We report two cases of fulminant Acute Motor and Sensory Axonal Neuropathy (AMSAN), who both progressed rapidly to complete loss of brain stem reflexes, mimicking brain death. Both patients had comorbid uncontrolled diabetes mellitus (DM) type II and responded to intravenous immunoglobulins (IVIg). However, one patient had an atypical recovery course. 

AMSAN is an acute inflammatory polyradiculoneuropathy characterized by progressive symmetric, ascending flaccid paresis, areflexia and decreased sensation. Rarely, fulminant forms may present with acute onset tetraparesis and absent brainstem reflexes.

Case 1: A 55 year old male with past medical history (PMH) of uncontrolled DM, essential hypertension, dyslipidemia and diabetic neuropathy presented with ascending numbness and weakness of lower extremities.  At nadir on day 6, patient had respiratory failure and absence of brainstem reflexes. Two earlier lumbar punctures revealed pleocytosis of cerebrospinal fluid (CSF) until day 13, when patient’s CSF showed albuminocytologic dissociation for the first time. Patient steadily improved and was transferred to a long term care center. After ten days at the facility, he developed a urinary tract infection and aspiration pneumonia leading to septic shock, culminating in death on day 36.

Case 2: A 52 year old male with PMH of uncontrolled DM type II, tobacco use and obesity presented with progressive weakness, respiratory distress and hypophonia which began one day prior to admission. At nadir on day 4, patient had quadriparesis, respiratory failure, and loss of brain stem reflexes with minimal eye movements. From day 1, patient’s CSF showed albuminocytologic dissociation. Patient steadily improved and was transferred to floor.

We present two cases of fulminant AMSAN. Unique features of these cases include uncontrolled DM, presentation of clinical brain death despite cortical activity per EEG, timeline of albuminocytologic dissociation within CSF, possible etiology of GBS, and slow atypical recovery course.