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VOLUME 1 , ISSUE 1 ( January-June, 2025 ) > List of Articles

ORIGINAL RESEARCH

A Spectrum of Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Children and Outcome: A Treatable Neurological Disorder: Three Cases from Single Center

Vykuntaraju K Gowda, Vidya S Naik, Sharath Babu, Aqila B Abdullah

Keywords : Abnormal reflexes, Chronic inflammatory demyelinating polyradiculoneuropathy, Intravenous immunoglobulin, Muscle hypotonia, Rituximab

Citation Information : Gowda VK, Naik VS, Babu S, Abdullah AB. A Spectrum of Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Children and Outcome: A Treatable Neurological Disorder: Three Cases from Single Center. J Acad Pediatr Neurol 2025; 1 (1):1-4.

DOI: 10.5005/jaopn-11028-0002

License: CC BY-NC 4.0

Published Online: 17-01-2025

Copyright Statement:  Copyright © 2025; The Author(s).


Abstract

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a treatable chronic disorder of the peripheral nervous system, with predominant motor involvement. The disease is characterized by insidious onset over several months or recurrent episodes. The pediatric population faces unique diagnostic challenges due to their rarity and therapeutic challenges due to lack of evidence, hence, we are reporting this series. Objective: To describe the clinical and laboratory characteristics and treatment outcomes of Pediatric CIDP, aiming to improve the insights for future management approaches. Results: This study presents three children diagnosed with CIDP based on their clinical presentation, nerve conduction studies (NCS), nerve biopsy findings, and criteria. Out of 3, two were male. The mean age of onset was 12.3 years (range 6–16 years). Two of the three cases exhibited gradual onset progressive weakness of limbs whereas one case had acute onset of quadriparesis mimicking Guillain-Barré syndrome. Two had cranial nerve involvement. All had abnormal NCS suggestive of demyelinating polyradiculoneuropathy. Nerve biopsy demonstrated evidence of inflammatory demyelination. All the cases were treated with pulse dosing of steroids and intravenous Immunoglobulin (IVIG) during the presentation, and subsequently managed with oral steroids. Steroid-sparing medications, rituximab and mycophenolate mofetil (MMF) were necessary for all of them for incomplete recovery and to prevent relapse, which led to a remarkably favorable response. Conclusion: This case series highlights the variable nature of CIDP in its initial presentation, its course, and the requirement of steroid-sparing agents to prevent relapses.


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