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Published on: October 7, 2021
An Atypical Pediatric Presentation of a Chronic Polyradiculoneuropathy
Wes Speer1, Christopher Szewczyk2, Ryan Jacobson3
1Psychiatry, Rush Medical College, Chicago, USA.
Insights
This case study details a 15-year-old male with chronic inflammatory demyelinating polyneuropathy (CIDP) refractory to initial treatment. Aggressive therapy with IVIg and steroids led to full recovery, highlighting CIDP
Area of Science:
- Neurology
- Immunology
Background:
- Presents a complex case of chronic inflammatory demyelinating polyneuropathy (CIDP) in a 15-year-old male.
- Highlights diagnostic challenges in young patients, particularly when complicated by malnutrition and weight loss.
Observation:
- Initial presentation included progressive weakness and areflexia, with electrodiagnostic studies not meeting typical CIDP criteria.
- Cerebrospinal fluid analysis revealed albuminocytologic dissociation, supporting the diagnosis despite atypical electrophysiology.
- The patient demonstrated refractoriness to initial intravenous immunoglobulin (IVIg) therapy, experiencing a relapse with worsening symptoms.
Findings:
- A second treatment course involving IVIg and high-dose intravenous methylprednisolone, followed by oral corticosteroids, resulted in significant clinical improvement.
- The patient achieved full strength recovery and normalized reflexes within one month of transitioning to oral corticosteroids.
- MRI revealed increased cauda equina enhancement during relapse, suggesting active inflammation.
Implications:
- Underscores the variable clinical presentation, disease course, and treatment response of CIDP in pediatric populations.
- Emphasizes the importance of considering confounding factors like malnutrition in diagnosing and managing CIDP.
- Suggests that aggressive immunosuppressive therapy may be necessary for refractory cases of CIDP in adolescents.
Abstract:
Here, we present a case of a 15-year-old male with polyradiculoneuropathy, which was diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), who was refractory to initial treatment. The patient presented with a one-and-a-half-month history of decreased strength, most notable in the bilateral hip flexors and finger flexors/extensors, and areflexia. Electromyography and nerve conduction studies did not fulfill diagnostic criteria for a demyelinating polyneuropathy; however, the cerebrospinal fluid analysis demonstrated albuminocytologic dissociation and his physical exam was otherwise consistent with the diagnosis. He was treated with IV immunoglobulin (IVIg). He relapsed less than one month later with worsening weakness. Imaging revealed increased cauda equina enhancement when compared to the MRI from the previous admission, and labs were otherwise similar to the initial presentation. He was treated with a second course of IVIg in addition to high-dose IV methylprednisolone. Upon his second discharge, he was transitioned to oral corticosteroids, and at a follow-up visit one month later, he had fully regained his strength and demonstrated normal reflexes. This case highlights the variable nature of CIDP in its initial presentation, its course, and its response to treatment, particularly in young patients. Additionally, we would like to emphasize that this case of CIDP was in the context of chronic malnutrition and significant weight loss, which made the diagnostic picture more complex.
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