Acute comitant strabismus in anti-GQ1b antibody syndrome

Lee, Sun-Uk; Baek, Seol-Hee; Kim, Hyojung; Choi, Jeong‐Yoon; Kim, Byung Jo; Kim, Ji‐Soo

doi:10.1007/s00415-022-11394-3

Cited by 3 publications

(6 citation statements)

References 45 publications

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“…54 Similar to typical paralytic ocular motor nerve palsies, the prognosis is excellent with a full recovery mostly within 6 months. 54…”

Section: Iridoplegiamentioning

confidence: 80%

“…However, the amount of ocular misalignment may be un-changed regardless of the direction of gaze or of which eye is fixating the target (ie, comitant). 53,54 In comitant strabismus, the saccadic velocity is mostly preserved. 53,54 Given the accompanying central ocular motor signs, such as spontaneous or positional downbeat nystagmus, saccadic hypermetria, or pulse-step mismatch, comitant strabismus observed in patients with anti-GQ1b antibody syndrome may be ascribed to dysfunction of the cerebellum or brainstem.…”

Section: Iridoplegiamentioning

confidence: 99%

“…53,54 In comitant strabismus, the saccadic velocity is mostly preserved. 53,54 Given the accompanying central ocular motor signs, such as spontaneous or positional downbeat nystagmus, saccadic hypermetria, or pulse-step mismatch, comitant strabismus observed in patients with anti-GQ1b antibody syndrome may be ascribed to dysfunction of the cerebellum or brainstem. 54 Similar to typical paralytic ocular motor nerve palsies, the prognosis is excellent with a full recovery mostly within 6 months.…”

Section: Iridoplegiamentioning

confidence: 99%

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Expanding Clinical Spectrum of Anti-GQ1b Antibody Syndrome

Lee,

Kim,

Choi

et al. 2024

JAMA Neurol

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ImportanceThe discovery of the anti-GQ1b antibody has expanded the nosology of classic Miller Fisher syndrome to include Bickerstaff brainstem encephalitis, Guillain-Barré syndrome with ophthalmoplegia, and acute ophthalmoplegia without ataxia, which have been brought under the umbrella term anti-GQ1b antibody syndrome. It seems timely to define the phenotypes of anti-GQ1b antibody syndrome for the proper diagnosis of this syndrome with diverse clinical presentations. This review summarizes these syndromes and introduces recently identified subtypes.ObservationsAlthough ophthalmoplegia is a hallmark of anti-GQ1b antibody syndrome, recent studies have identified this antibody in patients with acute vestibular syndrome, optic neuropathy with disc swelling, and acute sensory ataxic neuropathy of atypical presentation. Ophthalmoplegia associated with anti-GQ1b antibody positivity is complete in more than half of the patients but may be monocular or comitant. The prognosis is mostly favorable; however, approximately 14% of patients experience relapse.Conclusions and RelevanceAnti-GQ1b antibody syndrome may present diverse neurological manifestations, including ophthalmoplegia, ataxia, areflexia, central or peripheral vestibulopathy, and optic neuropathy. Understanding the wide clinical spectrum may aid in the differentiation and management of immune-mediated neuropathies with multiple presentations.

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“…54 Similar to typical paralytic ocular motor nerve palsies, the prognosis is excellent with a full recovery mostly within 6 months. 54…”

Section: Iridoplegiamentioning

confidence: 80%

Section: Iridoplegiamentioning

confidence: 99%

Section: Iridoplegiamentioning

confidence: 99%

See 1 more Smart Citation

Expanding Clinical Spectrum of Anti-GQ1b Antibody Syndrome

Lee,

Kim,

Choi

et al. 2024

JAMA Neurol

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“…Anti-GQ1b antibodies cause reversible damage to the brain stem. [3,6] One of the largest series of reports published showed, [7] T2-weighted images 11% of 47 Bickerstaff brainstem encephalitis patients had high intensity abnormalities in the pons (n = 4), thalamus (n = 2), cerebellum (n = 1), medulla oblongata (n = 1), midbrain (n = 1), superior cerebellar peduncle (n = 1), or corpus callosum (n = 1). Boeun Lee et al [5] conducted a retrospective analysis of 16 high-resolution MR examinations in 15 patients with anti-GQ1b antibody syndrome, as 1 patient (case 5) had repeated MRI examinations for delayed facial paralysis.…”

