Horizontal eyeball akinesia as an initial manifestation of CLIPPERS: Case report and review of literature

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. METHODS AND 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. CONCLUSION: 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.