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Childhood CIDP: Study of 31 patients and comparison between slow and rapid-onset groups
Sébastien Cabasson1, Marc Tardieu2, Ariane Meunier3
1Unité de neurologie de l'enfant et de l'adolescent, Hôpital Pellegrin-Enfants, CHU de Bordeaux, Place Amélie Raba-Léon, 33076 Bordeaux cedex, France.
Insights
Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) presents differently based on onset speed. Rapid-onset CIDP often involves cranial nerves and sensory symptoms, while slow-onset CIDP may feature ataxia and progressive decline.
Area of Science:
- Pediatric Neurology
- Clinical Electrophysiology
- Autoimmune Disorders
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Understanding the distinct clinical presentations and progression of childhood CIDP is crucial for effective management.
Purpose of the Study:
- To characterize 31 children with CIDP.
- To compare rapid-onset CIDP (peak impairment < 8 weeks) with slow-onset CIDP.
Main Methods:
- Retrospective chart review of 31 pediatric CIDP patients (24 confirmed, 7 possible).
- Data included time to peak impairment, clinical features, CSF, NCS, nerve biopsy, treatments, and modified Rankin Scale outcomes.
- Comparison between rapid-onset and slow-onset CIDP groups.
Main Results:
- Rapid-onset CIDP (42%) more frequently showed cranial nerve and sensory symptoms, and relapsing patterns.
- Slow-onset CIDP predominantly presented with progressive decline and ataxia.
- Electrophysiological criteria were met in 87%; early axonal involvement was noted.
- Intravenous immunoglobulins and corticosteroids were common treatments, with good initial recovery in most cases.
Conclusions:
- Observed differences between rapid- and slow-onset CIDP warrant further investigation in larger cohorts.
- Current electrophysiological criteria may be restrictive; early axonal involvement needs consideration.
- Prospective studies are needed to determine optimal first- and second-line treatments for pediatric CIDP.
Objectives:
To describe 31 children presenting a CIDP; to compare patients with rapid-onset disease vs. patients with slow-onset disease, a rapid-onset disease being defined by a time to peak impairment of less than 8 weeks.
Study Design:
A retrospective chart review identified 31 patients completing criteria for childhood CIDP, with 24 "confirmed CIDP" and 7 "possible CIDP". Data collected were time to peak impairment, clinical presentation, cerebrospinal fluid analysis, nerve conduction study, nerve biopsy, treatments. Evaluation at the end of follow-up was reported according to modified Rankin scale.
Results:
Thirteen patients (42%) exhibited symptoms in less than 2 months with more often cranial nerve abnormalities (38% vs. 6%, p = 0.059), and sensitive symptoms (62% vs. 11%, p = 0.0057). They evolved predominantly in a relapsing way (69% vs. 22%, p = 0.0047). Length of the disease was also longer in the rapid-onset group (5.5 years vs. 3.83 years) but without statistical difference. The slow-onset group exhibited more frequently ataxia at onset (28% vs. 8%, p > 0.05), and evolved predominantly in a progressive manner (61% vs. 15%, p > 0.05). Outcome was similar and good in the two groups. At least 3 out of the 4 major electrophysiological criteria were positive for 27/31 children (87%). Axonal involvement could be present very early. Immunoglobulins were given in 29 cases and corticosteroids in 22. A partial or complete recovery 1 month after first treatment was reported in 30 cases. Among second-line treatments, only azathioprine seemed effective in two out of three intractable children.
Conclusions:
The differences noted between the two groups should be tested in wider populations. Electrophysiological criteria are restrictive and axonal involvement should be studied. Prospective trials are required to find out the best first and second line treatments.
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