Clinical Characteristics and Longitudinal Videonystagmographic Monitoring in an Adolescent with Vimentin Antibody–Positive Cerebellar Ataxia: A Case Report
摘要
Background. Recently, vimentin IgG has been identified as a novel astrocytic autoantibody in patients with inflammatory meningoencephalomyelitis with prominent cerebellar involvement. This antibody has been reported to be associated with a characteristic inflammatory clinical syndrome. However, the functional impact of vimentin IgG on vestibulocerebellar pathways—particularly specific oculomotor abnormalities and their dynamic evolution during immunotherapy—remains poorly understood. Case Description A 15-year-old girl developed gait instability and dysarthria following an upper respiratory infection. Neurological examination revealed gaze-evoked nystagmus, impaired smooth pursuit, saccadic hypometria, limb ataxia, and pyramidal signs. Cerebrospinal fluid (CSF) showed pleocytosis, intrathecal IgG synthesis, positive oligoclonal bands, and anti-vimentin IgG (titer: 1:32), with serum anti-vimentin IgG titer of > 1:320. Brain MRI demonstrated widening of the superior vermian sulci. She received repeated courses of intravenous immunoglobulin and corticosteroids. Serial videonystagmography (VNG) demonstrated progressive improvement in optokinetic gain, saccadic accuracy, and pursuit smoothness, corresponding to recovery of vestibulocerebellar function. SARA scores improved from 20 to 14.5 and then to 8.5–9. CSF inflammatory markers and antibody titers decreased over time, though oligoclonal bands persisted. Conclusion This case illustrates a clinical presentation of cerebellar ataxia with corticospinal tract signs and oculomotor abnormalities in the presence of vimentin IgG. CSF testing for vimentin-IgG may be considered in selected cases of inflammatory ataxia of unclear etiology, particularly when conventional autoimmune panels are negative. VNG provides a sensitive, objective, and pathway-specific assessment and enables dynamic monitoring of immunotherapy-related recovery in vimentin antibody–associated vestibulocerebellar dysfunction.