Abstract
BackgroundThe recurrent Guillain-Barré syndrome (RGBS) is characterized by at least two GBS episodes with intervening remission. In a previous study of monophasic GBS, we reported that the magnitude of oxygen radical production ("respiratory burst") in peripheral blood leukocytes was inversely correlated to disease severity. The present study sought to establish a similar correlation in patients with RGBS.MethodsOxygen radical production in leukocytes was induced by formyl-Met-Leu-Phe (fMLF), Trp-Lys-Tyr-Met-Val-Met-NH2 (WKYMVM), or phorbol myristate acetate (PMA) and assessed by quantifying superoxide anion formed by the leukocyte NADPH oxidase.ResultsDisease severity, assessed using the MRC score, was negatively correlated to superoxide anion production triggered by fMLF or WKYMVM (p = 0.001 and 0.002, respectively; n = 10). Superoxide anion production also was significantly lower in RGBS patients with incomplete recovery after stimulation with fMLF (p = 0.004) or WKYMVM (p = 0.003).ConclusionWe conclude that a lower respiratory burst in leukocytes is strongly associated with a severe course of RGBS.
Highlights
The recurrent Guillain-Barré syndrome (RGBS) is a demyelinating disease of the peripheral nervous system characterized by episodes of relapsing and remitting symptoms with complete or near complete functional recovery between episodes [1,2,3]
A main finding in this study was that superoxide anion production by leukocytes in response to nicotinamide adenine dinucleotide phosphate (NADPH) oxidase-activating peptides was associated with severity of disease in patients with RGBS
We observed a significant association between the individual attack frequency and respiratory burst, indicating a higher tendency for the disease to recur in patients with low superoxide anion production
Summary
The recurrent Guillain-Barré syndrome (RGBS) is a demyelinating disease of the peripheral nervous system characterized by episodes of relapsing and remitting symptoms with complete or near complete functional recovery between episodes [1,2,3]. The nicotinamide adenine dinucleotide phosphate (NADPH) oxidase initiates the respiratory burst by reducing oxygen in phagocytic cells to form reactive oxygen species such as superoxide anions, hydrogen peroxide, toxic halids, and hydroxyl radicals. The recurrent Guillain-Barré syndrome (RGBS) is characterized by at least two GBS episodes with intervening remission. In a previous study of monophasic GBS, we reported that the magnitude of oxygen radical production ("respiratory burst”) in peripheral blood leukocytes was inversely correlated to disease severity. The present study sought to establish a similar correlation in patients with RGBS
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