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Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies,
Z Simmons1, J J Wald, J W Albers
1Division of Neurology, Pennsylvania State University College of Medicine, Hershey Medical Center, Hershey 17033, USA.
Insights
Pediatric Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) presents similarly to adult forms but with faster onset and more severe symptoms. Children with CIDP respond well to immunomodulating therapy.
Area of Science:
- Neurology
- Pediatric Neurology
- Immunology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare neurological disorder.
- Understanding pediatric CIDP is crucial due to its rarity and distinct presentation.
Purpose of the Study:
- To review and compare the clinical features of idiopathic CIDP in children versus adults.
- To evaluate the initial response of pediatric CIDP to immunomodulating therapy.
Main Methods:
- Retrospective review of 15 children with idiopathic CIDP.
- Comparison of pediatric CIDP features with 69 adult idiopathic CIDP cases.
- Analysis of clinical presentation, cerebrospinal fluid protein levels, and electrodiagnostic findings.
Main Results:
- Children and adults with CIDP share similarities in antecedent events, weakness, reflex loss, and elevated CSF protein.
- Pediatric CIDP is characterized by a more precipitous onset, frequent gait abnormalities, and significant neurological dysfunction.
- Electrodiagnostic criteria for CIDP were not always met in children, with segmental nerve abnormalities observed.
Conclusions:
- Pediatric CIDP shares core features with adult CIDP but presents more acutely and severely.
- Children with CIDP demonstrate an excellent initial response to immunomodulating therapies.
- Further research into pediatric CIDP is warranted to refine diagnostic and treatment strategies.
Abstract:
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare in children. We reviewed features of 15 children with idiopathic CIDP, and compared these to 69 adults with idiopathic CIDP. Children demonstrated many similarities to adults: (1) Antecedent events were uncommon. (2) There was a high frequency of weakness and reflex loss, a relatively high frequency of sensory loss, and a low frequency of pain and cranial neuropathies. (3) Cerebrospinal fluid protein levels were usually elevated. (4) On electrodiagnostic testing, not all nerve segments were abnormal, and not all children satisfied electrodiagnostic criteria for CIDP. Children differed from adults with CIDP in several ways: (1) The onset of symptoms was usually more precipitous. (2) Gait abnormalities were a more frequent presenting symptom. (3) Children always presented with significant neurological dysfunction, and not with the minor symptoms initially seen in some adults. The initial response of children with CIDP to immunomodulating therapy was excellent.
