title: Horizontal eyeball akinesia as an initial manifestation of CLIPPERS: Case report and review of literature.
abstract: BACKGROUND
  Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic inflammatory disorder in the central nervous system (CNS), which is characterized by magnetic resonance imaging (MRI) appearance with punctate and curvilinear gadolinium enhancement "peppering" the pons. Lesions of CLIPPERS mainly involve the pons and the cerebellum. Adjacent structures such as the medulla and the midbrain may also be involved. It is proposed that CLIPPERS is an immune-mediated inflammatory condition characteristic of T-cell-predominant infiltrates and good responsiveness to corticosteroids.
  
  
  RESULTS
  We report a 46-year-old woman who presented with horizontal eyeball akinesia and gait ataxia with characteristic MRI features of CLIPPERS. The possible pathogenesis, clinical manifestations, imaging features, treatment, and prognosis of this peculiar disorder are summarized.
  
  
  CONCLUSIONS
  This report contributes to the clinical understanding of CLIPPERS which may present with horizontal eyeball akinesia as an initial manifestation. The characteristic presentation of a subacute cerebellar and brainstem syndrome and pepper-like gadolinium enhancement was confirmed in this report. Long-term immunosuppressive treatment seems to be mandatory to sustain improvement. Azathioprine alone may be capable of maintaining remission.
authors: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University Department of Neurosurgery, the Second Hospital of Jilin University, Jilin University, Changchun, China.
authors: Xiaohe
authors: X
authors: Hou
authors: Xiaoke
authors: X
authors: Wang
authors: Bo
authors: B
authors: Xie
authors: Weihong
authors: W
authors: Lin
authors: Jun
authors: J
authors: Liu
authors: Dihui
authors: D
authors: Ma
authors: Hong-Liang
authors: HL
authors: Zhang
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D002493:Central Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D003937:Diagnosis, Differential; D004244:Dizziness / Q000209:etiology; D005260:Female; D020233:Gait Disorders, Neurologic / Q000209:etiology; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D015835:Ocular Motility Disorders / Q000209:etiology; D011149:Pons / Q000000981:diagnostic imaging
chemical_list: D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine; D008775:Methylprednisolone