Central Pathology in Guillain-Barré Syndrome
Source: Maier H, et al. Central nervous system pathology in patients with Guillain-Barré syndrome. Brain 1997;120: 451-464.
Aretrospective review of 13 autopsied guillain-Barré syndrome (GBS) cases from 1957 to 1991, who were examined between one day and 12 months following syndrome onset, revealed central nervous system (CNS) pathology in the spinal cord (8 of 13), medulla (8 of 12), pons (5 of 9), midbrain (1 of 4), basal ganglia (2 of 11), and periventricular hippocampal temporal cortex (1 of 11). None of the changes was demyelinating in nature but included microglial activation and mononuclear cell (macrophage and T lymphocyte) infiltration, and were in addition to the expected secondary changes consequent to peripheral nervous system pathology (anterior horn cell axonal reaction/chromatolysis in six of 13, dorsal column degeneration in three of 13). No patient had received immunoglobulin, only one received plasma exchange, and two were treated with steroids. These findings reveal more extensive changes than previous reports indicate and may reflect the predominantly untreated nature of this particular retrospective group of patients.
Unfortunately, clinicopathological correlation is not given. As a rule, GBS patients demonstrate no clinically important CNS abnormalities, though rarely Babinski signs, a sensory level, or ocular findings suggest a central origin and might be explained by lesions similar to those described. Though clinically insignificant and of uncertain etiopathogenesis, they may result from alterations of the blood-brain barrier allowing spillage of the inflammatory response into the CNS. Using rabbit retina, both interleukin-1 and tumor necrosis factor have been shown to increase endothelial pinocytic activity after intraocular injection. Interleukin-1 additionally induces inflammation with significant edema and hemorrhage formation (Brosnan CF, et al. Ann Neurol 1990;27 [suppl]:S75-S79). Elevated serum cytokine levels thus may increase vascular permeability and contribute to CNS pathology in GBS. mr