Abstract
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.
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References
Ropper A. The Guillain-Barré syndrome. N Engl J Med 1992;326:1130–1136.
Duncan R, Kennedy P. Guillain-Barré Syndrome following acute head trauma. Postgrad Med J 1987;63:479–480.
Stojkovic T, Verdin M, Hurtevent J, Laureau E, Krivosic R, Vermersch P. Guillain-Barré syndrome resembling brainstem death in a patient with brain injury. J Neurol 2001;248:430–432.
Arnason B, Soliven B. Acute inflammatory demyelinating polyradiculoneuropathy. In PJ Dyck: Peripheral Neuropathy, 3rd ed. Philadelphia: Saunders, 1993:1437–1497.
Ropper AH, Wijdicks EFM, Traux BT, eds. Guillain-Barré syndrome. Philadelphia: Davis, 1991.
Carroll W, Mastaglia F. Locked-in coma in postinfective polyneuropathy. Arch Neurol 1979;36:46–47.
Kotsoris H, Schleifer L, Menken M, Plum F. Total locked-in state resembling brain death in polyneuropathy. Ann Neurol 1984;16:150.
Drury I, Westmoreland B, Sharbrough F. Fulminant demyelinating polyradiculoneuropathy resembling brain death. Electroencephalogr Clin Neurophysiol 1987;67:42–43.
Kanda T, Hayashi H, Tanabe H, Tsubaki T, Oda M. A fulminant case of Guillain-Barré syndrome: topographic and fibre size related analysis of demyelinating changes. J Neurol Neurosurg Psychiatry 1989;52:857–864.
Coad N, Byrne A. Guillain-Barré syndrome mimicking brainstem death. Anaesthesia 1990;45:456–457.
Hassan T, Mumford C. Guillain-Barré syndrome mistaken for brain stem death. Postgrad Med J 1991;67:280–281.
Fuller G, Jacobs J, Lewis P, Lane R. Pseudoaxonal Guillain-Barré Syndrome-severe demyelination mimicking axonopathy. A case with pupillary involvement. J Neurol Neurosurg Psychiatry 1992;55:1079–1083.
Masso J, Suarez J, Lopez de Munain A, Carrera N. Clinical signs of brain death simulated by Guillain-Barré syndrome. J Neurol Sci 1993;120:115–117.
Tan A, Chee M. Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination. J Neurol Sci 1995;134:203–206.
Hughes R, McGuire G. Neurologic disease and the determination of brain death. Crit Care Med 1997;25:1923–1924.
Bakshi N, Maselli R, Gospe S, Ellis W, McDonald C, Mandler R. Fulminant demyelinating neuropathy mimicking cerebral death. Muscle Nerve 1997;20:1595–1597.
Bohlega S, Stigsby B, Haider A, McLean D. Guillain-Barré syndrome with severe demyelination mimicking axonopathy. Muscle Nerve 1997;20:514–516.
Berciano J, Figols J, Garcia A, et al. Fulminant Guillain-Barré syndrome with universal inexcitability of peripheral nerves: a clinicopathological study. Muscle Nerve 1997;20:846–8457.
Ragazzoni A, Grippo A, Tozzi F, Gaetano Z. Event related potentials in patients with total locked-in state due to fulminant Guillain-Barré syndrome. Int J Psychophysiol 2000;37:99–109.
Vargas F, Hilbert G, Gruson D, Valentino R, Benissan G, Cardinaud J. Fulminant Guillain-Barré syndrome mimicking cerebral death: case report and literature review. Intensive Care Med 2000;26:623–627.
New York State Department of Health. Clinical guidelines for the determination of death[online]. Available at: http://www.health.state.ny.us/nysdoh/bsd/attacha.htm. Accessed April 5, 2003.
Lang S, Lanigan D, van der Wal M. Trigeminocardiac reflexes: maxillary and mandibular variants of the oculocardiac reflex. Can J Anaesth 1991;38:757–760.
Van Brocklin M, Hirons R, Yolton R. The oculocardiac reflex: a review. J Am Optom Assoc 1982;53:407–413.
Hunyor A. Reflexes and the eye. Aust N Z J Ophthalmol 1994;22:155–159.
Haerer A. DeJong’s The Neurologic Examination, 5th ed. Philadelphia, Lippincott, 1992.
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Moussouttas, M., Chandy, D. & Dyro, F. Fulminant acute inflammatory demyelinating polyradiculoneuropathy. Neurocrit Care 1, 469–473 (2004). https://doi.org/10.1385/NCC:1:4:469
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DOI: https://doi.org/10.1385/NCC:1:4:469