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.
Highlights
Guillain–Barré syndrome (GBS) is a postinfectious autoimmune-mediated neuropathy, the clinical manifestations of which greatly vary among individual cases [1]
To clarify the pathogenesis of GBS, classifying GBS into subgroups based on the type of antecedent infection, such as “C. jejuniassociated GBS” and “cytomegalovirus (CMV)-associated GBS” could be important, because antecedent infections are closely associated with neurological phenotypes and specific autoantibodies [3]
Unlike for GBS, close associations of clinical features with types of antecedent infections are yet to be clarified for Fisher syndrome (FS), perhaps because of stereotypical consideration with regard to a relatively uniform clinical picture and presence of specific autoantibodies, and of limitations of our previous study which did not include cases of incomplete forms of FS, such as acute ophthalmoparesis without ataxia, and of advanced FS that included Bickerstaff brainstem encephalitis (BBE) [6]
Summary
Guillain–Barré syndrome (GBS) is a postinfectious autoimmune-mediated neuropathy, the clinical manifestations of which greatly vary among individual cases [1]. Fisher syndrome (FS) is a major clinical phenotype of GBS It is characterized neurologically by the triad of ophthalmoplegia, ataxia, and areflexia and immunologically by the frequent presence of anti-GQ1b IgG autoantibodies [5]. Our previous case-controlled serological study showed that C. jejuni and Haemophilus influenzae were the major identified agents of antecedent infection in FS [6]. Unlike for GBS, close associations of clinical features with types of antecedent infections are yet to be clarified for FS, perhaps because of stereotypical consideration with regard to a relatively uniform clinical picture and presence of specific autoantibodies (anti-GQ1b antibodies), and of limitations of our previous study which did not include cases of incomplete forms of FS, such as acute ophthalmoparesis without ataxia, and of advanced FS that included Bickerstaff brainstem encephalitis (BBE) [6]. The aim of this study was to clarify whether or not the clinical and laboratory features of FS vary as they do in GBS, according to antecedent infections
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