a 32-year - old man was transferred to an intensive care unit ( icu ) due to respiration difficulties from a hepatology ward where he had been admitted after a 7-day history of symptoms of acute viral hepatitis a ( ha ) and a 3-day history of progressive distal and proximal weakness of limbs with additional facial weakness . initial laboratory tests performed at the ward ( results listed in table 1 ) suggested acute viral ha infection with high igm anti - ha antibody titers . despite improvement in liver function and hepatitis symptoms , nerve conduction was studied and cerebrospinal fluid ( csf ) was tapped due to progressive weakness . a diagnosis of guillain - barr syndrome ( gbs ) was supported by albuminocytologic dissociation ( 1 white blood cell / mm , 2 red blood cells / mm , 115.0 mg / dl protein , and 58 mg / dl glucose ) and multiple motornerve - conduction defects with decreased compound motor action potentials ( table 2 ) . on the second day in the icu his heart rate was 85~120 beats / min and his arterial blood pressure was up to 200/110 mmhg . his pupils were dilated to 5 mm and not reactive to light . on day 4 a positive pupil response to a 0.1%-pilocarpine test indicated ciliary postganglionic parasympathetic neuropathy with supersensitivity to acetylcholine . these tests and signs indicated the absence of all brain reflexes , suggesting the presence of peripheral deafferentation . in addition , complementary laboratory tests for vasculitis or other infectious agents were normal except for the presence of latent herpes simplex virus ( hsv ) infection . a polymerase chain reaction for hsv was negative , and no symptoms of hsv infection were detected . a follow - up nerve - conduction study performed 2 weeks after transfer to the icu suggested severe demyelinating sensorimotor polyneuropathy ( table 2 ) . intravenous gammaglobulin ( ivig ) ( 0.4 g / kg / day ) was administered for 5 days , but his neurological status had worsened due to fulminant deafferentation . therefore , pulse therapy with methylprednisolone ( 500 mg / day ) were given for 3 days . after the initial 5-day course of therapy , ivig was added twice weekly 2 weeks later . three months later he recovered complete power in his upper and lower limbs , except for a residual deficit of left foot weakness ( dorsiflexor : grade 4 , plantar flexor : grade 4 ) and bilateral hypopathic sensory change of soles . the development of clinical symptoms of hepatitis , marked increases in bilirubin , aspartate aminotrasferase , alanine aminotrasferase ( alt ) , and -glutamyltransferase levels , and a positive igm - ha virus antibody test supported the diagnosis of acute ha . cases exhibiting an association between gbs and ha are extremely rare.1 the reported clinical features of nine reviewed cases of gbs following ha were as follows : ( 1 ) a uniformly good outcome of the neuropathic symptoms , independent of the level of alt , which corresponds to the severity of liver dysfunction ; ( 2 ) highest occurrence in men ; and ( 3 ) the interval between the onset of the hepatitis and the development of neuropathic symptoms is less than 14 days.2 the hsv infection in our patient , in spite of neither symptoms nor signs , might have aggravated the severity of gbs . the initial nerve - conduction study and blink - reflex test in this patient revealed inexcitability of most nerves , which was due to distal pathology of the motor axons : either a distal conduction block or axonal degeneration . the nature of this pathology can not be predicted by the results of an initial electrophysiological evaluation.3 the very prolonged distal motor latencies in the electromyogram recorded in a subsequent nerve - conduction study suggested the presence of severe demyelinating polyneuropathy and axonopathy . in the case of gbs mimicking cerebral death,4 a sural nerve biopsy indicated that demyelination was the early pathological mechanism . therefore , the exact physiopathology and whether our patient had distal demyelination and conduction block with secondary axonal loss or axonal degeneration , or both , remained unclear . the various treatments used for fulminant gbs mimicking cerebral death are plasma exchange , ivig , or plasma exchange and corticosteroids.4 our patient was treated with ivig to modulate the immunologic reaction , and with high - dose pulse therapy with methylprednisolone for suppressing the acute severe inflammation in the peripheral nervous system . there is no evidence that conventional doses of corticosteroids ( around 60 mg of prednisone daily ) are effective in shortening the course of or reducing residual deficits in acute gbs.5 however , high - dose steroids are perhaps currently best applied to patients who can not tolerate plasma exchange ( e.g. , due to severe cardiovascular dysautonomia ) or when other treatments are unavailable.6 - 8 our patient recovered progressively , and could walk alone with mild weakness of the left foot at 3 months after admission . gbs mimicking brain death usually has a poor recovery rate and a high mortality , particularly in relation to dysautonomia.9 the timely application of combination pulse therapy with both ivig and methylprednisolone might improve the prognosis of fulminant gbs . in summary , in rare cases gbs presents with signs of coma and absent brainstem reflexes .
a 32-year - old man was transferred to an intensive care unit due to respiratory difficulties with a 4-day history of progressive areflexic quadriparesis following acute hepatitis a. a nerve - conduction study revealed inexcitability of most nerves . 
 the cerebrospinal fluid showed albuminocytologic dissociation , suggesting guillain - barr syndrome ( gbs ) . 
 the patient appeared brain dead on day 4 , showing absent brainstem reflexes , respiratory failure , and fully dilated and fixed pupils . 
 this case is an example of how gbs can evolve and simulate a brain - dead state from fulminant deafferentation following acute hepatitis a.