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Very-Early-Onset Treatment-Refractory Pediatric Chronic Inflammatory Demyelinating Polyradiculoneuropathy Responsive
Hoda Y Tomoum1, Dalia M El-Mikkawy2, Rasha H El-Owaidy3
1Pediatric Neurology Unit, Children's Hospital, Ain Shams University, Cairo, Egypt.
Insights
Treatment-refractory very-early-onset chronic inflammatory demyelinating polyradiculoneuropathy in a young child responded well to a combination of pulsed methylprednisolone, plasma exchange, and rituximab therapy. This regimen offers a potential new approach for challenging pediatric cases.
Area of Science:
- Pediatric Neurology
- Immunology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable pediatric polyneuropathy.
- Treatment strategies for refractory very-early-onset CIDP (symptoms before age 4) are not well-defined.
Purpose of the Study:
- To report a successful treatment approach for a case of treatment-refractory, very-early-onset CIDP in a young child.
Main Methods:
- The patient, aged 30 months, initially received intravenous immunoglobulin and corticosteroid monotherapy without response.
- Treatment was switched to combined pulsed intravenous methylprednisolone and plasma exchange.
- Followed by induction and maintenance therapy with rituximab.
Main Results:
- The child showed a significant and sustained clinical improvement after the combined therapy.
- This regimen demonstrated efficacy in a treatment-refractory pediatric case.
Conclusions:
- Combined pulsed methylprednisolone, plasma exchange, and rituximab can be an effective treatment for refractory very-early-onset CIDP in children.
- This case highlights a potential therapeutic option for severe pediatric neuropathies.
Abstract:
Chronic inflammatory demyelinating polyradiculoneuropathy is a treatable pediatric polyneuropathy, with both a typical form and variants. The approach to treatment-refractory chronic inflammatory demyelinating polyradiculoneuropathy is not fully characterized, especially in children with very-early-onset chronic inflammatory demyelinating polyradiculoneuropathy, namely, those with symptom onset before 4 years of age. We herein report the case of a 30-month-old child with treatment-refractory, very-early-onset chronic inflammatory demyelinating polyradiculoneuropathy who failed to respond to initial intravenous immunoglobulin and then corticosteroid monotherapy. He was ultimately switched to combined pulsed intravenous methylprednisolone and plasma exchange, followed by induction and maintenance rituximab therapy. The patient exhibited a significant and sustained clinical response to this regimen.
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