Fulminant acute inflammatory demyelinating polyradiculoneuropathy: case report and literature review.

BACKGROUND

The inflammatory polyradiculoneuropathies (Guillain-Barré Syndrome) represent a diverse spectrum of diseases, with variable pathophysiological mechanisms (demyelinating versus axonal), clinical manifestations (motor, sensory, and autonomic), presentation patterns, and degrees of severity. Fulminant cases of total body paralysis with loss of all cranial nerve reflexes have been described, whereby patients appear to have lost all central nervous system (CNS) function.

METHODS

The case of a patient with a fulminant polyradiculoneuropathy in whom consideration was given to discontinuation of care and organ donation is presented. A positive oculocardiac response verified ongoing brainstem function and led to the diagnosis of an inflammatory polyradiculoneuropathy. The patient was successfully treated with plasmapheresis and intravenous immunoglobulins.

CONCLUSION

The oculocardiac response may be a useful addition to the routine examination in patients who are suspected of being cerebrally dead or of having absent brainstem function, especially in cases where a central lesion or underlying disease is not evident to explain the condition. Additional study and validation of the oculocardiac response in these situations is proposed.