Spectrum of Neurofascin-155 Autoimmune Nodopathy in a Pediatric Cohort: A Single Center Experience.
Allison Saunders, Margaret Martinez, Bennett Lavenstein et al.
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Case in brief
This single-center pediatric case series described four patients with anti-neurofascin-155 autoimmune nodopathy. All had distal weakness and sensory loss; three also had tremor and sensory ataxia. Three patients initially received intravenous immunoglobulin (IVIg): one continued IVIg, while two were treated with rituximab. The authors reported sustained functional gains with rituximab in three patients overall. They characterized the pediatric disease course as milder than that reported in adults and noted improvement with rituximab among patients with partial or poor IVIg response.
What made this case unusual
The pediatric presentation was described as milder than adult cohorts, despite features such as tremor and sensory ataxia; rituximab was associated with sustained functional gains in three patients.
Diagnostic / clinical pearl
Consider anti-NF155 autoimmune nodopathy in children with distal weakness and sensory loss, particularly when tremor or sensory ataxia is present and response to IVIg is partial or poor.
Why it matters
The series underscores the value of recognizing this distinct neuropathy in pediatric patients, in whom the clinical course may differ from adult cohorts.