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[Severe PERM syndrom mimicking tetanus].

F Wallet1, A Didelot2, B Delannoy1

  • 1Service de réanimation médicale, hôpital de la Croix-Rousse, CHU de Lyon, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.

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|August 30, 2014
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Summary

A rare neurological disorder, glycine antibody-associated hyperekplexia, mimicked tetanus in a patient. Prompt immunosuppressive therapy led to dramatic improvement, highlighting the need for accurate diagnosis in critical care.

Keywords:
HyperekplexiaHyperekplexiePERM syndromeTetanusTétanos

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Area of Science:

  • Neurology
  • Immunology
  • Critical Care Medicine

Background:

  • Progressive paralysis can present diagnostic challenges in intensive care settings.
  • Tetanus is a critical differential diagnosis for severe neuromuscular rigidity and paralysis.

Observation:

  • A 55-year-old male presented with paralysis mimicking tetanus, requiring mechanical ventilation.
  • Classical tetanus treatment was ineffective, and the patient's condition remained life-threatening.

Findings:

  • The patient was diagnosed with glycine antibody-associated hyperekplexia (progressive encephalomyelitis with rigidity syndrome).
  • Treatment with immunosuppressive therapy, including plasmapheresis, cyclophosphamide, and high-dose corticosteroids, resulted in dramatic clinical improvement.

Implications:

  • This case underscores the importance of considering rare neurological syndromes that mimic common critical illnesses.
  • Accurate diagnosis and opposite treatment strategies are crucial for improving outcomes in patients with glycine antibody-associated hyperekplexia.
  • Intensivists must be aware of this rare condition to avoid misdiagnosis and ensure timely, appropriate intervention for prolonged survival.