Childhood CIDP: Study of 31 patients and comparison between slow and rapid-onset groups.
OBJECTIVE
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.


METHODS
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.
Unité de neurologie de l'enfant et de l'adolescent, Hôpital Pellegrin-Enfants, CHU de Bordeaux, Place Amélie Raba-Léon, 33076 Bordeaux cedex, France. Electronic address: sebastiencabasson@yahoo.fr.
Sébastien
S
Cabasson
Service de neurologie Pédiatrique, Assistance Publique Hôpitaux de Paris, Université Paris Sud, Le Kremlin-Bicêtre, France. Electronic address: marc.tardieu@bct.aphp.fr.
Marc
M
Tardieu
Service de Neurologie Pédiatrique, CHU de Strasbourg, France. Electronic address: ari.meunier@gmail.com.
Ariane
A
Meunier
Unité de Neurophysiologie, CHU de Bordeaux, France. Electronic address: marie-france.rouanet@chu-bordeaux.fr.
Marie-France
MF
Rouanet-Larriviere
Unité de Neurophysiologie, Assistance Publique Hôpitaux de Marseille, France. Electronic address: christophe.boulay@ap-hm.fr.
Christophe
C
Boulay
Unité de neurologie de l'enfant et de l'adolescent, Hôpital Pellegrin-Enfants, CHU de Bordeaux, Place Amélie Raba-Léon, 33076 Bordeaux cedex, France. Electronic address: jean-michel.pedespan@chu-bordeaux.fr.
Jean-Michel
JM
Pedespan
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D001379:Azathioprine / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D007223:Infant; D008297:Male; D009431:Neural Conduction / Q000502:physiology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D001379:Azathioprine
Acquired neuropathy; Corticosteroids; Guillain–Barré; Inflammatory diseases; Intravenous immunoglobulins