Antecedent infections in Fisher syndrome: sources of variation in clinical characteristics

Abstract

The clinical features of Guillain-Barré syndrome (GBS) are highly variable, according to the type of antecedent infection. Although a major GBS phenotype, Fisher syndrome (FS), has been shown to be preceded by infections similar to those preceding GBS, whether or not the clinical features in FS also vary according to antecedent infection remains unclarified. Frequent antecedent infections among this study of 70 FS patients included Haemophilus influenzae [n = 15 (21%)], Campylobacter jejuni [n = 10 (14%)], and cytomegalovirus (CMV) [n = 6 (8.6%)]. Compared with other FS patients, H. influenzae-seropositive FS patients more frequently had a history of prior upper respiratory tract infection; double vision as the initial symptom; and, except for oculomotor disturbance, more rarely showed cranial nerve involvement. C. jejuni-related FS occurred predominantly in younger male patients and characteristically presented with blurred vision. According to GBS disability scale, CMV-related FS tended to be more severe, although every patient received immunotherapy. Serum anti-GQ1b IgG antibodies were detected in most cases, regardless of antecedent infection type. At the nadir of illness, the most frequent diagnosis in H. influenzae-related cases was "pure FS" without limb weakness or central nervous system involvement (71%), in C. jejuni-related cases "incomplete FS" such as acute ophthalmoparesis with or without ataxia (60%), and in CMV-related cases (50%) advanced conditions such as GBS overlap and Bickerstaff brainstem encephalitis. These findings indicate that the type of preceding infection determined the neurological features of FS. CMV-related FS appeared to be similar to H. influenzae- and C. jejuni-related FS regarding anti-GQ1b antibody-mediated pathogenesis, as opposed to CMV-related GBS.

Keywords: Antecedent infection; Campylobacter jejuni; Cytomegalovirus; Fisher syndrome; Haemophilus influenzae; Intercellular adhesion molecule 1.

Fig. 1

Serum concentration of soluble intercellular adhesion molecule 1 (sICAM-1) in a Fisher syndrome (FS) and b Guillain–Barré syndrome (GBS). The differences in serum concentrations between FS patients depending on antecedent infectious agents were not significant (a), whereas the GBS patients who had serological evidence of antecedent cytomegalovirus (CMV) infection showed a significantly higher concentration of sICAM-1 than patients with GBS in whom antecedent infectious agents had not been identified (*P = 0.04) (b). [None] denotes the patients in whom antecedent infectious agents had not been identified by the serological assays described in the text