An Atypical Pediatric Presentation of a Chronic Polyradiculoneuropathy

Wes Speer1, Christopher Szewczyk2, Ryan Jacobson3

  • 1Psychiatry, Rush Medical College, Chicago, USA.

Cureus
|October 2, 2023
PubMed

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.