Introduction <p>Anti-IgLON5 disease is a rare autoimmune encephalopathy characterized by heterogeneous neurological symptoms and is often under-recognized in acute settings. IgLON5 is a member of the IgLON family of cell adhesion molecules, which play essential roles in neuronal development and the organization of neural circuits. Beyond their neurological functions, these molecules are also being investigated for their potential involvement in systemic conditions such as cancer, depression, and obesity.</p> Case Report <p>We report a unique and severe presentation of this disorder in a 61-year-old woman who arrived at the emergency department with new-onset refractory status epilepticus (NORSE), a manifestation not previously associated with Anti-IgLON5 disease. Her prolonged hospital course included mechanical ventilation, marked autonomic instability, and a 134-day stay in the intensive care unit. Diagnostic evaluation revealed strong anti-IgLON5 antibody positivity, leading to a diagnosis of autoimmune encephalitis. The patient was treated with immunotherapy, including intravenous immunoglobulin, immunosuppressant, and anti-seizure medications, resulting in substantial neurological and functional recovery.</p> Conclusion <p>This case expands the clinical spectrum of Anti-IgLON5 disease and emphasizes the importance for emergency physicians to consider autoimmune causes in unexplained encephalopathy or status epilepticus presentations. Early recognition and targeted treatment may significantly influence outcomes in such rare but treatable conditions.</p>

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A Comprehensive Approach to an Unusual Case Presentation of Anti-IgLON 5 Associated Status Epilepticus with Literature Review

  • Pranjali Batra,
  • Niraj Kumar Srivastava,
  • Sushma Srivastava,
  • Deepika Joshi,
  • Anand Kumar,
  • Janki Makani

摘要

Introduction

Anti-IgLON5 disease is a rare autoimmune encephalopathy characterized by heterogeneous neurological symptoms and is often under-recognized in acute settings. IgLON5 is a member of the IgLON family of cell adhesion molecules, which play essential roles in neuronal development and the organization of neural circuits. Beyond their neurological functions, these molecules are also being investigated for their potential involvement in systemic conditions such as cancer, depression, and obesity.

Case Report

We report a unique and severe presentation of this disorder in a 61-year-old woman who arrived at the emergency department with new-onset refractory status epilepticus (NORSE), a manifestation not previously associated with Anti-IgLON5 disease. Her prolonged hospital course included mechanical ventilation, marked autonomic instability, and a 134-day stay in the intensive care unit. Diagnostic evaluation revealed strong anti-IgLON5 antibody positivity, leading to a diagnosis of autoimmune encephalitis. The patient was treated with immunotherapy, including intravenous immunoglobulin, immunosuppressant, and anti-seizure medications, resulting in substantial neurological and functional recovery.

Conclusion

This case expands the clinical spectrum of Anti-IgLON5 disease and emphasizes the importance for emergency physicians to consider autoimmune causes in unexplained encephalopathy or status epilepticus presentations. Early recognition and targeted treatment may significantly influence outcomes in such rare but treatable conditions.