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Final Program


Final Program

Electrodiagnostic / Neuromuscular Medicine

1522 - Immune-Mediated Neuropathy in a 3-Year-Old: An Evolving Case

Thursday, February 19, 2026
5:00 PM - 6:30 PM AST
Case Diagnosis: Progressive immune-mediated neuropathy, with ongoing evaluation for atypical chronic inflammatory demyelinating polyneuropathy (CIDP) versus nodopathy

Case Description: A 3-year-old male presented with two months of progressive weakness in the upper and lower extremities following gastrointestinal illness. Initial evaluation raised concern for Guillain-Barré syndrome; however cerebrospinal fluid studies were inconsistent. Electromyography and nerve conduction studies (EMG/NCS) demonstrated a subacute-chronic, demyelinating sensorimotor polyneuropathy with secondary axonal loss. This was suggestive of CIDP. The patient received intravenous immunoglobulin (IVIG) with partial improvement but was discharged home due to inability to attend rehabilitation. One month later he returned with recurrent weakness. Repeat EMG showed a severe, active, asymmetric, subacute, predominantly motor axonal polyradicular neuropathy with more prominent denervation compared to the prior study. Leading considerations included atypical CIDP versus immune-mediated nodopathy. A nodopathy antibody panel is planned for his next IVIG appointment as well as outpatient workup with neurology.

Discussions: This case highlights the diagnostic complexity of pediatric immune-mediated neuropathies. The relapsing course, demyelinating features, and partial IVIG response initially suggested CIDP. However, asymmetric progression and axonal predominance on follow-up EMG were not consistent with classic CIDP. Immune-mediated nodopathies, in which antibodies target nodal or paranodal proteins, represent an emerging category that may better explain this presentation. Differentiating between CIDP and nodopathies is clinically important, as prognosis, therapeutic response, and long-term management strategies may differ. Recognition of nodopathies in pediatric populations remains limited, underscoring the importance of case reports to guide awareness and diagnosis.

Conclusions: Immune-mediated neuropathies in children may present with overlapping features of CIDP and nodopathies. Serial electrodiagnostic studies and antibody testing are critical to refining diagnosis and guiding therapy. This case emphasizes the need to consider nodopathies in pediatric patients with relapsing motor-predominant neuropathies.