Decoding the Invisible Attack: Confronting Autoimmune Nodopathy Beyond the Usual with Immunotherapy and Expert Nursing Care—A Neurocritical Care Case Report

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.

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Publication Details

Journal
Nursing & Midwifery Research Journal
Published
2025-11-27
DOI
https://doi.org/10.1177/0974150x251397129
Primary Topic
Peripheral Neuropathies and Disorders
Type
article
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article

Decoding the Invisible Attack: Confronting Autoimmune Nodopathy Beyond the Usual with Immunotherapy and Expert Nursing Care—A Neurocritical Care Case Report

Prem Prakash, Soumya Sundaram
Nursing & Midwifery Research Journal
Peripheral Neuropathies and Disorders
article

Decoding the Invisible Attack: Confronting Autoimmune Nodopathy Beyond the Usual with Immunotherapy and Expert Nursing Care—A Neurocritical Care Case Report

Prem Prakash, Soumya Sundaram
article en

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.

Nursing & Midwifery Research Journal
Sree Chitra Thirunal Institute for Medical Sciences and Technology (IN)
Openalex Percentile: Top 49%
Peripheral Neuropathies and Disorders
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