Abstract
Introduction
Nodoparanodopathies have recently emerged as a distinct group of acquired immune-mediated peripheral neuropathies, characterized by autoantibodies directed against the nodal and paranodal proteins of myelinated peripheral nerves, such as neurofascin-186 and neurofascin-155. Unlike Guillain–Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP), these disorders often demonstrate a limited response to conventional therapies. Therefore, early and accurate diagnosis is essential to enable the use of targeted treatment strategies.
Case Presentation
We report a 54-year-old male who presented with quadriparesis, sensory disturbances, bulbar symptoms, and respiratory compromise. Diagnostic evaluation included cerebrospinal fluid (CSF) analysis, nerve conduction studies (NCS), and a nodopathy antibody panel, which confirmed anti-neurofascin-140 antibody positivity.
Results
The patient was treated with methylprednisolone, plasmapheresis, intravenous immunoglobulin (IVIG), and rituximab, followed by maintenance cyclophosphamide. He required intubation, tracheostomy, and prolonged intensive care unit (ICU) management. With comprehensive nursing care, including airway management, nutritional support, ventilator-associated pneumonia (VAP) prevention, and psychological support, he was successfully decannulated at 5 months and discharged at 6 months, with marked neurological recovery.
Discussion
This case underscores the importance of distinguishing nodoparanodopathies from classic GBS due to their unique pathophysiology and differential response to immunotherapy. It also highlights the critical role of neurocritical care nursing in optimizing patient outcomes.
Keywords
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