Guillain-Barré Syndrome with Absent Brainstem Reflexes – a Report of Two Cases

Abstract

Guillain-Barré syndrome, known for its diverse, atypical and heterogeneous range of presentations, can rarely present in an apparent comatose state with absent brainstem reflexes. Two patients presented in an unresponsive state with flaccid quadriplegia, total areflexia with no response to cephalic or peripheral painful stimuli. Pupils were mid-dilated with absent direct and consensual light reflex. All cranial nerve reflexes were absent. Preliminary laboratory investigations and complementary tests were normal. Cerebrospinal fluid evaluation showed albumincytological dissociation. Brain magnetic resonance imaging and electroencephalogram were normal. Both were diagnosed as fulminant Guillain-Barré syndrome. Despite an absence of brainstem reflexes neither patient fulfilled diagnostic criteria for brain death.

Are There Islands of Awareness?

“…Sensory-motor disconnection with preserved awareness can also be observed in extreme cases within the spectrum of acute inflammatory polyneuropathy, such as fulminant Guillain-Barré syndrome (GBS) [12]. In some GBS patients, a complete paralysis extending to cranial nerves can be accompanied by a severe (albeit not complete) blockage of multiple sensory nerves [13][14][15]. In the initial stages of the condition patients are clearly conscious and can communicate through residual movements.…”

Brain Death Guidelines Explained

“…There is always the concern that the patient presents with a mimicking condition such as profound accidental hypothermia, major drug intoxication, and extreme cases of severe Guillain-Barré syndrome. [2][3][4][5][6][7] Irreversibility is determined by two factors. First, the patient should have been treated aggressively, such as administration of osmotic agents, neurosurgical evacuation of a mass causing displacement of the brainstem (particularly in the cerebellum), ventriculostomy placement, or other measures to reduce intracranial pressure (ICP).…”

Muerte encefálica. Elementos clínicos y métodos auxiliares para su diagnóstico

“…Existen múltiples entidades que pueden simular ME y provocar incer tidumbre en su determinación, algunas de estas son síndrome de enclaustramiento, Guillain-Barré, hipotermia, intoxicación por organofosforados, etanol, lidocaína, baclofeno, vecuronio, antidepresivos tricíclicos, incluso por lesiones medulares cervicales altas. Sin embargo, esta duda diagnóstica puede esclarecerse de manera fidedigna si se realiza una historia clínica adecuada, además, en estos casos las alteraciones neurológicas suelen ser reversibles y no presentan un trazo isoeléctrico en el EEG [20][21][22][23][24][25][26][27][28][29][30][31] ; desde que se utilizan los criterios que recomienda la AAN, no existen reportes sobre recuperación de la función encefálica después de declarar ME 18 . Es importante mencionar que a pesar de la administración de barbitúricos, la determinación está permitida siempre y cuando sus niveles séricos se encuentren por debajo del rango terapéutico, en caso de que no sea posible cuantificarlos está indicado un periodo de observación, considerando su depuración e interacciones farmacológicas 17,18 .…”