Nystagmus only with fixation in the light: a rare central sign due to cerebellar malfunction

Sun Uk Lee, Hyo Jung Kim, Jeong Yoon Choi, Jae Hwan Choi, David S. Zee, Ji Soo Kim

Research output: Contribution to journalArticlepeer-review


Fixation nystagmus refers to the nystagmus that appears or markedly increases with fixation. While relatively common in infantile (congenital) nystagmus, acquired fixation nystagmus is unusual and has been ascribed to lesions involving the cerebellar nuclei or the fibers projecting from the cerebellum to the brainstem. We aimed to report the clinical features of patients with acquired fixation nystagmus and discuss possible mechanisms using a model simulation and diagnostic significance. We describe four patients with acquired fixation nystagmus that appears or markedly increases with visual fixation. All patients had lesions involving the cerebellum or dorsal medulla. All patients showed direction-changing gaze-evoked nystagmus, impaired smooth pursuit, and decreased vestibular responses on head-impulse tests. The clinical implication of fixation nystagmus is that it may occur in central lesions that impair both smooth pursuit and the vestibulo-ocular reflex (VOR) but without creating a spontaneous nystagmus in the dark. We develop a mathematical model that hypothesizes that fixation nystagmus reflects a central tone imbalance due to abnormal function in cerebellar circuits that normally optimize the interaction between visual following (pursuit) and VOR during attempted fixation. Patients with fixation nystagmus have central lesions involving the cerebellar circuits that are involved in visual–vestibular interactions and normally eliminate biases that cause a spontaneous nystagmus.

Original languageEnglish
Pages (from-to)3879-3890
Number of pages12
JournalJournal of Neurology
Issue number7
StatePublished - Jul 2022
Externally publishedYes


  • Cerebellum
  • Nystagmus
  • Smooth pursuit
  • Vertigo
  • Visual fixation


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