Spectrum of Neurofascin-155 Autoimmune Nodopathy in a Pediatric Cohort: A Single Center Experience.

BACKGROUND: Anti-neurofascin-155 (anti-NF155) autoimmune nodopathy (AN) is a distinct form of acquired neuropathy that has been described in adults with clinical features including severe relapsing polyneuropathy, cranial neuropathy, tremor, sensory ataxia, and poor response to intravenous immunoglobulin (IVIg). Few case series have been described in pediatrics. METHODS: Retrospective case series describing four pediatric patients with anti-NF155 AN. RESULTS: Distal weakness and sensory loss was present in all patients, and tremor and sensory ataxia was present in three patients. Three patients were initially treated with IVIg; one remained on IVIg, while two received rituximab. Rituximab led to sustained functional gains in three patients. CONCLUSIONS: This series highlights key clinical findings in pediatric anti-NF155 AN, including milder disease course than in adult cohorts. Patients with partial or poor IVIg response improved with rituximab, highlighting the importance of early recognition of AN in pediatrics to reduce overall symptom burden.