Anti‐<scp>GQ</scp>1b antibody syndrome: anti‐ganglioside complex reactivity determines clinical spectrum

Fukami, Yasuyuki; Wong, Anna Hiu Yi; Funakoshi, Kengo; Safri, Ahmad Yanuar; Shahrizaila, Nortina; Yuki, Nobuhiro

doi:10.1111/ene.12769

Cited by 36 publications

(36 citation statements)

References 27 publications

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“…Clinical signs can be explained by target-specific antibodies [14]. GQ1b antigen is strongly expressed at the surface of oculomotor nerves, large-diameter dorsal root ganglion neurons and muscle spindle cells.…”

Section: Discussionmentioning

confidence: 99%

Peduncular Hallucinosis and Overlapping Antiganglioside Antibody Syndrome

Demas¹,

Costentin²,

Langlois³

et al. 2019

AJPN

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Guillain Barre Syndrome (GBS), Miller Fisher Syndrome (MFS) and Bickerstaff Brainstem Encephalitis (BBE)form part of the same disease spectrum, sharing some clinical signs and auto-immune based mechanisms with antiganglioside antibodies. GBS, the most common cause of subacute flaccid paralysis, is a well-known and extensively described syndrome. MFS is characterized by subacute ophthalmoplegia, ataxia and areflexia. In 1957, Bickerstaff described some patients who, in addition to ataxia and ophthalmoplegia, had abnormal mental status, corresponding to brainstem involvment. Neurological manifestations associated with anti-GQ1b antibodies can occur in isolation or in association. We report the case of a woman with an unusual psychiatric presentation (peduncular hallucinosis) of anti-GQ1b antibody syndrome, with features of these three diseases, and a dramatic improvement with immunoglobulin therapy. Peduncular hallucinosis are a rare form of visual hallucination. They are often described as colourful visions of people and animals, and are usually associated with vascular lesions in the midbrain and thalamus. This provides further evidence of the broad spectrum of neurological diseases associated with antiganglioside antibodies. To the best of our knowledge this is the first description of PH complicating BBE. Clinicians should be aware of the existence of overlapping syndrome with combined central and peripherical neurological system involvement.

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Section: Discussionmentioning

confidence: 99%

Peduncular Hallucinosis and Overlapping Antiganglioside Antibody Syndrome

Demas¹,

Costentin²,

Langlois³

et al. 2019

AJPN

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“…Among these, the Miller–Fisher syndrome (MFS), pharyngeal‐cervical‐brachial weakness, and Bickerstaff brainstem encephalitis (BBE) share autoantibodies against ganglioside Q1b (GQ1b) and have similar presentations that only differ in the existence of peripheral or central nervous system involvement . These similarities and their shared common causative factor have led to the definition of term “anti‐GQ1b antibody syndrome.” GQ1b gangliosides are highly expressed in the 3 cranial oculomotor nerves and the deep cerebellar nuclei, suggesting an important role of anti‐GQ1b antibodies in the pathogenesis of MFS and explaining their association with ophthalmoplegia in both BBE and MFS . Rare cases of atypical MFS with anti‐GQ1b antibodies presenting as acute ophthalmoparesis (AO) without ataxia or areflexia have been described .…”

Section: Introductionmentioning

confidence: 99%

“…2 GQ1b gangliosides are highly expressed in the 3 cranial oculomotor nerves and the deep cerebellar nuclei, suggesting an important role of anti-GQ1b antibodies in the pathogenesis of MFS and explaining their association with ophthalmoplegia in both BBE and MFS. 1,2 Rare cases of atypical MFS with anti-GQ1b antibodies presenting as acute ophthalmoparesis (AO) without ataxia or areflexia have been described. [3][4][5][6][7] Although back and limb pain has been frequently reported in GBS, headache is a rare condition in these disorders.…”

Section: Introductionmentioning

confidence: 99%

Headache and Ophthalmoparesis: Case Report of an “Atypical” Incomplete Miller–Fisher Syndrome

2018

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“…Neuromuscular disorders , trauma , neuro‐inflammation , headache and more general neurological topics complete the 2016 spectrum of the Journal's purview.…”

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confidence: 99%