A rare case of fulminant GBS mimicking brain death with clinical recovery

Tanmay Trivedi, Rajashekhar Reddi, Puneet Agarwal

Abstract

A 28-year-old male patient admitted with an acute onset areflexic quadriparesis requiring mechanical ventilation within 24 h of admission. The clinical findings of absent motor response and loss of brainstem reflexes mimicked a “brain death” pattern. MRI brain and spine were normal. Nerve conduction studies were suggestive of early acute inflammatory demyelinating polyneuropathy and cerebrospinal fluid (CSF) showed albumino-cytological dissociation. Electroencephalographic (EEG) showed 5–6 Hz generalized slowing. He was treated with 2 cycles of intravenous immunoglobulins (0.4 gm/kg) for 5 days starting on day 1 and 19. Extensive work up was done to rule out other causes of acute flaccid areflexic quadriparesis with multiple cranial nerve involvement. Five sessions of plasma exchange (50 mL/kg) were carried out between day 45 to 55. He showed gradual recovery after being in “locked in” state for almost 55 days. At 9 months post on-set the patient has regained bulbar function and has MRC grade 3/5 power in all four limbs. Other published cases with a similar admission pattern were reviewed. The prognosis is usually very poor as most of the patients died or remained severely disabled.