Anti-GQ1b antibody syndrome

Takahiro Iizuka MD (

Dr. Iizuka of Kitasato University School of Medicine has no relevant financial relationships to disclose.

)
Francesc Graus MD PhD, editor. (

Dr. Graus, Emeritus Professor, Laboratory Clinical and Experimental Neuroimmunology, Institut D’Investigacions Biomédiques August Pi I Sunyer, Hospital Clinic, Spain, has no relevant financial relationships to disclose.

)
Originally released December 3, 2010; last updated November 30, 2020; expires November 30, 2023

Overview

Prior to the discovery of IgG antibodies against GQ1b, there were clinical variants that were considered linked to Guillain-Barré syndrome due to common clinical features shared with Guillain-Barré syndrome. The most notable are Miller Fisher syndrome, which is characterized by acute ophthalmoplegia, ataxia, areflexia, and Bickerstaff brainstem encephalitis, in which consciousness is also affected. Following the discovery in 1992 of an association of anti-GQ1b antibodies with Miller Fisher syndrome, other neurologic presentations with similar serological profiles emerged in the literature, which include Bickerstaff brainstem encephalitis, acute ophthalmoplegia, acute ataxic neuropathy, pharyngeal-cervical-brachial weakness, and acute vestibular syndrome. In this article, the author discusses the current understanding behind the anti-GQ1b antibody syndrome.

Key points

 

• Anti-GQ1b antibody syndrome represents a group of disorders that share a common serological profile with links also to Guillain-Barré syndrome.

 

• Anti-GQ1b antibodies are a serological marker of Miller Fisher syndrome and Bickerstaff brainstem encephalitis but can been detected in other neurologic syndromes as a continuous spectrum.

 

• The overall prognosis is good even without immunotherapy in those patients who have typical Miller Fisher syndrome or acute ophthalmoplegia, but intravenous immunoglobulin (IVIg) or plasma exchange is recommended when there is altered consciousness, limb weakness, or bulbar palsy.

 

• Important mimics to be excluded include brainstem syndromes and neuromuscular junction disorders. The presence of anti-GQ1b antibodies would make the latter disorders unlikely.

 

• It should be noted that Miller Fisher syndrome or cranial neuropathies can occur as early neurologic manifestations of SARS-CoV-2 infection without detection of anti-GQ1b antibodies.

Historical note and terminology

The most recognizable variant of Guillain-Barré syndrome is Miller Fisher syndrome, which was first described in 1956 by Charles Miller Fisher. He reported 3 patients who presented with a triad of total external ophthalmoplegia, ataxia, and areflexia subsequent to upper respiratory tract infection (Fisher 1956). The presence of cerebrospinal fluid albuminocytological dissociation and antecedent infection likened Miller Fisher syndrome to Guillain-Barré syndrome.

Prior to this, in 1951, Bickerstaff and Cloake described 3 patients who presented with prominent drowsiness followed by brainstem signs causing ophthalmoplegia, facial palsy, bulbar palsy, and ataxia following a prodrome of infective illness. The etiology of this condition was speculated to be similar to that of Guillain-Barré syndrome based on the presence of prodromal symptoms in all 3 patients, areflexia with CSF albuminocytological dissociation in 1 patient, and subsequent recovery of neurologic symptoms in all 3 patients (Bickerstaff and Cloake 1951). Six years later, Bickerstaff extended his observations including an additional 5 patients with similar patterns of brainstem signs, emphasizing the benign outcomes in all but 1 patient, who died following a seizure (Bickerstaff 1957). This condition has since been referred to as Bickerstaff brainstem encephalitis.

In 1992, Chiba and colleagues identified IgG antibodies against ganglioside GQ1b in 6 patients with typical Miller Fisher syndrome (Chiba et al 1992). The anti-GQ1b IgG antibodies were initially considered as a serological marker of Miller Fisher syndrome. However, the antibodies were also detected in patients with acute postinfectious ophthalmoplegia without ataxia (Chiba et al 1993), Guillain-Barré syndrome with ophthalmoplegia (Chiba et al 1993), and Bickerstaff brainstem encephalitis (Yuki et al 1993; Ito et al 2008). The latter findings supported the continuity between the 2 syndromes, with Miller Fisher syndrome representing 1 end of the spectrum and Bickerstaff brainstem encephalitis the other, with involvement of the central nervous system. Patients with milder clinical features, such as ophthalmoparesis without ataxia (Yuki 1996), or more extensive involvement with limb weakness (Mori et al 2001), were also found to have anti-GQ1b IgG antibodies on serological testing. In view of the common serological profile shared by this group of patients, it has been referred to as "anti-GQ1b antibody syndrome" (Shahrizaila and Yuki 2013).

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