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Chronic inflammatory demyelinating polyradiculoneuropathy in children: II. Long-term follow-up, with comparison to
Z Simmons1, J J Wald, J W Albers
1Division of Neurology, The Pennsylvania State University College of Medicine, Hershey Medical Center, 17033, USA.
Insights
Children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) experience relapsing courses but show excellent recovery. Long-term follow-up reveals a better prognosis for children compared to adults with this condition.
Area of Science:
- Neurology
- Pediatric Neurology
- Clinical Electrophysiology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Understanding long-term outcomes in pediatric CIDP is crucial for effective management.
Purpose of the Study:
- To report the long-term follow-up of children with idiopathic CIDP.
- To compare the clinical course and outcomes of pediatric CIDP with adult cases.
Main Methods:
- Longitudinal follow-up of 12 children with idiopathic CIDP.
- Comparison with a cohort of 62 adults with idiopathic CIDP.
- Analysis of clinical course, recovery patterns, treatment efficacy, and long-term prognosis.
Main Results:
- Pediatric CIDP courses were often more rapidly fluctuating and relapsing than in adults.
- Children demonstrated excellent recovery from episodes, superior to adults on average.
- Prednisone, plasma exchange, and IVIg were effective; treatment discontinuation was possible in relapsing pediatric cases.
- Ventilatory support was rarely needed in children, even with slowly evolving illness.
Conclusions:
- Idiopathic CIDP in children has an excellent long-term prognosis with superior recovery compared to adults.
- Relapsing disease is more common in children, but they generally tolerate treatments well and achieve better outcomes.
Abstract:
We previously reviewed the presentation, initial clinical course, and electrodiagnostic features of children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We now report the long-term follow-up of 12 children with idiopathic CIDP, and compare these to 62 adults with idiopathic CIDP. Children often had more rapidly fluctuating courses than adults. A relapsing course was significantly more common in children than in adults. The recovery of children from each episode of deterioration was usually excellent, and better, on average, than in adults. Ventilatory support was never required for children with slowly evolving illness; only 2 children with a precipitous onset clinically resembling Guillain-Barré syndrome required ventilatory support. Prednisone, plasma exchange, and intravenous immunoglobulin (IVIg) usually were effective in children. Multiple courses of IVIg could be given with continued efficacy. Treatment often could be discontinued in children with relapsing courses. The prognosis for children was excellent. Adults demonstrated a good, but more variable, outcome.