Section: Discussionmentioning

confidence: 99%

Brain death-like changes: A case report of anti-GQ1b antibody syndrome

et al. 2023

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Rationale: Anti-GQ1b antibody syndrome originates in the peripheral nervous system or the central nervous system. Various overlapping syndromes and atypical manifestations are also seen in clinic. It is rare to see multiple sites (almost all targets) of central and peripheral damage at the same time, resulting in brain death-like changes in patient.Patient concerns: This is a 55-year-old female patient, with a history of prodromal upper respiratory tract infection, began with headache, quickly developed body paralysis, eye paralysis, disturbance of consciousness, apnea, and tested positive for anti-GQ1b antibody. The patient is diagnosed clearly, the disease progresses rapidly, and almost all GQ1b sites in the central nervous system and peripheral nervous system are involved, which is rare.

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“…Thus, anti-GQ1b antibody syndrome can be considered in patients with acute/ subacute central vestibulopathy with normal imaging. 7,9 In addition, it can also be found in the acute sera of patients presenting with typical features of acute unilateral peripheral vestibulopathy (AUPV), 8 suggesting that the peripheral vestibular system is a potential target of antiganglioside antibodies. Furthermore, other ganglioside antibodies, such as anti-GD1b and GM1, may be involved in syndromes that may present as AVS.…”

Section: Introductionmentioning

confidence: 99%

Clinical Features and Neurotologic Findings in Patients With Acute Unilateral Peripheral Vestibulopathy Associated With Antiganglioside Antibody

Kim,

Park,

Lee

et al. 2023

Neurology

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Background and Objectives: Anecdotal studies have reported the presence of anti-ganglioside antibodies in acute unilateral peripheral vestibulopathy (AUPV). This study aimed to determine the prevalence, clinical characteristics, and neurotologic findings of AUPV associated with anti-ganglioside antibodies. Methods: Serum anti-gangliosides were measured in consecutive patients with AUPV according to the Bárány Society criteria during the acute and recovery phases, in a referral-based university hospital in South Korea from September 2019 to January 2023. Clinical characteristics and neurotologic findings were compared between those with and without anti-ganglioside antibodies. The results of video-oculography, video head-impulse and bithermal caloric tests, and other neurotologic evaluations including ocular and cervical vestibular-evoked myogenic potentials, subjective visual vertical were compared between the two. MRIs dedicated to the inner ear were also conducted when considered necessary. Results: One hundred and five patients (mean age±standard deviation [SD] = 60±13 years, 57 male) were included for analyses. During the acute phase, 12 patients (12/105, 11%) were tested positive for serum anti-ganglioside antibodies, including anti-GQ1b IgG (n=5) or IgM (n=4), anti-GM1 IgM (n=3), and anti-GD1a IgG (n=1, including one with a positive anti-GQ1b antibody). Patients with anti-ganglioside antibodies showed lesser intensity of spontaneous nystagmus (median [interquartile range (IQR)] = 1.8 [1.2–2.1] vs. 3.4 [1.5–9.5], p=0.003), a lesser degree of canal paresis (30 [17–47] vs. 58 [34–79], p=0.028), and gain asymmetry of the vestibulo-ocular reflex (VOR) for the horizontal semicircular canal (HC) during head impulse tests (HITs) (0.07 [-0.04–0.61] vs. 0.36 [0.18–0.47], p=0.032) than those without antibodies. Negative conversion of antibodies and vestibular recovery were observed in most patients (6/8, 75%). Among 30 AUPV patients with 4-hour delayed 3D-FLAIR dedicated to the inner ear, gadolinium enhancement was observed in 18 (18/30, 60%), either in the vestibule (n=9), semicircular canal (n=6), or vestibular nerve (n=5). The positivity rates based on specific antibodies could not be determined due to limited sample sizes. Discussion: The association between anti-ganglioside antibodies and AUPV suggests an immune-mediated mechanism in acute vestibular failure and extends the clinical spectrum of anti-ganglioside antibody syndrome.

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Acute comitant strabismus in anti-GQ1b antibody syndrome

Cited by 3 publications

References 45 publications

Expanding Clinical Spectrum of Anti-GQ1b Antibody Syndrome

Expanding Clinical Spectrum of Anti-GQ1b Antibody Syndrome

Brain death-like changes: A case report of anti-GQ1b antibody syndrome

Clinical Features and Neurotologic Findings in Patients With Acute Unilateral Peripheral Vestibulopathy Associated With Antiganglioside Antibody

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