A NOVEL autoimmune meningoencephalomyelitis associated with autoantibodies targeting the astrocytic intermediate filament protein vimentin has been identified in a recent case series. This discovery emerged from a study involving 14 patients who presented with relapsing neurological symptoms and specific cerebrospinal fluid (CSF) and MRI findings.
The study, conducted at a tertiary referral hospital between April 2021 and May 2024, initially focused on two female patients exhibiting unknown encephalomyelitis. Their CSF samples demonstrated astrocytic reactivity on rat tissue but tested negative for glial fibrillary acidic protein (GFAP) IgG. Single-cell RNA sequencing of CSF cells from these patients revealed that 46% of clonally expanded B cell clonotypes expressed IgG autoantibodies targeting vimentin. Subsequent screening identified 12 additional patients with similar clinical profiles.
The cohort, comprising 10 females and 4 males with a median age of 33 years, shared a unique clinical presentation characterized by:
Relapsing disease course
Prominent involvement of the cerebellum, brainstem, and corticospinal tract
Elevated CSF protein levels and cell counts
Intrathecal immunoglobulin synthesis
Bilateral symmetric lesions on the corticospinal tracts observed in MRI
Notably, 67% of patients who received first-line immunotherapy during their initial episode responded favorably. However, by the last follow-up, 79% of patients experienced significant disability, indicated by a modified Rankin Scale score of 3 or higher.
This study highlights the importance of considering vimentin autoantibodies in patients with undifferentiated meningoencephalomyelitis, especially those presenting with the described clinical and radiographic features. Further research is warranted to understand the pathogenic role of these autoantibodies and to develop targeted therapeutic strategies.
Reference: Wan D et al. Novel Meningoencephalomyelitis Associated With Vimentin IgG Autoantibodies. 2025. JAMA Neurol. doi:10.1001/jamaneurol.2024.4763.
Anaya Malik | AMJ
