received only limited attention. Its cause is as yet unknown. The pathologic characteristics are infiltration of T lymphocytes into the perivascular spaces of the pons, responsiveness to immunotherapy, and gadolinium-enhancing punctiform lesions in the brainstem seen on magnetic resonance imaging (MRI).
  
  
  METHODS
  We report here on the clinical, MRI, and brain biopsy findings in a 68-year-old man who presented with dysphagia, numbness and paresthesia on the right side of his face, as well as progressive gait ataxia. Brain and spinal MRI showed lesions in the pons and in the cervical spinal cord. The pontine lesion became progressively larger extending to the middle cerebellar peduncle and a tumor was suspected. After repeated biopsy, the histopathologic diagnosis confirmed CLIPPERS.
  
  
  CONCLUSIONS
  CLIPPERS syndrome may become manifest with a progressive tumor-like pontine lesion. This report adds clinical and radiologic aspects to the limited number of CLIPPERS cases reported to date, and underlines the importance of considering CLIPPERS in the differential diagnosis of tumor-like pontine processes.
authors.affiliation: Department of Neurosurgery, Hannover Medical School, Hannover, Germany. Electronic address: esmaeilzadeh.majid@mh-hannover.de.
authors.forename: Majid
authors.initials: M
authors.lastname: Esmaeilzadeh
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover, Germany.
authors.forename: Özlem
authors.initials: Ö
authors.lastname: Yildiz
authors.affiliation: Department of Neurosurgery, Hannover Medical School, Hannover, Germany.
authors.forename: Josef M
authors.initials: JM
authors.lastname: Lang
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Wegner
authors.affiliation: Institute of Diagnostic and Therapeutic Neuroradiology, Hannover Medical School, Hannover, Germany.
authors.forename: Bernd
authors.initials: B
authors.lastname: Haubitz
authors.affiliation: Institute of Pathology, Neuropathology, Hannover Medical School, Hannover, Germany.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Feuerhake
authors.affiliation: Institute of Neuropathology, University of Göttingen, Göttingen, Germany.
authors.forename: Arne
authors.initials: A
authors.lastname: Wrede
authors.affiliation: Institute of Neuropathology, University of Göttingen, Göttingen, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Brück
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dengler
authors.affiliation: Department of Neurosurgery, Hannover Medical School, Hannover, Germany.
authors.forename: Joachim K
authors.initials: JK
authors.lastname: Krauss
mesh_terms: D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D017252:CD3 Complex; D002531:Cerebellum / Q000473:pathology; D004660:Encephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery*; D017809:Fatal Outcome; D006801:Humans; D007167:Immunotherapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D019635:Neurosurgical Procedures / Q000379:methods*; D010291:Paresis / Q000209:etiology; D011149:Pons / Q000473:pathology* / Q000601:surgery*; D013116:Spinal Cord / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use; D013577:Syndrome; D013601:T-Lymphocytes
chemical_list: D000893:Anti-Inflammatory Agents; D017252:CD3 Complex; D013256:Steroids
keywords: Biopsy; CLIPPERS; Lymphocytic inflammation; Pons; Steroids