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Antineurofascin IgG2-associated paediatric autoimmune nodopathy
Rachel E Harris1, Mark Atherton1, Johann Te Water Naude2
1Department of Paediatric Neurology, Sheffield Children's Hospital, Sheffield, UK.
This study identifies IgG2 antineurofascin antibodies in pediatric autoimmune nodopathy, a novel finding. These antibodies show varying responses to immunotherapy, suggesting potential for targeted treatment strategies.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Autoimmune nodopathy is a rare neurological disorder.
- Antineurofascin antibodies are implicated in some cases of autoimmune neuropathies.
Observation:
- This case series describes four pediatric patients with autoimmune nodopathy.
- Three patients presented with predominant IgG2 antineurofascin antibodies, a pattern distinct from adult cases.
Findings:
- Antibody subclasses varied, with IgG2 predominant in three cases and IgG1/IgG4 in one.
- Patients with IgG2 antibodies showed some response to intravenous immunoglobulin (IVIG), while the other patient responded to steroids.
Implications:
- The findings suggest a potential serological-phenotypic correlation in pediatric autoimmune nodopathy.
- Identifying IgG2 antineurofascin antibodies may aid in prognostication and guide therapeutic decisions in children.
- Further research is needed to clarify the clinical significance of IgG2 antineurofascin antibodies in childhood polyneuropathy.
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