Guillain-Barré syndrome (GBS) encompasses a spectrum of acute immune-mediated polyneuropathies. Rarely, in its fulminant form, it can rapidly progress to mimic brain death. We present a case with diagnostic challenges due to a limited history.
A 58-year-old gentleman presented generally unwell and within hours of admission choked on his dinner and suffered a cardiorespiratory arrest. Review on ITU revealed absent brainstem reflexes apart from a weak pupillary light response.
EEG showed slowing with subtle reactivity, MRI Brain was normal, and CSF revealed albuminocytological dissociation.
On day 27 of admission, he was found to follow commands with eye movements, appearing to be in an incomplete locked-in state. Nerve conduction studies confirmed a severe axonal sensorimotor neuropathy with sural sparing. He was treated with plasma exchange and intravenous immunoglobulins and continues to make significant motor improvements each week.
This case highlights the crucial recognition of this extreme presentation of GBS, as a misdiagnosis of brain death may lead to the withdrawal of ventilatory support. In a third of reported similar cases there has been a good recovery and in the majority (as in our case) serum ganglioside antibodies have been negative, raising the possibility of alternative nodal and paranodal target antigens.
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