Disclosures: Cristina Brea, MD: No financial relationships or conflicts of interest

Case Diagnosis: A 13-year-old female with extremity weakness suggestive of an inflammatory demyelinating polyneuropathy experienced a relapsing-remitting course.

Case Description: Workup included lumbar puncture revealing albuminocytologic dissociation and MRI demonstrating enhancement of cauda equina nerve roots, characteristic of Guillain-Barre syndrome (GBS). Partial functional recovery was appreciated after initial IVIG. However, she relapsed several times, each with worsening severity of presentation and less functional improvement. She was treated with IVIG, plasmapheresis, and finally Rituximab after her 4th admission.

Setting: Tertiary Care HospitalAssessment/

Results: Upon presentation, the patient demonstrated a grade 3 GBS Disability Scale score and recovered to a grade 1. On her first and second relapse, she worsened to grade 3 and recovered to grade 2 after treatment. Upon her third relapse, she presented as a grade 4 and recovered to grade 3.

Discussion: Treatment-Related Fluctuations (TRF) in GBS have been associated with IVIG and plasmapheresis. It is difficult to distinguish these fluctuations from early acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP). Typically, individuals who undergo a GBS course and later experience relapses are diagnosed with A-CIDP. Conversely, TRF is defined as improvement in the GBS disability scale of at least one grade after completion of immunotherapy followed by a worsening, of at least one grade, within the first 2 months. Based on this definition, this patient was diagnosed with TRF; however, these persistent symptoms may suggest A-CIDP.

Conclusion: Given the sparsity of evidence of TRF vs A-CIDP in the literature and clinical practice, the question remains whether these are unique diagnoses or if these relapsing-remitting symptoms represent the typical course of A-CIDP. As rehabilitation providers, our goal is to maximize function and independence. Thus, we must make this distinction promptly as intervention, expectations, and prognosis may differ. With hopes of obtaining long-term functional recovery, we need to understand more about these conditions to promote better outcomes.

Level of Evidence: Level V

« Back to AAPM&R Annual Assembly 2021

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/guillain-barre-syndrome-treatment-related-fluctuations-vs-acute-onset-chronic-inflammatory-demyelinating-polyneuropathy-a-case-report/