Fulminant Guillain Barre Syndrome AMSAN Variant Mimicking Brain Death: A Lesson in Persistence

Abstract

Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis. It commonly presents as acute symmetric ascending lower motor neuron palsy which typically occurs about one to three weeks following either a gastrointestinal or upper respiratory tract infection. GBS is termed fulminant when associated with rapid deterioration with flaccid quadriparesis, absent brainstem reflexes, and ventilator requirement. Here, we report a case of a female in her twenties who presented with a history of weakness and sensory loss a few days after recovering from a diarrheal illness. She developed progressive ascending paralysis of her muscles within 24 hours and was intubated anticipating respiratory failure. Initial examination revealed dilated pupils and the presence of ocular movements, as well as bilateral facial nerve involvement.  Investigative findings were consistent with GBS and intravenous immunoglobulin (IVIg) treatment was promptly initiated. Despite this, the patient deteriorated further and lost brainstem reflexes and failed to demonstrate spontaneous breaths. EEG indicated a deep coma. We continued with supportive care and physiotherapy and, after approximately 10 days of no initial response, our patient remarkably regained muscle power and sensations. She was eventually weaned off ventilation and has since returned to full functional status. This case report highlights the importance of persistence in treatment despite atypical presentations and initial clinical deterioration. Further, this case of fulminant GBS, AMSAN variant, involving the short ciliary nerve and mimicking brain death is a rare entity in clinical literature.