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Childhood chronic inflammatory demyelinating neuropathies: clinical course and long-term follow-up
Y Nevo1, A Pestronk, A J Kornberg
1Department of Neurology, Barnes Hospital, Washington University School of Medicine, St. Louis, MO, USA.
Insights
Chronic inflammatory demyelinating neuropathy (CIDP) in children is rare. This study found two CIDP patient groups: one with a good prognosis and quick recovery, and another with prolonged illness and lasting weakness.
Area of Science:
- Pediatric Neurology
- Rare Diseases
- Immunology
Background:
- Chronic inflammatory demyelinating neuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Childhood CIDP presents unique challenges in diagnosis and management.
- Understanding long-term outcomes is crucial for pediatric patients.
Purpose of the Study:
- To review the clinical features of childhood CIDP.
- To evaluate treatment responses and long-term prognosis.
- To identify distinct prognostic subgroups in pediatric CIDP.
Main Methods:
- Retrospective review of 13 children diagnosed with CIDP.
- Analysis of clinical presentation, preceding events, disease course, and treatment response.
- Long-term follow-up to assess outcomes and identify prognostic factors.
Main Results:
- Lower extremity weakness and gait difficulty were common presenting symptoms.
- Over half of patients had preceding infections or vaccinations.
- While all responded to steroids short-term, 77% had residual weakness at 6-year follow-up.
- Two subgroups emerged: one with rapid progression and favorable prognosis, another with slower progression and chronic morbidity.
Conclusions:
- Childhood CIDP may comprise distinct subgroups with differing prognoses.
- Rapidly progressive CIDP (<3 months) often leads to complete recovery.
- Slowly progressive CIDP (>3 months) is associated with prolonged treatment needs and long-term weakness.
Abstract:
Chronic inflammatory demyelinating neuropathy (CIDP) is a rare disease in childhood. We reviewed the clinical characteristics, response to therapy, and long-term prognosis in 13 children (1.5 to 16 years of age) diagnosed with CIDP at Washington University Medical Center, St. Louis, and the Royal Children's Hospital, Melbourne, Australia, between 1979 and 1994. The most common presenting symptom (in 11/13 [85%]) was lower extremity weakness associated with difficulty in walking. Preceding events within 1 months of onset, mostly intercurrent infections or vaccinations, occurred in seven children (54%). The disease was monophasic in three children (23%). One relapse occurred in four (30%) and multiple relapses in six (46%). All patients had at least short-term response to steroids. Three children (23%) recovered completely during the first year. Ten children (77%) had residual weakness after an average follow-up of 6 years. There seems to be two populations of children with CIDP. One subgroup, with a favorable prognosis, progressed to peak disability over less than 3 months; these children often have a monophasic course with complete resolution of symptoms and signs and withdrawal from all medications by 1 year after onset. A second subgroup progressed for 3 months or longer; these children all required substantial does of prednisone for prolonged periods and had considerable long-term morbidity with persistent weakness.